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After unilateral peripheral vestibular lesions, the neural activity of neurons in the ipsi-lesional medial vestibular nucleus (ipsi-MVe) are markedly decreased, resulting in static and dynamic asymmetries of the vestibulo-ocular and vestibulo-spinal reflexes. Consequently, static vestibular symptoms such as spontaneous nystagmus and postural deviation and dynamic vestibular symptoms such as oscillopsia and swaying gait are induced. However, these behavioral asymmetries gradually recover after the lesion. Progressive balance restoration is termed vestibular compensation, which is divided into two phases: static and dynamic. Static vestibular compensation is further divided into initial and late processes. In the initial process of static vestibular compensation after unilateral labyrinthectomy (UL) in rats, plastic changes in the cerebello-vestibular and vestibular commissural inhibitory pathways suppress neurons in the contra-lesional MVe (contra-MVe), resulting in the restoration of symmetrical resting activity of MVe neurons on both sides at low levels. The declining frequency of spontaneous nystagmus after UL is an index of the initial process, and short-term administration of diazepam, a GABA A receptor agonist, has been shown to accelerate the initial process in rats. Accordingly, short-term administration of diazepam is recommended for the treatment of acute vertigo in patients with unilateral vestibular dysfunction. In the late process of static vestibular compensation after UL in rats, the resting activity of ipsi-MVe neurons gradually recovers due to changes in cell membrane properties, resulting in the reinforcement of balanced intervestibular nuclear activities to nearly normal levels without the suppression of contra-MVe neurons. The declining number of MK801-induced Fos-positive neurons in contra-MVe after UL is an index of the late process, and long-term administration of betahistine, a histamine H 3 receptor antagonist, has been shown to accelerate the late process in rats. Accordingly, long-term administration of betahistine is recommended for the treatment of subacute vertigo in patients who were not compensated for unilateral vestibular dysfunction. In the process of dynamic vestibular compensation after UL, the sensitivity of ipsi-MVe neurons to head velocity and acceleration is restored due to synaptic changes such as long-term potentiation and sprouting of commissures, resulting in the restoration of the dynamic vestibulo-ocular and vestibulo-spinal reflexes. To facilitate dynamic vestibular compensation, early ambulation and subsequent vestibular rehabilitation exercise are recommended for the treatment of chronic vertigo in patients with uncompensated unilateral vestibular dysfunction. Although vestibular compensation after bilateral vestibular loss is not expected, vestibular rehabilitation with a sensory-substitution strategy can improve imbalance in patients with bilateral vestibular lesions. [ABSTRACT FROM AUTHOR]

The aim was to evaluate the vestibular system of children who had undergone cochlear implant surgery and to compare them with the healthy population by vestibulo-ocular reflex (VOR) gains in unilateral and bilateral implant users, with the implants both on and off. Patients older than 5 years who had undergone cochlear implant surgery between 2012 and 2020 and who were cochlear implant users for at least one year were included. After consent was obtained, a video head impulse test (VHIT) was performed to evaluate the three semicircular canals, with devices on and off, and VOR gains were evaluated. VHIT was also used to assess VOR gains in the control group. The VOR gains of the study and control groups, VOR gains of unilateral and bilateral implant users, and VOR gains with implants on and off were compared. When the VOR gains of 24 unilateral and 13 bilateral cochlear implant users and the control group (n = 30) were compared, a significant difference was found only in the anterior semicircular canal, although the VOR gains were found to be low in all three semicircular canals in the implant users (p < 0.05). There was no significant difference between the VOR gains of unilateral and bilateral implant users. There was no significant difference between the VOR gains when either on or off. There was no correlation between cochlear implant usage time, implant insertion age, patient age and VOR gain. The effects of cochlear implant surgery on the vestibular system continue in the late period, but no correlation was observed between implant usage time and VOR gain after the first year. It was observed that having the cochlear implant on or off had no effect on VOR gain. Furthermore, bilateral implant surgery did not lead to additional vestibular dysfunction compared to unilateral implant surgery. [ABSTRACT FROM AUTHOR]

