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Objectives: Symptomatic vestibulopathy impairs patients' lives. However, few studies have explored the lives of patients with compensated or asymptomatic vestibulopathy. This study investigated the quality of life (QOL), psychological health, and physical function of patients with vestibulopathy., Materials and Methods: Using the eighth Korea National Health and Nutrition Examination Survey database, we included individuals with data on demographic factors, diabetes, hypertension, dizziness experiences, pure-tone audiometry, video head impulse test (vHIT), Health-related Quality of Life Instrument with 8 Items, General Anxiety Disorder 7-item scale, stress, and walking and sitting times. Participants were classified into the following groups: an uncompensated group with abnormal vHIT result and chronic dizziness, a compensated group with abnormal vHIT result and a history of dizziness, an asymptomatic group with abnormal vHIT result and no history of dizziness, and a normal group without abnormal vHIT result or a history of dizziness., Results: Uncompensated vestibulopathy was more common in older individuals and women. The uncompensated group showed impairments in climbing stairs (P < 0.001), pain (P < 0.001), vitality (P = 0.001), working (P < 0.001), depression (P < 0.001), sleep (P = 0.001), happiness (P = 0.002), anxiety (P = 0.006), and stress (P = 0.003). The compensated group showed deficits in pain (P < 0.001), work (P = 0.006), sleep (P = 0.001), and happiness (P = 0.001). The asymptomatic group had no deficits in QOL, psychological health, or physical function. These tendencies were similar after controlling for age and gender., Conclusion: Vestibulopathy with a history of dizziness has a long-lasting impact on QOL and emotional status, even after compensation. Uncompensated vestibulopathy has a significant effect on QOL and mental health. Notably, though, the compensated group also showed a reduction in QOL. Appropriate interventions for each category of patients should be provided based on their impaired functions., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Han et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Objectives: Vestibular disorders can impact cognitive domains, including spatial orientation and memory, which are also affected in Alzheimer's disease. This study aimed to examine the association between Alzheimer's disease and a prior diagnosis of peripheral vestibular disorders in the elderly Taiwanese population., Methods: The case-control study sample was retrieved from Taiwan's Longitudinal Health Insurance Database 2010. We included 3138 cases with Alzheimer's disease and 9414 propensity-matched controls. We conducted multivariable logistic regression modeling to investigate the association between Alzheimer's disease and a prior diagnosis of peripheral vestibular disorders after accounting for sociodemographic characteristics and medical comorbidities including diabetes, coronary heart disease, hypertension, hyperlipidemia, and hearing loss., Results: The results revealed a statistically significant difference in the prevalence of prior peripheral vestibular disorders between patients with Alzheimer's disease and controls; 20.6% among patients with Alzheimer's disease and 11.4% among controls (p < 0.001). Multivariable logistic regression analysis found that patients with Alzheimer's disease were twice as likely as controls to have had a prior diagnosis of peripheral vestibular disorders, adjusted odds ratio 2.040 (95% confidence interval: 1.829-2.274)., Conclusions: The findings suggest the possibility of shared or related pathophysiological pathways in Alzheimer's disease and vestibular dysfunction disorders., Level of Evidence: 3 Laryngoscope, 134:4730-4735, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose of Review: Children experiencing dizziness frequently go unevaluated or experience delays in correct diagnosis due to systemic and inherent barriers. While most causes of pediatric dizziness do not involve the peripheral vestibular system, otolaryngologists are frequently consulted for expert opinion. This study offers a broad overview of the leading causes of pediatric dizziness and imbalance to assist otolaryngologists with evaluating and managing this challenging population., Recent Findings: Evaluation of children with dizziness should exclude serious central nervous system conditions. Approximately 70% of nonhazardous cases of pediatric dizziness are caused by migraine-related conditions, concussion, or functional disorders. Etiologies for peripheral vestibular dysfunction include inner ear malformations and vestibular disorders more commonly seen in adults. Audiometric and vestibular testing can provide helpful information, although correct