Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS): a family with five affected sibs from Turkey.

BACKGROUND: Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS), a relatively common cause of late-onset progressive ataxia, is a genetic disease characterised by biallelic pentanucleotide AAGGG repeat expansion in intron 2 of the replication factor complex subunit 1 gene. Herein, we describe the first molecularly confirmed CANVAS family with five affected siblings from Turkey. CASE PRESENTATION: The family comprised seven siblings born from healthy non-consanguineous parents. CANVAS phenotype was present in five of them; two were healthy and asymptomatic. Chronic cough was the first symptom reported in all five siblings, followed by the development of sensory symptoms, oscillopsia and imbalance. Clinical head impulse test (HIT) was positive in all cases and video HIT performed on three patients revealed very low vestibulo-ocular reflex gains bilaterally. Magnetic resonance imaging and nerve conduction studies revealed cerebellar atrophy and sensory neuronopathy, respectively. RP-PCR confirmed the homozygous presence of the AAGGG repeat expansion in all five cases. CONCLUSION: Genetic screening for CANVAS should be considered in all patients with late-onset ataxia, sensory disturbances and vestibular involvement, especially in the presence of chronic cough.

Effects of intermittent seating upright, lower body negative pressure, and exercise on functional tasks performance after head-down tilt bed rest.

Introduction: Bed rest can be used as a ground-based analog of the body unloading associated with spaceflight. In this study, we determined how strict head-down tilt bed rest affects subjects' performance of functional tests (sit-to-stand, tandem walk, walk-and-turn, dynamic posturography) that challenge astronauts' balance control systems immediately after they return from space. Methods: Forty-seven participants were assessed before and a few hours after 30 days of 6° head down tilt bed rest at the DLR:envihab facility. During this bed rest study, called SANS-CM, the participants were divided into 4 groups that either a) were positioned in head-down tilt continuously throughout the 30 days; b) sat upright for 6 h a day; c) were exposed to lower body negative pressure (LBNP) for 6 h a day; or d) exercised for 60 min and then wore venous-occlusive cuffs for 6 h a day. Results: Results showed that strict head-down tilt bed rest caused deficits in performance of functional tasks that were similar to those observed in astronauts after spaceflight. Seated upright posture mitigated these deficits, whereas exercise or LBNP and cuffs partly mitigated them. Discussion: These data suggest that more direct, active sensorimotor-based countermeasures may be necessary to maintain preflight levels of functional performance after a long period of body unloading.

[Acupuncture at Niwanneibazhen points combined with vestibular rehabilitation for unilateral peripheral vestibular dysfunction: a randomized controlled trial].

OBJECTIVE: To observe the clinical effect on unilateral peripheral vestibular dysfunction treated with acupuncture at Niwanneibazhen points combined with vestibular rehabilitation. METHODS: A total of 84 patients with unilateral peripheral vestibular dysfunction were randomly divided into a trial group (42 cases, 2 cases dropped out) and a control group (42 cases, 1 case dropped out). In the control group, vestibular rehabilitation was given. In the trial group, based on the treatment in the control group, Niwanneibazhen points were located. Taking Baihui (GV 20) as the center, three equal section was divided on the line from Baihui (GV 20) to Yintang (GV 24+). A circle was drawn with the radius from the inner 1/3 equal-section point to Baihui (GV 20), and divided into eight equal parts. Acupuncture was delivered at Baihui (GV 20) and the eight equal-part points, with needles retained for 30 min. The intervention with acupuncture was operated once every three days, twice a week, consecutively for 4 weeks. Before and after treatment completion, using the Berg balance scale (BBS), dizziness handicap inventory (DHI), hospital anxiety and depression scale (HADS), and Pittsburgh quality index (PSQI), the balance function, the degree of vertigo, emotional conditions, and sleep quality were evaluated in the patients of the two groups, and the clinical effect was assessed. RESULTS: After 4 weeks of treatment, the BBS scores were higher than those before treatment (P<0.01), and the scores of DHI, HADS, and PSQI were lower than those before treatment (P<0.01) in the two groups. The reduction of HADS and PSQI scores in the trial group was larger than that of the control group (P<0.01). The total effective rate was 90% (36/40) in the trial group, higher than that of the control group (78.1%, 32/41, P<0.05). CONCLUSION: Acupuncture at Niwanneibazhen points combined with vestibular rehabilitation can effectively ameliorate balance function and vertigo degree, relieve the emotions of anxiety and depression, and improve the quality of sleep in the patients with unilateral peripheral vestibular dysfunction.

