This paper describes the diagnostic criteria for bilateral vestibulopathy (BVP) by the Classification Committee of the Bárány Society. The diagnosis of BVP is based on the patient history, bedside examination and laboratory evaluation. Bilateral vestibulopathy is a chronic vestibular syndrome which is characterized by unsteadiness when walking or standing, which worsen in darkness and/or on uneven ground, or during head motion. Additionally, patients may describe head or body movement-induced blurred vision or oscillopsia. There are typically no symptoms while sitting or lying down under static conditions.The diagnosis of BVP requires bilaterally significantly impaired or absent function of the vestibulo-ocular reflex (VOR). This can be diagnosed for the high frequency range of the angular VOR by the head impulse test (HIT), the video-HIT (vHIT) and the scleral coil technique and for the low frequency range by caloric testing. The moderate range can be examined by the sinusoidal or step profile rotational chair test.For the diagnosis of BVP, the horizontal angular VOR gain on both sides should be <0.6 (angular velocity 150-300°/s) and/or the sum of the maximal peak velocities of the slow phase caloric-induced nystagmus for stimulation with warm and cold water on each side <6°/s and/or the horizontal angular VOR gain <0.1 upon sinusoidal stimulation on a rotatory chair (0.1 Hz, Vmax = 50°/sec) and/or a phase lead >68 degrees (time constant of <5 seconds). For the diagnosis of probable BVP the above mentioned symptoms and a bilaterally pathological bedside HIT are required.Complementary tests that may be used but are currently not included in the definition are: a) dynamic visual acuity (a decrease of ≥0.2 logMAR is considered pathological); b) Romberg (indicating a sensory deficit of the vestibular or somatosensory system and therefore not specific); and c) abnormal cervical and ocular vestibular-evoked myogenic potentials for otolith function.At present the scientific basis for further subdivisions into subtypes of BVP is not sufficient to put forward reliable or clinically meaningful definitions. Depending on the affected anatomical structure and frequency range, different subtypes may be better identified in the future: impaired canal function in the low- or high-frequency VOR range only and/or impaired otolith function only; the latter is evidently very rare.Bilateral vestibulopathy is a clinical syndrome and, if known, the etiology (e.g., due to ototoxicity, bilateral Menière's disease, bilateral vestibular schwannoma) should be added to the diagnosis. Synonyms include bilateral vestibular failure, deficiency, areflexia, hypofunction and loss.

The brain integrates sensory input from the otolith organs, the semicircular canals, and the somatosensory and visual systems to determine self-orientation relative to gravity. Only the otoliths directly sense the gravito-inertial force vector and therefore provide the major input for perceiving static head-roll relative to gravity, as measured by the subjective visual vertical (SVV). Intraindividual SVV variability increases with head roll, which suggests that the effectiveness of the otolith signal is roll-angle dependent. We asked whether SVV variability reflects the spatial distribution of the otolithic sensors and the otolith-derived acceleration estimate. Subjects were placed in different roll orientations (0-360 degrees, 15 degrees steps) and asked to align an arrow with perceived vertical. Variability was minimal in upright, increased with head-roll peaking around 120-135 degrees, and decreased to intermediate values at 180 degrees. Otolith-dependent variability was modeled by taking into consideration the nonuniform distribution of the otolith afferents and their nonlinear firing rate. The otolith-derived estimate was combined with an internal bias shifting the estimated gravity-vector toward the body-longitudinal. Assuming an efficient otolith estimator at all roll angles, peak variability of the model matched our data; however, modeled variability in upside-down and upright positions was very similar, which is at odds with our findings. By decreasing the effectiveness of the otolith estimator with increasing roll, simulated variability matched our experimental findings better. We suggest that modulations of SVV precision in the roll plane are related to the properties of the otolith sensors and to central computational mechanisms that are not optimally tuned for roll-angles distant from upright.

When qHIT is used as a reference, bHIT sensitivity is adequate and therefore clinically useful in the hands of both neuro-otological experts and non-experts. We advise performing quantitative head impulse testing with search coils or high speed video methods when bHIT is not conclusive.