diagnosis depends on a detailed history., Summary: Otolaryngologists should be familiar with the conditions that cause dizziness in children. While most underlying causes are outside their scope of practice, understanding possible etiologies can facilitate correct diagnosis and appropriate management., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: To analyse the effectiveness of optokinetic stimulation (OKS) for improving symptoms and function in patients with vestibular and balance disorders., Methods: PubMed (MEDLINE), SCOPUS, Web of Science (WOS), CINAHL Complete, and PEDro databases were searched to identify randomized controlled trials (RCTs) that included patients with vestibular and balance disorders and compared the effects of OKS versus other interventions or no intervention on subjective or objective functional outcomes. Data were analysed by the standardized mean difference (SMD) and its 95% confidence interval., Results: A total of 10 studies were selected including 468 patients, 177 of whom received OKS. There were no significant differences in scores on the Dizziness Handicap Inventory (DHI) (SMD = 0.02; 95% CI - 0.18 to 0.23; p = 0.83) or the visual analogue scale (VAS) for vertigo (SMD = 0.16; 95% CI - 1.25 to 1.58; p = 0.82). However, there were statistically significant differences in the timed up and go (TUG) test, with a large effect (SMD = - 1.13; 95% CI -2 to - 0.28; p = 0.009), and in the sensory organization test (SOT), with a medium effect (SMD = - 0.7; 95% CI - 1.21 to - 0.19; p = 0.007). Subgroup analysis showed significant effects of OKS on VAS (p = 0.017), TUG (p = 0.009) and SOT (p = 0.001) only in patients with balance disorders without vestibular disease (p > 0.05)., Conclusions: OKS may improve dizziness intensity measured with VAS or dynamic balance measured whit TUG and SOT in patients with balance disorders not due to vestibular disease. The quality of the evidence was low or very low due to the small number of included studies., Prospero Registry Number: CRD42023445024., (© 2024. The Author(s).)

Background: Children with Attention Deficit Hyperactivity Disorder (ADHD) demonstrate a heterogeneous sensorimotor, emotional, and cognitive profile. Comorbid sensorimotor imbalance, anxiety, and spatial disorientation are particularly prevalent among their non-core symptoms. Studies in other populations presented these three comorbid dysfunctions in the context of vestibular hypofunction., Objective: To test whether there is a subgroup of children with ADHD who have vestibular hypofunction presenting with concomitant imbalance, anxiety, and spatial disorientation., Methods: Children with ADHD-only (n = 28), ADHD + Developmental Coordination Disorder (ADHD + DCD; n = 38), and Typical Development (TD; n = 19) were evaluated for vestibular function by the Dynamic Visual Acuity test (DVA-t), balance by the Bruininks-Oseretsky Test of motor proficiency (BOT-2), panic anxiety by the Screen for Child Anxiety Related Emotional Disorders questionnaire-Child version (SCARED-C), and spatial navigation by the Triangular Completion test (TC-t)., Results: Children with ADHD vs. TD presented with a high rate of vestibular hypofunction (65 vs. 0 %), imbalance (42 vs. 0 %), panic anxiety (27 vs. 11 %), and spatial disorientation (30 vs. 5 %). Children with ADHD + DCD contributed more frequent and severe vestibular hypofunction and imbalance than children with ADHD-only (74 vs. 54 %; 58 vs. 21 %, respectively). A concomitant presence of imbalance, anxiety, and spatial disorientation was observed in 33 % of children with ADHD, all sharing vestibular hypofunction., Conclusions: Vestibular hypofunction may be the common pathophysiology of imbalance, anxiety, and spatial disorientation in children. These comorbidities are preferentially present in children with ADHD + DCD rather than ADHD-only, thus likely related to DCD rather than to ADHD disorder. Children with this profile may benefit from a vestibular rehabilitation intervention., Competing Interests: Declaration of competing interest The authors received no financial support for this article's research, authorship, and/or publication., (Copyright © 2024 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Congenital mitral stenosis is a rare anomaly. We report successful surgical management of congenital mitral stenosis in a patient with Kabuki syndrome. A 15-month-old male with Kabuki syndrome was referred and echocardiography showed isolated congenital mitral stenosis. The valve was not repairable as it was showing severe stenotic features in multilevel fashion. Valve was replaced with mechanical valve. Postoperative course was uneventful without any rhythm problem and he has been doing well clinically during the 25 months follow-up with regular international normalized ratio checks.