Validating the splicing effect of rare variants in the SLC26A4 gene using minigene assay.

BACKGROUND: The SLC26A4 gene is the second most common cause of hereditary hearing loss in human. The aim of this study was to utilize the minigene assay in order to identify pathogenic variants of SLC26A4 associated with enlarged vestibular aqueduct (EVA) and hearing loss (HL) in two patients. METHODS: The patients were subjected to multiplex PCR amplification and next-generation sequencing of common deafness genes (including GJB2, SLC26A4, and MT-RNR1), then bioinformatics analysis was performed on the sequencing data to identify candidate pathogenic variants. Minigene experiments were conducted to determine the potential impact of the variants on splicing. RESULTS: Genetic testing revealed that the first patient carried compound heterozygous variants c.[1149 + 1G > A]; [919-2 A > G] in the SLC26A4 gene, while the second patient carried compound heterozygous variants c.[2089 + 3 A > T]; [919-2 A > G] in the same gene. Minigene experiments demonstrated that both c.1149 + 1G > A and c.2089 + 3 A > T affected mRNA splicing. According to the ACMG guidelines and the recommendations of the ClinGen Hearing Loss Expert Panel for ACMG variant interpretation, these variants were classified as "likely pathogenic". CONCLUSIONS: This study identified the molecular etiology of hearing loss in two patients with EVA and elucidated the impact of rare variants on splicing, thus contributing to the mutational spectrum of pathogenic variants in the SLC26A4 gene.

Angular Trajectory of the Vestibular Aqueduct in a Cohort of Chinese Patients with Unilateral Ménière's Disease: Association with Other Imaging Indices and Clinical Profiles.

OBJECTIVE: We aimed to investigate the association between the angular trajectory of the vestibular aqueduct (ATVA) with other radiological parameters of temporal bone and clinical characteristics in patients with Ménière's disease (MD). METHODS: A total of 125 unilateral MD patients and 118 controls were enrolled. Computer tomography (CT)-based radiological parameters included ATVA, vestibular aqueduct (VA) visibility, VA morphology, the vertical part of the posterior semicircular canal-the posterior fossa distance (PPD), and peri-VA pneumatization. The clinical characteristics of MD patients included gender, age of diagnosis/onset, disease duration, migraine history, clinical staging, and the results of audio-vestibular tests. The radiological parameters and clinical characteristics in MD patients were compared. RESULTS: Compared with control ears, ATVA ≥ 140° was more prevalent and ATVA ≤ 120° was less frequent in the MD-affected side. For the MD-affected side, MD patients with ATVA ≥ 140° exhibited more severe VA invisibility and obliteration and higher male preponderance than those with ATVA ≤ 120°. Other radio-clinical features did not differ between these two subgroups. CONCLUSION: In the current study, ATVA ≥ 140°, an indicator of a hypoplastic endolymphatic sac, was found in approximately one-third of the affected and unaffected ears of patients with MD, as well as in a minority of controls. This suggests that the indices may be a predisposing factor rather than a specific marker for the MD ear. The male preponderance in MD patients with hypoplastic ES suggests a gender difference in the anatomical factors for MD pathogenesis.

Chronic Unilateral Vestibular Hypofunction: Insights into Etiologies, Clinical Subtypes, Diagnostics and Quality of Life.