Objective: To evaluate how often the positive sign of HINTS (Head-Impulse, Gaze Evoked Nystagmus, Test of Skew) appears in patients with acute peripheral vestibular lesion, HINTS findings were quantitatively measured and analyzed in patients with peripheral vestibulopathy accompanying spontaneous nystagmus.Methods: HINTS was evaluated in 14 vertigo patients with spontaneous nystagmus. Horizontal vestibulo-ocular reflex (VOR) gain was measured using the video head impulse test (vHIT). To evaluate gaze-evoked nystagmus (GEN), slow-phase velocities at different points of lateral gaze were measured and plotted, then the slope and its inverse value, the neural integrator time constant, were calculated. Skew deviation was tested using anaglyph filters to simulate the alternate cover test, and the degree and latency of vertical eyeball deviation were measured. The ABCD2 score was calculated to evaluate the risk of stroke.Results: Among 13 patients of peripheral vestibulopathy, 7 showed positive signs in HINTS (normal vHIT: 5, direction-changing GEN: 0, skew deviation: 3). One patient with a cerebellopontine angle tumor presented with both a peripheral and central pattern and showed positive HINTS findings (presence of direction-changing GEN). The mean VOR gain of patients with abnormal vHIT was 0.58±0.29 and 1.10±0.11 in the affected and contralateral side, respectively, while those in patients with normal vHIT were 1.04±0.21 and 1.13±0.12, respectively. The neural integrator time constant calculated from the mean slope of horizontal slow-phase velocity according to horizontal eye position was 42.9 s. The mean vertical eyeball deviation of patients with positive skew was 2.14±1.18° while uncovering the eye on the affected side, and -1.97±1.59° while uncovering the eye on the unaffected side. The median ABCD2 score of 14 patients was 2 (range, 1-3).Conclusions: HINTS findings were objectively measured in vertigo patients with spontaneous nystagmus. Although positive findings of HINTS have been recognized as a central sign, 54% (7/13) of cases with peripheral vestibulopathy showed positive HINTS signs. HINTS results should be interpreted carefully considering that a substantial proportion of peripheral vestibulopathy shows a positive HINTS sign. [ABSTRACT FROM AUTHOR]

To identify the etiology of vertigo/dizziness and determine the effectiveness of the video-head impulse test (vHIT) and the suppression head impulse paradigm (SHIMP) tests in distinguishing between peripheral and non-peripheral etiologies in children who presented to the otolaryngology department with complaints of vertigo/dizziness. The vHIT and SHIMP tests were applied to the children. The vestibulo-ocular reflex (VOR) gain and saccade parameters were compared. In 27 children presenting with vertigo/dizziness, the most common etiological factor was inner ear malformation (IEM) (n = 6/27, 22.2%), followed by cochlear implant surgery (11.1%) and migraine (11.1%). Vestibular hypofunction was indicated by the vHIT results at a rate of 60% (9/15 children) and SHIMP results at 73.3% (11/15 children) among the children with a peripheral etiology, while these rates were 8.3% (1/12 children) and 25% (3/12 children), respectively, in the non-peripheral etiology group. SHIMP-VOR and vHIT-VOR gain values had a moderate positive correlation (p = 0.01, r = 0.349). While there were overt/covert saccades in the vHIT, anti-compensatory saccade (ACSs) were not observed in the SHIMP test (p = 0.041). The rates of abnormal vHIT-VOR gain (p = 0.001), over/covert saccades (p = 0.019), abnormal vHIT response (p = 0.014), ACSs (p = 0.001), and abnormal SHIMP response (p = 0.035) were significantly higher in the peripheral etiology group. IEM was the most common etiological cause, and the rate of vestibular hypofunction was higher in these children with peripheral vertigo. vHIT and SHIMP are effective and useful vestibular tests for distinguishing peripheral etiology from non-peripheral etiology in the pediatric population with vertigo/dizziness. These tests can be used together or alone, but the first choice should be the SHIMP test, considering its short application time (approximately 4–5 min) and simplicity. [ABSTRACT FROM AUTHOR]