Objectives: Dizziness is among the most common reasons people seek medical care. There are data indicating patients with dizziness, unsteadiness, or vertigo may have multiple underlying vestibular disorders simultaneously contributing to the overall symptoms. Greater awareness of the probability that a patient will present with symptoms of co-occurring vestibular disorders has the potential to improve assessment and management, which could reduce healthcare costs and improve patient quality of life. The purpose of the current investigation was to determine the probabilities that a patient presenting to a clinic for vestibular function testing has symptoms of an isolated vestibular disorder or co-occurring vestibular disorders., Design: All patients who are seen for vestibular function testing in our center complete the dizziness symptom profile, a validated self-report measure, before evaluation with the clinician. For this retrospective study, patient scores on the dizziness symptom profile, patient age, and patient gender were extracted from the medical record. The dizziness symptom profile includes symptom clusters specific to six disorders that cause vestibular symptoms, specifically: benign paroxysmal positional vertigo, vestibular migraine, vestibular neuritis, superior canal dehiscence, Meniere disease, and persistent postural perceptual dizziness. For the present study, data were collected from 617 participants (mean age = 56 years, 376 women, and 241 men) presenting with complaints of vertigo, dizziness, or imbalance. Patients were evaluated in a tertiary care dizziness specialty clinic from October 2020 to October 2021. Self-report data were analyzed using a Bayesian framework to determine the probabilities of reporting symptom clusters specific to an isolated disorder and co-occurring vestibular disorders., Results: There was a 42% probability of a participant reporting symptoms that were not consistent with any of the six vestibular disorders represented in the dizziness symptom profile. Participants were nearly as likely to report symptom clusters of co-occurring disorders (28%) as they were to report symptom clusters of an isolated disorder (30%). When in isolation, participants were most likely to report symptom clusters consistent with benign paroxysmal positional vertigo and vestibular migraine, with estimated probabilities of 12% and 10%, respectively. The combination of co-occurring disorders with the highest probability was benign paroxysmal positional vertigo + vestibular migraine (~5%). Probabilities decreased as number of symptom clusters on the dizziness symptom profile increased. The probability of endorsing vestibular migraine increased with the number of symptom clusters reported., Conclusions: Many patients reported symptoms of more than one vestibular disorder, suggesting their symptoms were not sufficiently captured by the symptom clusters used to summarize any single vestibular disorder covered by the dizziness symptom profile. Our results indicate that probability of symptom clusters indicated by the dizziness symptom profile is comparable to prior published work on the prevalence of vestibular disorders. These findings support use of this tool by clinicians to assist with identification of symptom clusters consistent with isolated and co-occurring vestibular disorders., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To review the vestibular, aural, and perceptual symptoms of vestibular migraine (VM) that may present alongside vertigo., Recent Findings: Increased research attention to the wide spectrum of symptoms presenting in VM patients has improved understanding of this disorder, with recent identification of five different VM phenotypes. Research into the clinical overlap between VM and other chronic vestibular syndromes such as persistent postural-perceptual dizziness and mal-de-debarquement syndrome reveals a range of vestibular symptoms and hints at pathophysiological connections between migraine and vestibular dysfunction. Studies of migraine treatment for hearing loss suggest patients presenting with aural symptoms may have an underlying diagnosis of migraine and deserve a trial of migraine preventives. Research into the neurologic basis of the perceptual disorder Alice in Wonderland syndrome has revealed brain areas that are likely involved and may help explain its prevalence in VM patients. VM is a sensory processing disorder that presents with more than just vertigo. Understanding the range of potential symptoms improves diagnosis and treatment for migraine patients whose diagnosis may be missed when only the symptoms identified in the diagnostic criteria are considered., (© 2024. The Author(s).)