Background/Objectives: Chronic unilateral vestibular hypofunction (UVH) can lead to disabling vestibular symptoms and a decrease in quality of life. The aim of this study was to investigate etiologies, clinical subtypes, symptoms, and quality of life (QoL) in patients with chronic UVH. Methods: A retrospective study was performed on 251 UVH patients in a tertiary referral center. Inclusion criteria comprised reduced or absent caloric responses, with a caloric asymmetry ratio ≥25%. Patients with central vestibular pathology, symptom duration <3 months, and incomplete responses to questionnaires were excluded. Patient records were assessed for etiologies, secondary vestibular diagnoses, clinical subtypes, and questionnaires related to QoL. Additionally, multiple linear regression analysis was performed to evaluate factors influencing QoL. Results: Thirteen different etiologies were identified, with Menière's Disease as the most prevalent (31%, n = 79). The most frequently reported secondary vestibular diagnoses were benign paroxysmal positional vertigo (BPPV) (21%, n = 54) and persistent postural perceptual dizziness (PPPD) (19%, n = 47). Five distinct clinical subtypes were identified: recurrent vertigo with UVH (47%), rapidly progressive UVH (25%), idiopathic/unknown UVH (18%), slowly progressive UVH (8%), and congenital UVH (2%). Over 80% of UVH patients experienced moderate-to-severe handicap, as indicated by the Dizziness Handicap Inventory. Approximately 20-25% of UVH patients exhibited moderate-to-severe depression and anxiety, based on the Hospital Anxiety and Depression Scale. Multiple linear regression analyses demonstrated that the presence of PPPD significantly reduced QoL in chronic UVH patients. Conclusions: Chronic UVH is a heterogeneous disorder. Secondary vestibular diagnoses like BPPV and PPPD often co-exist and can significantly impact QoL. A structured diagnostic approach and tailored interventions are crucial to address the diverse needs of UVH patients.

Efficacy of Nortriptyline and Migraine Lifestyle Modifications in Vestibular Migraine Management.

OBJECTIVE: To evaluate the effectiveness of nortriptyline regimen and migraine dietary/lifestyle modifications on dizziness and stress levels in patients diagnosed with vestibular migraine (VM). METHODS: A total of 35 patients diagnosed with definite VM based on the International Classification of Headache Disorders were included in this intervention study. Patients self-selected to receive either nortriptyline regimen alone (10-40 mg daily with biweekly escalation) (group A, n = 17) or migraine dietary/lifestyle modifications alone (group B, n = 18). Main outcome measures were dizziness severity and stress level measured by the visual analog scale (VAS). RESULTS: At 4-week post-treatment, dizziness decreased from 6.0 ± 2.5 to 4.2 ± 3.4 (p = 0.069) in group A and from 8.7 ± 1.5 to 3.6 ± 3.0 (p < 0.001) in group B. VAS for stress changed from 5.5 ± 1.3 to 5.4 ± 2.9 (p = 0.93) and from 6.9 ± 3.2 to 5.0 ± 2.7 (p = 0.025) in groups A and B, respectively. The δ values of the VAS score for dizziness were 1.8 ± 3.7 and 5.1 ± 3.1 and the δ values of the VAS score for stress were 0.06 ± 2.9 and 1.9 ± 3.3 in groups A and B, respectively. Quality of life (QOL) improved in 88% patients in group A and 94% patients in group B. CONCLUSIONS: Nortriptyline, at a maximum dose of 40 mg, effectively alleviates patient symptoms, while a migraine diet and lifestyle modifications notably reduce vertiginous symptoms and stress levels in VM patients in 4 weeks. Both interventions are equally effective in ameliorating the QOL of patients. The ideal treatment for patients would likely need to include both medication and diet/lifestyle changes.

Improvement in Body's Dynamic Adaptation during Walking with Vestibular Rehabilitation Therapy in Patients with Cerebellopontine Angle Tumor Resection.

BACKGROUND: Our study aimed to investigate the effects of vestibular rehabilitation therapy on functional gait performance in patients with balance disorders. METHODS: A total of 40 post-operative patients with balance disorders were included in the study. They were divided into two groups and participated in a vestibular rehabilitation program during their hospital stay. After discharge, the intervention group performed vestibular exercises at home, while the control group did not. Balance was assessed using the Functional Gait Assessment Scale at discharge and three months after surgery. RESULTS: The intervention group included 15 women and 5 men with an average age of 45 years, while the control group included 7 women and 13 men with an average age of 50 years. Three months after surgery, the change in Functional Gait Assessment (FGA) scores exceeded the clinically significant threshold of 5 points in 17 patients in the intervention group and 14 in the control group. There was a statistically significant difference in FGA progression between the groups (p = 0.034). After three months post-surgery, 7 patients in the intervention group experienced falls compared to 12 in the control group. CONCLUSION: Three months after surgery, we observed a significant improvement in the performance of balance tasks while walking and a lower risk of falls in the intervention group.

Vestibular Neurostimulation for Parkinson's Disease: A Novel Device-Aided Non-Invasive Therapeutic Option.