Objective: In this retrospective study the aim of the authors was to examine the effect of gentamicin on the individual semicircular canals after low dose, single injection intratympanal gentamicin therapy in Meniere's disease.Methods: Data of 32 patients treated between 2011 and 2015 were collected. The high frequency, high acceleration vestibuloocular reflex (VOR) gain was measured in the individual semicircular canals using video head impulse test immediately before the first intratympanal gentamicin instillation and approximately two months later.Results: In all cases 'AAO-HNS Class A' vertigo control could be attained at least for several months. In 13 cases only one instillation was necessary. In the other 19 cases the attacks returned after a few months. In 11 cases the injection had to be repeated a second time, in 4 cases 3 injections, in 2 cases 4, in 1 case 5 injections and in another 6 injections were necessary. The initial VOR gain was normal in all cases and two months after one injection it decreased in average by 40% in a highly significant manner. However, there were cases in which, although the patients became free of attacks, the gain values remained normal.Conclusion: It was possible to demonstrate a significant correlation between the gain decrease of the individual canals. There was no prognostic correlation between the initial gain decrease after the first injection and the necessity of further injections. Gain values also decreased slightly but significantly in the lateral and posteriors canals on the contralateral, untreated side, possibly because of the missing disfacilitation from the treated side. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the lateralization value of video head-impulse test (vHIT) for the diagnosis of vestibulopathy and to analyze cases showing dissociated results with caloric test.Methods: In total, 19 healthy volunteers and 92 dizzy patients who underwent both a caloric test and a vHIT were enrolled. Patients were divided into two groups depending on their fluctuating pattern of vertigo. The vestibulo-ocular reflex (VOR) gain and gain asymmetry (GA) of a vHIT as well as unilateral weakness (UW) and the sum of the slow-phase velocities (SPVs) of warm and cold irrigation of the same side were compared. A cutoff value of VOR gain of a vHIT was also calculated using a receiver-operating characteristic curve.Results: A VOR gain in an affected ear and GA of a vHIT showed a statistically significant correlation with UW in a caloric test. The cutoff value of a vHIT was determined to be 0.875, derived under the assumption that UW of a caloric test ≤25% is normal. However, the parameters of the two tests were dissociated in 18%.Conclusion: A VOR gain of vHIT is a valuable objective parameter with a lateralization value determining vestibular hypofunction. However, considering substantial dissociation between a vHIT and a caloric test, these tests can be complementary tools for the lateralization of vestibular impairment for the comprehensive evaluation of patients' VOR. [ABSTRACT FROM AUTHOR]

Objective It has long been suggested that antivertiginous medications exert their symptomatic effect through inhibition of the vestibulo-ocular reflex (VOR). We tested this hypothesis by directly measuring the VOR after administration of three agents from different substance classes: an antihistamine, a benzodiazepine and a calcium channel antagonist. Methods The gain and the variability of the high velocity VOR was assessed using video head impulses (vHIT) under the following conditions: baseline, after dimenhydrinate, after diazepam and after cinnarizine. Results We found that all three medications did not change any VOR gain or variability parameter: At 60 ms, the gain was 0.95 at baseline, 0.99 under dimenhydrinate, 0.99 under diazepam and 0.96 under cinnarizine. The gain variability across repetitive head impulses remained also uninfluenced. Conclusions The human high frequency VOR remains robust to pharmacological perturbations at common clinical doses and the assumption that symptomatic vertigo relief is achieved merely through impairment of the VOR requires re-examination. Significance Alternative mechanisms of pharmacological action might be operant, such as the modulation of vestibulo-cortical pathways, a differential effect on the low frequency VOR and an altered sensitivity to drugs in acute unilateral vestibulopathy. [ABSTRACT FROM AUTHOR]

Objective The caloric test and the video head-impulse test are diagnostic tools to examine dizzy patients through assessing the function of the semicircular canals. There are major differences between the two tests as regards stimulus characteristics, methodology, and function examined. The aim of this study is to evaluate the results of vHIT in children and adolescents with normal caloric test. Materials and methods This work was performed on 63 patients, but 14 were excluded because of technical problems in the caloric test. So, this is a prospective work in 49 patients (27 females and 22 males) with different types of vestibular disease seen because of vertigo in which both procedures were performed the same day. The caloric test was performed with air at two different temperatures in which both ears were irrigated alternately. Then, the video head-impulse test was carried out. Main outcome measures were the gain of vestibulo-ocular reflex, gain asymmetry, and refixation saccades in the vHIT. Results in all studied cases, caloric test was normal. The mean age of patients was 16 years. By vHIT, in 8 patients (16%) no abnormality was detected, while abnormal findings were found in 41 patients. Single canal affection was seen in 29 patients whereas 12 patients had combined canal affection. The right side was affected in 27 and left side in 22 patients. In single canal affection, isolated horizontal canals were affected in 4, anterior canals in 5 and posterior canals in 20 patients. While in combined canal affection, the affection is seen in the same ear. Moreover the most common pattern seen is affection of left anterior and left posterior canals. Conclusion The caloric and vHIT is very important tests in diagnosis of dizzy patients. The information from both methods is redundant in some cases but complementary in most. vHIT is a “child friendly,” relatively easy-to-use, and simple tool to evaluate each of the 6 semicircular canals. [ABSTRACT FROM AUTHOR]