Purpose: To evaluate the efficacy of vestibular rehabilitation therapy (VRT) for management of patients with persistent postural perceptual dizziness (PPPD) utilizing subjective and objectives outcome measures and to study the effect of degree of both anxiety and depression in patients on the response of vestibular rehabilitation therapy., Methods: Thirty-three PPPD patients participated in this study. Selection of patients was based on the diagnostic criteria for PPPD stated by Barany society in the International Classification of Vestibular Disorders (2017). Every patient was subjected to history taking, anxiety and depression assessment, Arabic version of Dizziness Handicap Inventory (DHI), and sensory organization test (SOT). All patients received vestibular rehabilitations therapy. Assessment of VRT outcome was conducted after 6 weeks of VRT., Results: The mean patients' age was 40.9 ± 16.3 years, and nearly equal gender distribution. Vestibular migraine was the most precipitating condition (24.2%) in patients with PPPD. (39.4%) of patients had abnormal scores of anxiety and depression tests, all patients had from moderate to severe degrees of handicap caused by dizziness as measured by DHI, most of patients had abnormal findings in all conditions of SOT. After vestibular rehabilitation therapy, DHI and SOT scores showed significant improvement after VRT. More improvement was found among the group with no anxiety and depression., Conclusion: VRT were effective in improving balance abnormalities in patients with PPPD evidenced by subjectively by DHI scores and objectively by SOT results. PPPD patients with concomitant psychiatric disorders; anxiety and depression experienced the least degree of improvement., (© 2023. The Author(s).)

Objective: This study aimed to identify the potential subgroups of migraines based on the patterns of migraine associated symptoms, vestibular and auditory symptoms using latent class analysis and to explore their characteristics., Method: A total of 555 patients with migraine participated in the study. Symptoms such as nausea, vomiting, photophobia, phonophobia, osmophobia, visual symptoms, vestibular symptoms (dizziness, vertigo), and auditory symptoms (tinnitus, hearing loss, aural fullness) were assessed. Latent class analysis was performed to identify subgroups of migraines. Covariates such as gender, age of migraine onset, frequency of migraine attacks per month, and family history were also considered., Results: The analysis revealed four latent classes: the Prominent Vestibular; Prominent Nausea; Presenting Symptoms but not prominent or dominant; and Sensory Hypersensitivity groups. Various covariates, such as gender, age of migraine onset, and frequency of migraine attacks, demonstrated significant differences among the four groups. The Sensory Hypersensitivity group showed the presence of multiple sensory symptoms, earlier age of migraine onset, and higher proportion of females. The Prominent Vestibular group had the highest probability of dizziness or vertigo but lacked the presence of auditory symptoms. The Prominent Nausea group exhibited prominent nausea. The Presenting Symptoms but not prominent or dominant group comprised individuals with the highest migraine attacks per month and proportion of chronic migraine., Conclusion: This study identifies four subgroups of migraines based on the patterns of symptoms. The findings suggest potential different but overlapped mechanisms behind the vestibular and auditory symptoms of migraine. Considering the different patterns of migraine-related symptoms may provide deeper insights for patients' prognosis and clinical decision-making., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Orthostatic hypotension (OH) is one of the most common symptoms in patients with multiple system atrophy (MSA). Vestibular system plays an important role in blood pressure regulation during orthostatic challenges through vestibular-sympathetic reflex. The current study aimed to investigate the relationship between vestibular function and OH in patients with MSA., Methods: Participants with MSA, including 20 with OH (mean age, 57.55 ± 8.44 years; 7 females) and 15 without OH (mean age, 59.00 ± 8.12 years; 2 females) and 18 healthy controls (mean age, 59.03 ± 6.44 years; 8 females) were enrolled. Cervical and ocular vestibular evoked myogenic potentials (cVEMPs and oVEMPs) tests were conducted to evaluate vestibular function., Results: Patients with MSA presented with significantly higher rate of absent cVEMPs (57.1% vs 11.1%, p = 0.001) and oVEMPs (25.7% vs 