[4] Gaze-paretic nystagmus, one of the most common symptoms among patients results in poor gaze-holding due to neuron integrator dysfunction.

Initially, symptoms present as isolated episodic attacks but occur at increasing frequency over time and may eventually become a permanent condition.

[2] In conjunction with eye abnormalities patients also present with periodic attacks of vertigo, tinnitus, and ataxia that are associated with sudden changes in head position.

[6] During a typical attack, patients reported having ataxic gait with a tendency to fall to either side while lacking the ability to walk heel-to-toe.

These conditions do not consistently cause the symptoms of dizziness and ocular impairment that have been localized to the vestibulocerebellum, leading researchers to characterize vestibulocerebellar syndrome as a distinct disorder.

The main function of the vestibulocerebellum is to receive sensory input from the vestibular nuclei in the brainstem and to regulate equilibrium, balance, and the vestibulo-ocular reflex accordingly.

Chromosome 13q31-q33, however, has not been seen to correspond to any known existing gene or locus responsible for congenital nystagmus, one of the primary symptoms of vestibulocerebellar syndrome, or for better-understood cerebellar ataxias.

Lying down stabilizes the head in a fixed position while closing the eyes removes the unstable sensory input responsible for the dizziness.

Treatment is presently focused on addressing specific symptoms to alleviate the nausea that often accompanies attacks and to make daily life more manageable.

In trials of patients who have vestibulocerebellar syndrome, acetazolamide either eliminated or significantly decreased the frequency and severity of vertigo episodes.

[10] When a diagnosis is made in early adulthood on the basis of periodic attacks of vertigo, diplopia, and tinnitus, one can expect recurrent episodes of progressive ataxia later in life.

Periodic vestibulocerebellar syndrome has been discovered in several generations of three families with genetic ties to Johnston County, North Carolina.

The second case was studied and published in 1984 by Vance et al.[6][11] Individuals in these families were categorized as affected if they exhibited one form of primary criteria (ataxia, marked loss of smooth pursuit, gaze-evoked nystagmus, and impaired vestibulo-ocular reflex suppression) and at least one secondary criteria (mild loss of smooth pursuit, mild gaze-evoked nystagmus, and esophoria or esotropia).

[11] In addition to these variations, vestibulocerebellar syndrome is also difficult to distinguish from other neurological disorders that result in similar degenerative symptoms such as ataxia and multiple sclerosis.

Although no individuals have presented with brain abnormalities, it is possible that one of these candidate genes could have a more minor mutation, leading to the symptoms of vestibulocerebellar syndrome.