Follow-up of vestibular function in bilateral vestibulopathy

Objective: Bilateral vestibulopathy (BV) leads to a bilateral deficit of the vestibulo-ocular reflex and has various aetiologies. The main goal of this study was to determine the frequency and degree of recovery or worsening of vestibular function over time.Methods: 82 patients (59 males, 23 females; mean age at the time of diagnosis 56.3 (SD 17.6) years) were re-examined 51 (36) months after the first examination. All patients underwent a standardised neuro-ophthalmological and neuro-otological examination. Electronystagmography with bithermal caloric irrigation was analysed by measurement of the mean peak slow phase velocity (SPV) of the induced nystagmus. Patients evaluated the course of their disease in terms of balance, gait unsteadiness and health related quality of life.Results: Statistical analysis of the mean peak SPV of caloric induced nystagmus revealed a non-significant worsening over time (initial mean peak SPV 3.0 (3.5)°/s vs 2.1 (2.8)°/s). With respect to subgroups of aetiology, only patients with BV due to meningitis exhibited an increasing, but non-significant SPV (1.0 (1.4)°/s vs 1.9 (1.6)°/s). Vestibular outcome was independent of age, gender, time course of manifestation and severity of BV. Single analysis of all patients showed that a substantial improvement ⩾5°/s occurred in two patients on both sides (idiopathic n = 1, Sjögren's syndrome n = 1) and in eight patients on one side (idiopathic n = 6, meningitis n = 1, Menière's disease n = 1). In 84% of patients there was impairment of their health related quality of life (42% slight, 24% moderate, 18% severe). Forty-three per cent of patients rated the course of their disease as stable, 28% as worsened and 29% as improved.Conclusions: Our data support the view that more than 80% of patients with BV do not improve. Thus the prognosis of BV is less favourable than assumed

Central compensation of deviated subjective visual vertical in Wallenberg's syndrome

The central compensation of vestibular tonus imbalance due to unilateral peripheral vestibular lesions has been repeatedly documented. Little is known, however, about the central compensation of vestibular tonus imbalance due to central lesions. Dorsolateral medullary infarctions (Wallenberg's syndrome) typically cause a central vestibular tonus imbalance in the roll plane with deviations of perceived verticality and ipsiversive body lateropulsion. The course of normalisation of the tilts of subjective visual vertical (SVV) in 50 patients who had acute Wallenberg's syndrome were retrospectively compared with that in 50 patients with acute vestibular neuritis. The initial displacement of SVV was 9.8° in Wallenberg's syndrome and 7° in vestibular neuritis. The deviation of SVV significantly decreased over time within days to weeks in both groups. This finding shows that the time courses of the central compensation for dorsolateral medullary infarctions and peripheral vestibular lesions are similar

A randomised double-blind, cross-over trial of 4-aminopyridine for downbeat nystagmus--effects on slowphase eye velocity, postural stability, locomotion and symptoms

Objective The effects of 4-aminopyridine (4-AP) on downbeat nystagmus (DBN) were analysed in terms of slow-phase velocity (SPV), stance, locomotion, visual acuity (VA), patient satisfaction and side effects using standardised questionnaires. Methods Twenty-seven patients with DBN received 5 mg 4-AP four times a day or placebo for 3 days and 10 mg 4-AP four times a day or placebo for 4 days. Recordings were done before the first, 60 min after the first and 60 min after the last drug administration. Results SPV decreased from 2.42 deg/s at baseline to 1.38 deg/s with 5 mg 4-AP and to 2.03 deg/s with 10 mg 4-AP (p<0.05; post hoc: 5 mg 4-AP: p=0.04). The rate of responders was 57%. Increasing age correlated with a 4-AP-related decrease in SPV (p<0.05). Patients improved in the ‘get-up-and-go test’ with 4-AP (p<0.001; post hoc: 5 mg: p=0.025; 10 mg: p<0.001). Tandem-walk time (both p<0.01) and tandem-walk error (4-AP: p=0.054; placebo: p=0.059) improved under 4-AP and placebo. Posturography showed that some patients improved with the 5 mg 4-AP dose, particularly older patients. Near VA increased from 0.59 at baseline to 0.66 with 5 mg 4-AP (p<0.05). Patients with idiopathic DBN had the greatest benefit from 4-AP. There were no differences between 4-AP and placebo regarding patient satisfaction and side effects. Conclusions 4-AP reduced SPV of DBN, improved near VA and some locomotor parameters. 4-AP is a useful medication for DBN syndrome, older patients in particular benefit from the effects of 5 mg 4-AP on nystagmus and postural stability

Resting in darkness improves downbeat nystagmus: evidence from an observational study.

Resting in an upright position during daytime decreases downbeat nystagmus (DBN). When measured in brightness only, that is, without intermitting exposure to darkness, it does not make a significant difference whether patients have previously rested in brightness or in darkness. In real-world scenarios, people are often exposed to brightness and darkness intermittently. The aim of this study was to analyze whether resting in brightness or resting in darkness was associated with a lower post-resting DBN after intermitting exposures to brightness and darkness. Eight patients were recorded with three-dimensional video-oculography in brightness and darkness conditions, each following two 2-h resting intervals under either brightness or darkness resting conditions. The dependent variable was DBN intensity, measured in mean slow phase velocity. A repeated measures ANOVA with the factors measurement condition (brightness vs. darkness), resting condition (brightness vs. darkness), and time (after first vs. second resting interval) showed a significant effect for the factor resting condition, where previous resting in darkness was associated with a significantly lower DBN relative to previous resting in brightness (P < 0.01). The clinical relevance is to advise patients with DBN to rest in darkness