0, p = 0.021) than controls. MSA patients with OH showed more absent cVEMPs (75.0% vs 11.1%, Bonferroni corrected p < 0.001) and oVEMPs (40.0% vs 0, Bonferroni corrected p = 0.003) than controls. Patients with OH also showed higher rate of absent cVEMPs than those without OH (33.3%, Bonferroni corrected p = 0.014)., Conclusions: Our results demonstrated that impairment of vestibular function was associated with MSA, particularly in those with OH. Absent VEMPs may be a potential marker for MSA severity. Our findings suggest that impaired vestibular function is involved in OH development and may serve as an intervention target., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Objective: Persistent postural-perceptual dizziness classifies patients with chronic dizziness, often triggered by an acute episode of vestibular dysfunction or threat to balance. Unsteadiness and spatial disorientation vary in intensity but persist for over three months, exacerbated by complex visual environments., Method: Literature suggests diagnosis relies on a clinical history of persistent subjective dizziness and normal vestibular and neurological examination findings. Behavioural diagnostic biomarkers have been proposed, to facilitate diagnosis., Results: Research has focused on understanding the neural mechanisms that underpin this perceptual disorder, with imaging data supporting altered connectivity between neural brain networks that process vision, motion and emotion. Behavioural research identified the perceptual and motor responses to a heightened perception of imbalance., Conclusion: Management utilises head and body motion detection, and downregulation of visual motion excitability, reducing postural hypervigilance and anxiety. Combinations of physical and cognitive therapies, with antidepressant medications, help if the condition is associated with mood disorder.

Objective: To investigate the clinical manifestations and complete auditory function in primary tinnitus patients with and without migraine or vestibular migraine., Design: Retrospective case-control study., Setting: A tertiary referral center., Participants: This study enrolled 298 patients from the Kaohsiung Veterans General Hospital. All patients were diagnosed with primary tinnitus by a neurotologist between April 2020 and August 2021. Patients were excluded if they had histories of chronic otitis media, idiopathic sudden sensorineural hearing loss, Ménière's disease, skull base neoplasm, or temporal bone trauma., Interventions: Twenty-five-item Tinnitus Handicap Inventory (THI), speech audiometry including speech recognition threshold, most comfortable level, uncomfortable loudness levels, dynamic range, and pure-tone audiometry., Main Outcomes Measures: Objective hearing loss is defined as a mean threshold greater than 25 dB. Extremely elevated THI is defined as a score greater than 1 standard deviation above the mean THI., Results: Among the 298 patients with tinnitus, 149 were women and 149 were men, with a mean age of 57.06 (range, 19.22-94.58) years.A total of 125 patients completed the THI questionnaire during their initial visit. The median THI score was 32 (95% confidence interval: 13.98-56.00), and the mean score was 34.99 with a standard deviation of 21.01. The sole contributing factor significantly associated with higher total THI score was the diagnosis of migraine or vestibular migraine (p < 0.001, odds ratio = 19.41).Tinnitus patients with migraine or vestibular migraine exhibited significantly lower mean pure-tone auditory thresholds (right 22.2 versus 29.5, p = 0.002; left 22.5 versus 30.4, p < 0.001), speech recognition threshold (right 20.0 versus 25.2, p = 0.016; left 20.2 versus 25.5, p = 0.019), and most comfortable levels values (right 46.1 versus 51.4, p = 0.007; left 46.9 versus 51.4, p = 0.021) compared with the tinnitus patients without migraine., Conclusions: In this population-based study, patients with primary tinnitus experienced significantly higher THI scores and exhibited concurrent symptoms, including dizziness/vertigo, cervicalgia, and migraine or vestibular migraine. Among these parameters, the diagnosis of migraine or vestibular migraine was the sole contributor to significant higher THI score., Competing Interests: The authors have no conflicts of interest to declare. All authors have seen and agree with the contents of the article, and there is no financial interest to report. The authors certify that the submission is original work and is not under review at any other publication., (Copyright © 2024, Otology & Neurotology, Inc.)