Dopaminergic replacement therapy remains the mainstay of symptomatic treatment for Parkinson's disease (PD), but many unmet needs and gaps remain. Device-based treatments or device-aided non-oral therapies are typically used in the advanced stages of PD, ranging from stereotactic deep brain stimulation to levodopa or apomorphine infusion therapies. But there are concerns associated with these late-stage therapies due to a number of procedural, hardware, or long-term treatment-related side effects of these treatments, and their limited nonmotor benefit in PD. Therefore, there is an urgent unmet need for low-risk adjuvants or standalone therapies which can address the range of burdensome motor and nonmotor symptoms that occur in PD. Recent studies suggest that non-invasive neurostimulation of the vestibular system may be able to address these gaps through the stimulation of the vestibular brainstem sensory network which extensively innervates brain regions, regulating both motor and a range of nonmotor functions. Therapeutic non-invasive vestibular stimulation is a relatively modern concept that may potentially improve a broad range of motor and nonmotor symptoms of PD, even at early stages of the disease. Here, we review previous studies supporting the therapeutic potential of vestibular stimulation for the treatment of PD and discuss ongoing clinical trials and potential areas for future investigations.

Hearing preservation and quality of life in small to medium sized vestibular schwannomas after a wait and scan approach or stereotactic radiosurgery: a systematic review and meta-analysis.

BACKGROUND: The management of vestibular schwannomas (VS) encompasses a choice between conservative "wait-and-scan" (WAS) approach, stereotactic radiosurgery (SRS) or open microsurgical resection. Currently, there is no consensus on the optimal management approach for small to medium sized VS. This study aims to compared outcomes related to hearing in patients with small and medium sized VS who underwent initial treatment with WAS versus SRS. METHODS: A systematic review of the available literature was conducted using PubMed/MEDLINE, Embase, and Cochrane up December 08, 2023. Meta-analysis was performed using a random-effect model to calculate mean difference (MD) and relative risk (RR). A leave-one-out analysis was conducted. The risk of bias was assessed via the Risk of Bias in Non-randomized Studies-Interventions (ROBINS-I) and Cochrane Risk of Bias assessment tool (RoB-2). Ultimately, the certainty of evidence was evaluated using the GRADE assessment. The primary outcomes were serviceable hearing, and pure-tone average (PTA). The secondary outcome was the Penn Acoustic Neuroma Quality of Life Scale (PANQOL) total score. RESULTS: Nine studies were eligible for inclusion, comprising a total of 1,275 patients. Among these, 674 (52.86%) underwent WAS, while 601 patients (47.14%) received SRS. Follow-up duration ranged from two to eight years. The meta-analysis indicated that WAS had a better outcome for serviceable hearing (0.47; 95% CI: 0.32 - 0.68; p < 0.001), as well as for postoperative functional measures including PTA score (MD 13.48; 95% CI 3.83 - 23.13; p < 0.01), and PANQOL total score (MD 3.83; 95% CI 0.42 - 7.25; p = 0.03). The overall certainty of evidence ranged from "very low" to "moderate". CONCLUSIONS: Treating small to medium sized VS with WAS increases the likelihood of preserving serviceable hearing and optimized PANQOL overall postoperative score compared to SRS. Nevertheless, the limited availability of literature and the methodological weakness observed in existing studies outline the need for higher-quality studies.

A Proposal for Comprehensive Audio-Vestibular Test Battery Protocol for Diagnosis and Follow-Up Monitoring in Patients with Vestibular Schwannoma Undergoing Surgical Tumor Removal.

Background: A vestibular schwannoma (VS) is a benign tumor, causing audiological and vestibular symptoms. This study aimed to propose a comprehensive audio-vestibular test battery protocol for diagnosis and follow-up monitoring in patients with unilateral VSs undergoing surgical removal. Methods: The detailed interpretation of audiological and vestibular findings was presented in two example cases. The surgery was performed through the middle cranial fossa (#1) and translabyrinthine approach (#2). The participants were evaluated with tonal, speech, and impedance audiometry, ABR, caloric test, vHIT, cVEMP, oVEMP, SOT, and DHI. Patient and tumor characteristics were retrieved from the patient's history. Results: In the postoperative period, the reduction in gain of the lateral semicircular canal was observed in the vHITs of both patients. The DHI in case #1 increased after surgery, while it decreased in case #2. The improvement in postural performances compared to the preoperative SOT (CON 5, CON 6, composite score) and immediately after the procedure was observed. Conclusions: A specific diagnostic protocol is necessary to compare the results of different surgical techniques and approaches. Diagnostic tests performed before the surgery should be repeated within a specific time frame during postoperative follow-up to enable the comparison of results. The proposed protocol can help us better understand the processes ongoing during tumor growth and postoperative vestibular compensation.

