A randomized double-blind, placebo-controlled, cross-over trial (Vestparoxy) of the treatment of vestibular paroxysmia with oxcarbazepine.

Objective: Vestibular paroxysmia (VP) is characterized by short, often oligosymptomatic attacks of vertigo which occur spontaneously or are sometimes provoked by turning the head. Despite the description of the disease almost 40 years ago (first termed "disabling positional vertigo"), no controlled treatment trial has been published to date. The Vestparoxy trial was designed as a randomized, placebo-controlled, double-blind cross-over trial to examine the therapeutic effect of oxcarbazepine (OXA) in patients with definite or probable VP.

Vestibular function in patients with Niemann-Pick type C disease.

We investigated whether vestibular dysfunction may cause or contribute to postural imbalance and falls in patients with Niemann-Pick type C disease (NP-C). Eight patients with NP-C disease and 20 healthy controls were examined using the video-based head impulse test (vHIT) and caloric irrigation to investigate horizontal canal function as well as ocular- and cervical vestibular evoked myogenic potentials (o- and cVEMP), and binocular subjective visual vertical estimation (SVV) for otolith function, and static posturography. There were no significant differences in vestibulo-ocular gain, caloric excitability, o-/cVEMP measures or SVV between the two groups.

Normal pressure hydrocephalus: Increase of utricular input in responders to spinal tap test.

Objective: To investigate whether there is a change in ocular (oVEMP) and cervical (cVEMP) vestibular evoked myogenic potentials in patients with normal pressure hydrocephalus (NPH) before and after spinal tap test (STT).

Methods: In 25 patients (6 females, age 62-83years) c/oVEMP were measured before and after STT. Patients with an increase of >20% of walking velocity were classified as responders (n=10).

Comparison of linear motion perception thresholds in vestibular migraine and Menière's disease.

Linear motion perceptual thresholds (PTs) were compared between patients with Menière's disease (MD) and vestibular migraine (VM). Twenty patients with VM, 27 patients with MD and 34 healthy controls (HC) were examined. PTs for linear motion along the inter-aural (IA), naso-occipital axes (NO), and head-vertical (HV) axis were measured using a multi-axis motion platform.

Update on the Pharmacotherapy of Cerebellar Ataxia and Nystagmus.

Pharmacological treatment of cerebellar ataxias and cerebellar nystagmus still remains difficult. The efficacy of most of the agents recommended in the past for symptomatic or even causative therapy could not be proven in larger state-of-the art clinical trials. Exceptions are (a) 4-aminopyridine (4-AP) for episodic ataxia type 2 (EA2): one observational and one randomized controlled trial showed a significant effect on the number of attacks of ataxia and quality of life; (b) aminopyridines in cerebellar downbeat nystagmus (DBN): two randomized controlled trials and several observational studies demonstrate a significant improvement of the intensity of DBN, visual acuity, and postural imbalance.

Acetyl-dl-leucine in Niemann-Pick type C: A case series.

Objective: To assess the effects of the modified amino acid acetyl-dl-leucine (AL) on cerebellar ataxia, eye movements, and quality of life of patients with Niemann-Pick type C (NP-C) disease.

Methods: Twelve patients with NP-C disease were treated with AL 3 g/d for 1 week and then with 5 g/d for 3 weeks with a subsequent washout period of 1 month. The Scale for the Assessment and Rating of Ataxia (SARA), the Spinocerebellar Ataxia Functional Index (SCAFI), the modified Disability Rating Scale (mDRS), EuroQol 5Q-5D-5L, and the visual analog scale (VAS) were administered.

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.

Pharmacotherapy of vestibular and cerebellar disorders and downbeat nystagmus: translational and back-translational research.

There are currently eight groups of drugs for the pharmacotherapy of vertigo, nystagmus, and cerebellar disorders: antiemetics; anti-inflammatories, antimenieres, and antimigraineous medications; antidepressants, anticonvulsants, aminopyridines, and acetyl-DL-leucine ("the eight A's"). In acute unilateral vestibulopathy, corticosteroids improve the recovery of peripheral vestibular function, but there is not sufficient current evidence for a general recommendation. There is also insufficient evidence that 48 or 144 mg/day betahistine has an effect in Ménière's disease.

Central ocular motor disorders, including gaze palsy and nystagmus.

An impairment of eye movements, or nystagmus, is seen in many diseases of the central nervous system, in particular those affecting the brainstem and cerebellum, as well as in those of the vestibular system. The key to diagnosis is a systematic clinical examination of the different types of eye movements, including: eye position, range of eye movements, smooth pursuit, saccades, gaze-holding function and optokinetic nystagmus, as well as testing for the different types of nystagmus (e.g.

Ocular VEMPs indicate repositioning of otoconia to the utricle after successful liberatory maneuvers in benign paroxysmal positioning vertigo.

Conclusions: This study showed a transient increase of ocular vestibular evoked myogenic potential (oVEMP) amplitudes in the affected ear after successful liberatory maneuvers and no changes in cervical VEMP (cVEMP) amplitudes. These findings support the hypothesis that successful liberatory maneuvers can lead to a repositioning of otoconia to the utricle.

Objectives: To evaluate whether oVEMP amplitudes increase after successful liberatory maneuvers in patients with posterior semicircular canal benign paroxysmal positioning vertigo (pc-BPPV), while cVEMP amplitudes do not change.

Clinical testing of otolith function: perceptual thresholds and myogenic potentials.

Cervical and ocular vestibular-evoked myogenic potential (cVEMP/oVEMP) tests are widely used clinical tests of otolith function. However, VEMP testing may not be the ideal measure of otolith function given the significant inter-individual variability in responses and given that the stimuli used to elicit VEMPs are not physiological. We therefore evaluated linear motion perceptual threshold testing compared with cVEMP and oVEMP testing as measures of saccular and utricular function, respectively.

[Cardinal symptom vertigo from the neurologist's perspective].

In most patients with vertigo, the first and clinically most important question posed to neurologists is whether it is a central or a peripheral syndrome. In more than 90 % of cases, this differentiation is made possible by systematically recording the patient history (asking about the type of vertigo, the duration, triggers and accompanying symptoms) and conducting a physical examination. Particularly in the case of acute vertigo disorders, a five-step procedure has proven useful: 1.

Semicircular canal, saccular and utricular function in patients with bilateral vestibulopathy: analysis based on etiology.

The diagnosis of bilateral vestibulopathy (BV) is typically established based on bilateral semicircular canal dysfunction. The degree to which both otolith organs-the saccule and utricle-are also impaired in BV is not well-established, particularly with respect to the etiology and severity of BV. The aim of this study was to evaluate semicircular canal, saccular and utricular function in patients with BV due to aminoglycoside ototoxicity and bilateral Menière's disease, and with different severities of BV.