Objective: This study analyzed the systemic and oral abnormalities in individuals with Kabuki syndrome (KS) that might be investigated to enhance the early diagnosis and treatment by a multidisciplinary team, minimizing the consequences to the individual's health., Study Design: Clinical examination was conducted on 15 individuals to investigate orodental alterations such as tooth abnormalities and cleft lip and/or palate, and the patient records were also reviewed to investigate systemic diseases such as cardiopathies, infectious and immunologic diseases, nephropathies, and delayed neuropsychomotor development., Results: All individuals with KS presented cleft lip and/or palate, 11 (73.34%) tooth abnormalities, 5 (33.34%) congenital cardiopathies, 12 (80%) infectious or immunologic diseases, 1 (6.67%) nephropathy, and 14 (93.34%) had an intellectual disability., Conclusion: Individuals with KS often have dental anomalies such as hypodontia, cleft or palate, and systemic disorders such as congenital heart disease and infectious diseases. Intellectual disability is present in most cases. These alterations should be investigated as early as possible to prevent the increase in morbidity in these individuals., Competing Interests: Declaration of interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background and Purpose: Identifying vestibular causes of dizziness and unsteadiness in multi-sensory neurological disease can be challenging, with problems typically attributed to central or peripheral nerve involvement. Acknowledging vestibular dysfunction as part of the presentation provides an opportunity to access targeted vestibular rehabilitation, for which extensive evidence exists. A diagnostic framework was developed and validated to detect vestibular dysfunction, benign paroxysmal positional vertigo or vestibular migraine. The specificity and sensitivity of the diagnostic framework was tested in patients with primary mitochondrial disease., Methods: Adults with a confirmed diagnosis of primary mitochondrial disease were consented, between September 2020 and February 2022. Participants with and without dizziness or unsteadiness underwent remote physiotherapy assessment and had in-person detailed neuro-otological assessment. The six framework question responses were compared against objective neuro-otological assessment or medical notes. The output was binary, with sensitivity and specificity calculated., Results: Seventy-four adults completed the study: age range 20-81 years (mean 48 years, ±SD 15.05 years); ratio 2:1 female to male. The framework identified a vestibular diagnosis in 35 participants, with seven having two diagnoses. The framework was able to identify vestibular diagnoses in adults with primary mitochondrial disease, with a moderate (40-59) to very high (90-100) sensitivity and positive predictive value, and moderate to high (60-74) to very high (90-100) specificity and negative predictive value., Conclusions: Overall, the clinical framework identified common vestibular diagnoses with a moderate to very high specificity and sensitivity. This presents an opportunity for patients to access effective treatment in a timely manner, to reduce falls and improve quality of life., (© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Objective: Anxiety and depression are psychiatric comorbidities potentially associated with dizziness and migraine, which can influence disease state, prognosis, and clinical outcomes. Vestibular migraine (VM) is a disease that causes repeated vestibular symptoms in people with a history of migraines. We investigated the prevalence and contributing factors of anxiety and depression in patients with VM. Methods: A total of 74 patients with VM were enrolled in this study. All patients underwent pure-tone audiometry, the examination of spontaneous nystagmus, Dix-Hallpike maneuver or supine-roll test, video head impulse test, and caloric testing on the day of the visit. We used the Hospital Anxiety and Depression Scale (HADS) to assess the symptoms of anxiety and depression. Dizziness Handicap Inventory was used to measure the intensity of vestibular symptoms. The participants were divided into normal and abnormal groups based on their HADS anxiety and depression scores, and demographic and clinical factors were analyzed. To identify factors associated with anxiety and depression, multivariate logistic regression analyses were performed. Results: A total of 36 (48.6%) patients demonstrated clinically relevant anxiety levels, and 24 (32.4%) demonstrated depression. Twenty-five (33.8%) patients were diagnosed with peripheral vestibular dysfunction. In the multivariable analyses, peripheral vestibular dysfunction and severe intensity vestibular symptoms were significantly associated with anxiety and depression. None of migraine features were significantly associated with anxiety and depression. Conclusion: The prevalence of anxiety in patients with VM is considerably higher than in the depression. VM patients with peripheral vestibular dysfunction are particularly susceptible to anxiety and depression. Therefore, timely screening for vestibular function and psychiatric disorders in VM patients should be considered., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Importance: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing., Objectives: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL., Design, Setting, and Participants: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023., Main Outcomes and Measures: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay., Results: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment., Conclusions and Relevance: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.