The Impact of Tumor Elongation on Facial Nerve Outcome after Surgery for Koos Grade 3 and 4 Vestibular Schwannomas in the Semi-Sitting Position via the Retrosigmoid Approach.

Background: Facial nerve paralysis is a severe dysfunction after vestibular schwannoma (VS) surgery. Methods: This monocentric study analyzed 61 patients who underwent sporadic VS surgery in a standardized manner. The primary endpoint was the facial nerve outcome (FNO) at 3 months after VS surgery. FNO was dichotomized into "good" (House-Brackmann (HB) score ≤ 2) and "poor" (HB > 2). Results: Poor FNO was observed in 11 patients (18.0%) at 3 months after VS surgery. Radiomic tumor shape features were analyzed, and the AUC of elongation in the prediction of a poor HB at 3 months was 0.70 (95% CI: 0.56-0.85, p = 0.03) and the optimum threshold value (≤/>0.35) yielded a sensitivity and specificity of 64.0% and 75.4%, respectively. Multivariable logistic regression analyses considering the extent of resection (0.35) revealed that more elongated VSs (≤0.35; OR: 5.8; 95%CI: 1.2-28.2; p = 0.03) and those with an increased EoR (≥93.4%; OR: 6.5; 95%CI: 1.0-42.5; p = 0.05) are independently associated with poorer FNO at 3 months after surgery. Conclusions: Highly elongated VS shape seems to be a risk factor for worsened facial nerve outcome at 3 months after surgery for Koos grade 3 and 4 tumors.

Vestibular Function and Postural Control in Children with Autism Spectrum Disorder.

Background: Postural control deficits have been documented in children with autism spectrum disorder (ASD), yet vestibular system contributions to postural control have not been widely considered. The purpose of this study is to explore the relationship between functional balance, postural sway, and vestibular function in children with ASD. Methods: Ten children with a confirmed diagnosis of ASD according to DSM-V guidelines along with ten children with no known neurodevelopmental or motor delays participated in the study. Bruininks-Oseretsky Test of Motor Proficiency and the Paediatric Balance Scale measured functional balance ability, and postural sway was measured using static posturography with modified sensory inputs. Peripheral vestibular function was measured using cervical vestibular evoked myogenic potentials and video head impulse testing. Correlations between measures were performed. Results: When visual cues were removed, children with ASD demonstrated larger path velocities indicative of reduced postural control, and different patterns of postural sway. Functional balance was correlated with path velocities for conditions where sensory information was modified. No differences in peripheral vestibular function were noted between groups, and functional balance was not correlated with vestibular function. Conclusions: Findings suggest that while peripheral vestibular function is similar between groups, postural control differences in children with ASD remain, particularly for conditions where sensory information is modified. Furthermore, demonstrated patterns of postural sway suggest sensory system integration is less developed in children with ASD. These findings highlight the importance of utilising a range of clinical tools to quantify balance ability and consideration of postural control measures to inform intervention.

Distinguishing Peripheral from Central Causes of Dizziness and Vertigo without using HINTS or STANDING.

Three validated diagnostic algorithms for diagnosing patients with acute onset dizziness or vertigo (HINTS, HINTS-plus and STANDING) exist. All are extremely accurate in distinguishing peripheral from central causes of dizziness when done by experienced clinicians. However, uptake of these diagnostic tools in routine emergency medicine practice has been sub-optimal, in part, due to clinicians' unease with the head impulse test, the most useful component contained of these algorithms. Use of these validated algorithms is the best way to accurately diagnose patients with acute dizziness. For clinicians who are unfamiliar with or uncomfortable performing or interpreting HINTS and STANDING, this article will suggest alternative approaches to help with accurate diagnosis of patients with acute dizziness or vertigo.

Masseteric Vestibular Evoked Myogenic Potentials in Vestibular Neuritis: A Case Series.