The video head impulse test (vHIT) assesses the vestibulo-ocular reflex (VOR) during a rapid high-velocity low amplitude (10°–20°) head rotation. Patients with peripheral vestibulopathy have a reduced VOR gain with corrective catch-up saccades during the head turn. There are several pitfalls, mainly technical, which may interfere with interpretation of vHIT data. In addition, intrusive eye movement disorders such as spontaneous nystagmus that affect normal eye position and tracking can affect the vHIT results. To date there has been little study of neurological saccadic eye movements that may interfere with the interpretation of vHIT data. Here, in ten patients with a range of central neurological disorders, we describe oculomotor abnormalities on vHIT in the presence of normal range VOR gain values, recorded at a tertiary vestibular neurology service. • Certain apparent catch-up saccades visible on the vHIT data may be due to the gaze-evoked nystagmus, induced by the head turn and transient eccentric positioning of the eyes. • Saccadic oscillations may affect the vHIT by appearing immediately after the head impulse and may be indistinguishable from an overt saccade if seen without clinical correlation. • Our cases highlight that oculomotor abnormalities can interfere with quantitative recording of the head impulse VOR responses and may impact the clinical diagnosis. [ABSTRACT FROM AUTHOR]

Benign Paroxysmal Positional Vertigo (BPPV) is among the most common vestibular disorders, characterized by brief vertigo spells triggered by head position changes with abrupt onset and rapid decrease. BPPV is ascribed to otoconial matter dislodged from utricular macula and attached to the cupula of the affected semicircular canal (cupulolithiasis) or free-floating within its lumen (canalolithiasis). According to the vestibulo-ocular reflex pathophysiology, each cupular deflection, either exciting or inhibiting the corresponding ampullary afferents, generates the contraction of specific extraocular muscles couples leading to pathognomonic nystagmus. The Upright BPPV Protocol (UBP) is a diagnostic approach to BPPV conducted in the sitting position slowly bending the patient's head along the spatial axes, aiming to move canaliths by gravity within the involved semicircular canal, under continuous nystagmus monitoring by video-Frenzel goggles. UBP starts with the evaluation of pseudo-spontaneous nystagmus in the primary gaze position and continues with the upright Head Pitch Test (uHPT) by forward and backward head bendings along the pitch plane. The uHPT can indicate whether horizontal or vertical semicircular canal is involved. If horizontal canal is suspected, the upright Head Roll Test (uHRT) usually provides the diagnosis of the involved side and arm by tilting the patient's head rightward and leftward along the roll plane. Conversely, canalolithiasis involving the posterior semicircular canal can be diagnosed with the uHPT alone. Nevertheless, if necessary, the diagnostic sensitivity can be increased by head movements along the right anterior – left posterior (RALP) and left anterior – right posterior (LARP) canal planes (uRALP/uLARP test). Following the UBP, most BPPV form can be diagnosed in upright position, allowing clinicians to proceed immediately with proper physical treatment and avoiding unpleasant maneuvers to patients. • BPPV diagnosis should be based on the step-by-step observation of the evoked nystagmus. • The Minimum Stimulus Strategy aims to minimize the patient's discomfort. • The Upright BPPV Protocol is performed in the sitting position through head movements along the spatial planes. • The sensitivity of the head pitch test for horizontal canal-BPPV is increased by the upright head roll test. • The sensitivity of the head pitch test for posterior canal-BPPV is increased by the upright RALP/LARP test. [ABSTRACT FROM AUTHOR]