ABSTRACToften becomes symmetrical, leading to a normal canal paresis factor. The objective of this study was to compare results of

Abstract. This paper describes the diagnostic criteria for vestibular paroxysmia (VP) as defined by the Classification Committee of the Bárány Society. The diagnosis of VP is mainly based on the patient history and requires: A) at least ten attacks of spontaneous spinning or non-spinning vertigo; B) duration less than 1 minute; C) stereotyped phenomenology in a particular patient; D) response to a treatment with carbamazepine/oxcarbazepine; and F) not better accounted for by another diagnosis. Probable VP is defined as follows: A) at least five attacks of spinning or non-spinning vertigo; B) duration less than 5 minutes; C) spontaneous occurrence or provoked by certain head-movements; D) stereotyped phenomenology in a particular patient; E) not better accounted for by another diagnosis.Ephaptic discharges in the proximal part of the 8th cranial nerve, which is covered by oligodendrocytes, are the assumed mechanism. Important differential diagnoses are Menière's disease, vestibular migraine, benign paroxysmal positional vertigo, epileptic vestibular aura, paroxysmal brainstem attacks (in multiple sclerosis or after brainstem stroke), superior canal dehiscence syndrome, perilymph fistula, transient ischemic attacks and panic attacks. Current areas of uncertainty in the diagnosis of VP are: a) MRI findings of vascular compression which are not diagnostic of the disease or predictive for the affected side because they are also observed in about 30% of healthy asymptomatic subjects; and b) response to treatment with carbamazepine/oxcarbazepine supports the diagnosis but there are so far no randomized controlled trials for treatment of VP.

Downbeat nystagmus is a frequent ocular motor sign in patients with lesions of the vestibulocerebellum. The upward drift in downbeat nystagmus is a combination of a gaze-evoked drift, due to an impaired vertical neural integrator, and a velocity bias. Using a three-dimensional turntable, we analyzed the influence of gravity on these two mechanisms. Patients with cerebellar downbeat nystagmus (n = 6) and healthy subjects (n = 12) were placed in various whole-body positions along the roll, pitch, and oblique vertical planes of the head. Ocular drift was monitored with scleral search coils. Although there was no gravity dependence of the vertical gaze-evoked drift, the vertical velocity bias consisted of two components: a gravity-dependent component that sinusoidally modulated as a function of body position along the pitch plane, and a gravity-independent component that was directed upward. The combination of the two components led to an overall drift that was minimal in supine and maximal in prone position. In healthy subjects, only the gravity-dependent component was present, but in a scaled-down manner. Our results suggest that the intact vestibulocerebellum minimizes an overacting otolith-ocular reflex elicited by pitch tilt and cancels an inherent upward ocular drift that is independent of gravity-modulated otolith signals.

Objective: To translate the Dizziness Handicap Inventory into German (DHI-G) and investigate reliability, assess the association between selected items of the University of California Los Angeles Dizziness Questionnaire (UCLA-DQ) and the DHI-G and compare the scores of patients and healthy participants.Design: Cross-sectional design.Setting: Tertiary centre for vertigo, dizziness or balance disorders. Subjects:One-hundred forty-one patients with vertigo, dizziness and unsteadiness associated with a vestibular disorder; mean age 51.5 (13.2) and fifty-two healthy individuals participated.Interventions: Fourteen patients participated in the cognitive debriefing; onehundred twenty-seven patients completed the questionnaires once or twice within one week. Conclusions:The DHI-G demonstrated good reliability and is recommended as a measure of disability in patients with dizziness and unsteadiness.Dizziness Handicap Inventory -German version 2

Objective: To identify under-diagnosed neuro-otological disorders and to evaluate whether under-diagnosing depends on the age of the patient. Materials and methods: Retrospective analysis of medical charts from 951 consecutive patients (685 under and 266 above the age of 65 years) who entered diagnostic procedures at the Interdisciplinary Center for Vertigo and Balance Disorders, University Hospital Zurich, Switzerland. Final diagnoses were compared to referral diagnoses. Results: Relative to referral diagnoses, the proportion of patients finally diagnosed with benign paroxysmal positional vertigo (BPPV) almost doubled both in younger (<65 year from 12.7 to 25.1%) and older patients (from 20.7 to 37.6%). Striking relative increases were found for the diagnoses multisensory dizziness in older patients (from 20.7 to 37.6%) and vestibular migraine in younger patients (1.8 to 20.2%). In both age groups, the proportion of patients with undetermined diagnoses was reduced by about 60% (younger: 69.8 to 9.8%; older: 69.2 to 12.4%) by the diagnostic procedures in the vertigo center. These changes were all significant (p < 0.05) in McNemar tests with continuity correction (2 × 2 tables: focused diagnosis vs. other diagnoses, referral vs. final). Conclusion: Significant changes of diagnoses can be expected by a specialized neuro-otological work-up. In particular, BPPV, multisensory dizziness, and vestibular migraine are under-diagnosed by referring physicians. This finding calls for better education of primary care takers in the field of neuro-otology.