The mechanism of the vestibulo-ocular reflex.<unk>  is caused by a failure in the function of the flocculus of the vestibulocerebellum, one of the three main divisions of the cerebellum. Generally, the cerebellum is responsible for regulating motor commands. 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. The vestibulo-ocular reflex, one of the primary areas affected by<unk> , is responsible for counterrotating the eyes in response to head movements. This allows gaze to stay fixed on a specific point. A failure in this reflex results in a variety of eye movement abnormalities, such as those exhibited in<unk> .<unk>  has been categorized as an autosomal dominant neurological disorder although the specific effect on the vestibulocerebellum is unknown. It is possible that inheritance causes abnormalities in either the flocculus or in structures that project into the flocculus to maintain stability of the retinal image of stationary or moving visual objects. Pathological symptoms of the disorder may appear within the first 1–2 years of life although time of onset varies greatly among patients. The severity of symptoms typically progresses with age. The exact cause of the disorder and its pathogenic effect on the flocculus is unknown. A single genetic locus, however, critical in early eye movement control pathways on chromosome 13q31-q33 has been discovered. This locus may be involved in some of the ocular abnormalities that occur in affected individuals. 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<unk> , or for better-understood cerebellar ataxias.<unk>  shares clinical similarities with autosomal dominant ataxias, particularly episodic ataxia types 1 and 2. These similarities include gaze-evoked and rebound nystagmus and vertigo. Furthermore, the symptoms appear to progress over time. The molecular basis of many of these other disorders has been thoroughly established and in some cases a genetic locus has been identified. Despite the similarities between symptoms of episodic ataxia and<unk> , studies of affected individuals have shown that the disorder is genetically distinct from these other similar neurological conditions. To date, the molecular basis of<unk>  remains undefined.
Vestibulocerebellar syndrome
Cerebellar stroke syndrome
Lateral pontine syndrome
Occipital horn syndrome
Dejerine–Roussy syndrome
Medial pontine syndrome
Lateral medullary syndrome
Amyotrophic lateral sclerosis