Introduction: Masseteric VEMPs have been effective in evaluating a diverse spectrum of vestibular conditions associated with various brainstem pathologies, but they have rarely been explored among patients with vestibular neuritis. Case Report: The current investigation included a case series highlighting mVEMP responses in addition to cVEMPs and oVEMPs in three patients diagnosed with vestibular neuritis. In the study, all three patients were found to have absent or diminished responses in cVEMPs, oVEMPs, and mVEMPs. Conclusions: In the present study, the distinctions in cVEMP and oVEMP findings can be attributed to the involvement of inferior and superior vestibular nerve respectively. Furthermore, mVEMP presents a more intricate scenario, both in terms of its genesis and outcomes. This emphasizes the clinical relevance of mVEMP when used in combination with cVEMP and oVEMP, rather than as a substitute for the other VEMPs.

Asymmetry and rehabilitation of the subjective visual vertical in unilateral vestibular hypofunction patients.

Aims: Patients with acute unilateral peripheral vestibular hypofunction (AUVP) show postural, ocular motor, and perceptive signs on the diseased side. The subjective visual vertical (SVV) test measures the perceived bias in earth-vertical orientation with a laser line in darkness. This study was aimed at (1) examining whether SVV bias could depend on preset line orientation and angles, and (2) investigating whether vestibular rehabilitation (VR) can improve SVV normalization. To our knowledge, SVV symmetry/asymmetry and impact of VR on SVV normalization have never been documented in the literature. Participants and methods: We investigated the SVV bias in a retrospective study (Study 1: n = 42 AUVP patients) comparing the data recorded for line orientation to the ipsilateral and contralateral sides at preset angles of 15° and 30°. We investigated the effects of VR on SVV normalization in a prospective study (Study 2: n = 20 AUPV patients) in which patients were tilted in the roll plane using a support tilted to the hypofunction side with the same amplitude as the SVV bias. This VR protocol was performed twice a week for 4 weeks. Supplementary data on body weight distribution and medio-lateral position of the center of foot pressure (CoP) were obtained using posturography recordings. Results: Study 1 showed asymmetrical values of the SVV bias. On average, the SVV errors were significantly higher for ipsilateral compared to contralateral line orientation (6.98° ± 3.7° vs. 4.95° ± 3.6°; p < 0.0001), and for 30° compared to 15° preset angle (6.76° ± 4.2° vs. 5.66° ± 3.3°; p < 0.0001). Study 2 showed a fast SVV normalization with VR. Non-pathological SVV bias (below ±2°) was found after only 3 to 5 VR sessions while pathological SVV values were still observed at the same time after symptoms onset in patients without VR (1.25° ± 1.46° vs. 4.32° ± 2.81°, respectively; p < 0.0001). A close temporal correlation was observed in the time course of body weight distribution, mediolateral CoP position, and SVV bias over time, suggesting beneficial effects of the VR protocol at both the perceptive and postural levels. Conclusion: We recommend routine assessment of the ipsilateral and contralateral SVV bias separately for a better evaluation of otolith organs imbalance that can trigger chronic instability and dizziness. The SVV bias and the postural impairment caused by the imbalanced otolith inputs after unilateral vestibular loss can be rapidly normalized by tilting the patients in the roll plane, an additional means in the physiotherapist's toolbox. The protocol likely reweights the visual and somatosensory cues involved in the perception of verticality.

An overview of the current and emerging treatment options for vestibular migraine.

INTRODUCTION: Vestibular migraine is a relatively common syndrome characterized by the occurrence of vertigo and other vestibular symptoms, frequently -but not always- accompanied by migraine symptoms, such as headache, photophobia, and phonophobia. AREAS COVERED: The authors review the acute or abortive treatment during an attack and prophylactic treatment of vestibular migraines, including pharmacological and non-pharmacological options. EXPERT OPINION: Since its recognition as an independent clinical entity is recent, studies concerning its different treatment alternatives are still scarce, and the level of evidence of published studies is generally low. For acute treatment, although available data are conflicting, triptans are usually tried first. Neurostimulating devices can also be considered for acute treatment of vestibular migraine. Regarding the preventive treatment of vestibular migraine, based on the results of two randomized clinical trials and its pharmacological properties, flunarizine should be considered as the first treatment option. Second-line preventive treatments encompass propranolol, topiramate, venlafaxine, and valproic acid. Among non-pharmacological options, vestibular rehabilitation seems to be a sound prophylactic treatment option.

Semicircular canal and vestibular plugging in patients with Meniere's disease: a preliminary study.

