Downbeat nystagmus: evidence for enhancement of utriculo-ocular pathways by ocular vestibular evoked myogenic potentials?

Downbeat nystagmus (DBN) is caused by an impairment of Purkinje cells in the flocculus. The decreased cerebellar inhibitory input affects otolith pathways. Since ocular and cervical vestibular evoked myogenic potentials (o-/cVEMP) test the otoliths, the VEMP were measured in DBN patients and in controls. Sixteen patients with DBN, 14 cerebellar oculomotor disorder patients without DBN (COMD), and 16 healthy controls were examined with o-/cVEMP. Computational modeling was used to predict VEMP differences between groups. DBN patients had significantly higher oVEMP peak-to-peak (PP) amplitudes than COMD patients without DBN and controls. Cervical VEMP did not differ. The computational model of DBN predicted a twofold oVEMP increase for DBN patients. These findings suggest an enhancement of the utriculo-ocular response. The unchanged cVEMP indicate no effect on the otolith-cervical reflex in DBN. Computational modeling suggests that the utriculo-ocular enhancement is caused by an impaired vertical neural integrator resulting in the increased influence of utricular signals. This also explains the gravitational dependence of DBN.

Gray-matter atrophy after chronic complete unilateral vestibular deafferentation.

It has been shown in blind patients that the abolition of sensory input can lead to changes in white- and cortical gray-matter volumes. Here the white- and gray-matter changes found with whole brain voxel-based morphometry in 16 patients with complete chronic unilateral vestibular deafferentation (UVD) due to vestibular schwannoma removal several years prior are reported on. Subtle deficits in spatial memory and navigation were previously shown in patients with right UVD. Images of the brains of right-UVD patients were flipped, standard preprocessing steps were performed, and the data were modulated. Patients showed a gray-matter volume reduction in the cerebellum due to schwannoma removal, in the supramarginal gyrus ipsilateral to the lesion, as well as in the postcentral and superior temporal gyrus, areas involved in the vestibular cortical network, and in the motion-sensitive area MT/V5. There was no correlation with behavioral navigational abilities. No gray-matter atrophy was found in the insular cortical vestibular region or the hippocampus, both of which receive bilateral vestibular projections. The thalamus and tegmentum of the mesencephalon showed gray-matter reduction on the opposite side; this was possibly due to reduced auditory input, which is known to cross at this level. In comparison to healthy controls, no regional increases in gray-matter volume were seen. No white-matter changes were detected at the selected threshold.

Downbeat nystagmus caused by a paramedian ponto-medullary lesion.

Downbeat nystagmus (DBN) is rarely caused by lesions in sites other than the cerebellar (para-)floccular lobe. We describe a case of DBN secondary to a hemorrhaged venous cavernoma at the pontomedullary junction. This case provides new insights into the neuro-anatomical substrate of DBN. We propose that DBN arises from lesions in a brainstem-cerebellar feedback loop, which comprises cells of the pontine paramedian tract (PMT).

Spatial memory and hippocampal volume in humans with unilateral vestibular deafferentation.

Patients with acquired chronic bilateral vestibular loss were recently found to have a significant impairment in spatial memory and navigation when tested with a virtual Morris water task. These deficits were associated with selective and bilateral atrophy of the hippocampus, which suggests that spatial memory and navigation also rely on vestibular input. In the present study 16 patients with unilateral vestibular deafferentation due to acoustic neurinoma were examined 5- to 13-yrs post-surgery. Volumetry of the hippocampus was performed in patients and age- and sex-matched healthy controls by manually tracing the structure and by an evaluator-independent voxel-based morphometry. Spatial memory and navigation were assessed with a virtual Morris water task. No significant deficits in spatial memory and navigation could be demonstrated in the patients with left vestibular failure, whereas patients with right vestibular loss showed a tendency to perform worse on the respective tests. Impairment was significant only for one computed measure (heading error). The subtle deficiencies with right vestibular loss are compatible with the recently described dominance of the right labyrinth and the vestibular cortex in the right hemisphere. Volumetry did not reveal any atrophy of the hippocampus in either patient group.

A clinical test of otolith function: static ocular counterroll with passive head tilt.

When roll-tilted around the naso-occipital axis, humans exhibit compensatory torsional rotation of the eyes in the opposite direction owing to the torsional vestibulo-ocular reflex. In the static condition (sustained head roll), the utricles act as responsible sensors for 'static ocular counterroll'. Contributions of cervico-ocular reflexes remain unknown. To find an easy, clinically useful test of utricular function, we induced ocular counterroll in 10 healthy study participants (two men, mean age 27+/-2 years) under three stimulation conditions (active/passive head tilt and passive whole body tilt in roll plane), used three-dimensional video-oculography to measure it, and compared values. Active head-tilt-induced ocular counterroll varied most and was thus less reliable than passive head and body tilt-induced ocular counterroll. Utricular function can thus be tested simply by measuring passive head tilt with video-oculography.