To evaluate the clinical and genetic features of children with hearing loss associated with one of the most common malformations of the inner ear: bilateral enlargement of vestibular aqueducts (EVA). Clinical and genetic features were investigated in 28 children with hearing loss diagnosed with bilateral EVA by computed tomography from January 2008 to September 2019. Fourteen subjects had undergone newborn hearing screening (NHS). Nine subjects (64.3%) were referred in both ears, 4 subjects (28.6%) were referred in one ear, and one subject (7.1%) passed in both ears. Nineteen of 26 subjects (73.1%) who were followed for more than 3 years had hearing fluctuations, while 17 (65.4%) had hearing loss progression. Eleven of 28 subjects (39.2%) had vertigo attacks. Pathogenic variants were identified in two alleles of the SLC26A4 gene in 24 of 27 subjects (88.9%) by sequencing of all exons and flanking introns, leading to genetic diagnosis of Pendred syndrome/DFNB4. Our results indicate that genetic screening for specific SLC26A4 variants using a commercial clinical laboratory test in Japan would have achieved genetic diagnoses in 13 of the 27 subjects (54.2%). Although there was no statistically significance in the frequency of hearing fluctuation or progression depending on the presence or absence of the gene variant, mean hearing level was severe in subjects with two pathogenic variants in SLC26A4 gene. The most common variant detected in our subjects was p.His723Arg (13 alleles, 27.1%), followed by c. 919-2A > G (four alleles, 8.3%). Two novel variants were detected in this study: c.1544+1G > T and c.1614+5G > A. Our data suggest that some subjects may present with bilateral EVA that cannot be detected by NHS. We estimated that genetic diagnosis for SLC264 gene would not have been made in almost half subjects with the commercial genetic screening approach used in the present study in Japan. Although there were some limitations in this study, the subjects with pathogenic variants in two alleles of the SLC26A4 gene could have more severe hearing loss. [ABSTRACT FROM AUTHOR]

Acute vestibular syndrome is most often caused by vestibular neuritis or stroke, although demyelinating diseases may be responsible for 4% of all AVS episodes. The authors present the case of a previously healthy 17-year-old female patient complaining of spontaneous vertigo and right-sided hearing loss. Otoneurological examination suggested a peripheral vestibular cause and video head impulse test revealed a reduced vestibulo-ocular reflex gain. The presence of sensorineural hearing loss raised the suspicion of a central cause and prompted imaging evaluation. A brain MRI evidenced demyelinating lesions in the right middle cerebellar peduncle and the patient was ultimately diagnosed with Multiple Sclerosis. [ABSTRACT FROM AUTHOR]

Aim To investigate the natural course of progression of ocular motor signs in patients with progressive supranuclear palsy (PSP). Methods 114 patients with a possible or probable PSP following NINDS-SPSP criteria seen at the Department of Neurology and the German Center for Vertigo and Balance Disorders (LMU, Munich) were included in this retrospective study. All patients underwent a structured neuro-ophthalmological testing by a orthoptist at initial diagnostic evaluation, 35 patients several times during their course of disease. The following ocular motor signs were extracted from the charts: Saccadic slowing (with normal range of movement) and saccadic paresis (loss of saccade initiation or severe saccadic hypometria) in vertical and horizontal direction, range of up- and downward as well as horizontal ocular motility during smooth pursuit in mm, presence of square wave jerks, function of the vestibulo-ocular reflex on head impulse testing. Clinical ocular motor signs were analysed in all patients in relation to the duration of disease at the time of examination (based on the medical history of the first sign indicative for PSP) or longitudinally during the course of disease in a subgroup of 35 patients. Results In the whole group of PSP patients saccadic abnormalities showed the following distribution over time: at 1 year of disease duration 10% showed only saccadic slowing on upgaze, 40% on up- and downgaze, 21% saccadic paresis on upgaze and 29% complete vertical saccade paresis; at 2 years the proportion of vertical saccade paresis was 32%, at 3 years 65% and at 4 years 82%. Progression of horizontal saccade paresis was slower (year 1: 6%, year 2: 9%, year 3: 21%, year 4: 41%). The subgroup of patients with longitudinal follow up showed a similar tendency. Ocular motility in this group decrease by a mean of 1.5 mm/a on upgaze, 1.6 mm/a on downgaze and 1.4 mm/a on horizontal gaze. The degree of motility loss on up- and downgaze over time showed a good correlation (R2 = 0.71). Conclusion Ocular motor examination can be used as a robust marker of disease progression. Variability between patients however is considerable. Prospective clinical and apparative quantification of ocular motor markers in well-characterized cohorts of PSP patients is needed. [ABSTRACT FROM AUTHOR]