BACKGROUND: Therapeutic options are limited for patients with intractable Meniere's disease who present with recurrent episodes of vertigo and drop attacks. AIMS/OBJECTIVES: To investigate the effectiveness and safety of simultaneous semicircular canal plugging and vestibular plugging in the treatment of Meniere's disease with drop attacks. MATERIAL AND METHODS: This was a single-center study with a 6-month post-operative follow-up. It included five patients with intractable Meniere's disease who presented with recurrent vertigo and drop attacks. All patients underwent surgery of semicircular canal plugging and vestibular plugging. The main outcome measures were vertigo control, drop attack control, hearing, and vestibular function; they were evaluated pre- and post-operatively. RESULTS: Of the five patients, none had a recurrence of drop attacks; four had no recurrence of vertigo, and one patient had one episode of vertigo. Two patients had residual hearing before surgery, which was preserved postoperatively. All patients showed altered results in vestibular tests. CONCLUSIONS: Semicircular canal plugging and vestibular plugging appear to be an effective treatment for intractable Meniere's disease presenting with recurrent vertigo and drop attacks. More studies are needed to confirm these results. SIGNIFICANCE: This novel surgical procedure can control vestibular symptoms of Meniere's disease while hopefully preserving the hearing function.

Different types of mastoid vibrations affect the amount and structure of minimum toe clearance variability differently.

BACKGROUND: The majority of research primarily examines the role of the vestibular system in regulating balance by assessing gait parameters in the transverse plane while neglecting those in the sagittal plane. The present study aimed to examine the impact of various forms of mastoid vibration (MV) on minimum toe clearance (MTC) and its pattern of variability. This study proposed two hypotheses: 1) the application of MV reduced the MTC, and 2) the application of different forms of MV influenced the amount and structure of MTC variability. METHODS: A total of twenty young adults participated in this study. A high-resolution motion capture system with eight cameras captured the minimum toe clearance. Three locomotor tasks were randomly assigned to these young participants: 1) walking normally on the treadmill, 2) walking with unilateral MV, and 3) walking with bilateral MV. The dependent variables were the mean of MTC, the amount, and the structure of MTC variability. The amount of MTC variability was calculated by the coefficient of variation represented, and the structure of MTC variability was measured using a sample entropy measure for a total of 200 MTCs. RESULTS: Applying unilateral and bilateral MV decreased the MTC significantly (-1.6 %, p = 0.038; -4.3 %, p < 0.001) compared to normal walking. Also, applying unilateral MV increased the amount (11.8 %, p = 0.001) and structure of MTC variability (14.3 %, p < 0.001) compared to normal walking. However, applying bilateral MV decreased the amount (-8.8 %, p = 0.001) and structure of MTC variability (-9.0 %, p < 0.001) compared to regular walking. CONCLUSION: Although the statistical differences in MTC and MTC variability were observed in the present study, the mean differences among the different MV conditions were relatively small, thereby requiring meticulous deliberation when extrapolating the results when implementing this MTC in the pathological cohort.

Vestibular-Evoked Myogenic Potential in Otosclerosis: A Systematic Review.

Background: Vestibular symptoms are noted in about 40% of the individuals with otosclerosis. Vestibular-evoked myogenic potential (VEMP) is a short latency potential that assesses the functioning of otolithic organs in the vestibular system. Aim/Objectives: This systematic review explored the VEMP findings in individuals with otosclerosis. Material and Methods: Three databases, PubMed, Scopus, and Cochrane were used to perform a systematic literature review regarding VEMP findings among individuals with otosclerosis. Results: A total of 14 studies that encompasses various VEMP parameters in otosclerosis patients were included. The air-conducted vestibular-evoked myogenic potential (AC-VEMP) often had poor response rates in otosclerosis due to conductive hearing loss. Presence of bone-conducted vestibular-evoked myogenic potential (BC-VEMP) before surgery indicated intact otolithic organs in otosclerosis. Postoperatively, presence of VEMP indicated intact vestibular structures postsurgery, while its absence could indicate vestibular trauma, though other factors like stimulus intensity or efficacy of the fitted piston could influence the results. AC-VEMP responses postsurgery suggested resolution of conductive pathology or absence of lasting effects of otosclerosis. Studies show cVEMP is more affected, indicating saccular dysfunction. Conclusion: Otosclerosis affects VEMP responses, with variations observed between AC- and BC-VEMPs. While AC-VEMP responses may be affected by conductive hearing loss, BC-VEMPs offer insights into inner ear function.