Background/introduction: In patients with acute vestibular syndrome (AVS), differentiating between stroke and nonstroke causes is challenging in the emergency department (ED). Correct diagnosis of vertigo etiology is essential for early optimum treatment and disposition., Objectives: The aim of this systematic review and meta-analysis was to summarize the published evidence on the potential of blood biomarkers in the diagnosis and differentiation of peripheral from central causes of AVS., Methods: A literature search was conducted for studies published until January 1, 2023, in PubMed, Ovid Medline, and EMBASE databases analyzing biomarkers for the differentiation between central and peripheral AVS. The Quality Assessment of Diagnostic Accuracy Studies questionnaire 2 was used for quality assessment. Pooled standardized mean difference and 95% confidence intervals were calculated if a biomarker was reported in two or more studies. Heterogeneity among included studies was investigated using the I 2 metric., Results: A total of 17 studies with 859 central and 4844 peripheral causes of acute dizziness or vertigo, and analysis of 61 biomarkers were included. The general laboratory markers creatinine, blood urea nitrogen, albumin, C-reactive protein, glucose, HbA1c, leukocyte counts, and neutrophil counts and the brain-derived biomarkers copeptin, S100 calcium-binding protein β (S100β), and neuron-specific enolase (NSE) significantly differentiated central from peripheral causes of AVS., Conclusions: This systematic review and meta-analysis highlights the potential of generalized inflammatory markers and brain-specific blood protein markers of NSE and S100β as diagnostic biomarkers for central from peripheral differentiation in AVS. These results, as a complement to clinical characteristics, provide guidance for future large-scale diagnostic research, in this challenging ED patient population., (© 2024 Society for Academic Emergency Medicine.)

Objective: To determine the utility of the motion sensitivity quotient (MSQ) in diagnosing pediatric vestibular migraine (VM) and to characterize the role of motion sensitivity and headache control on vestibular rehabilitation (VR) outcomes in pediatric VM., Study Design: Retrospective cohort analysis., Setting: Pediatric tertiary referral center., Patients: Children (≤18 years old) with dizziness who completed vestibular testing from January 2016 to August 2022, diagnosed with either VM or another vestibular disorder., Interventions: VR, which included MSQ testing., Main Outcome Measures: Initial MSQ, number and duration of vestibular physical therapy (PT) sessions, PT goals met, and posttreatment MSQ., Results: Two hundred fifty-seven patients met study criteria. MSQ was not a reliable diagnostic marker in pediatric VM as there was no difference in initial MSQ between VM and non-VM patients (9.4 vs. 7.8 in non-VM, p = 0.014). Both VM (n = 116) and non-VM (n = 141) patients demonstrated significant improvement in MSQ after VR (p = 0.004). However, VM patients tended to be less likely to meet at least one PT goal (60 vs. 77% in non-VM, p = 0.016, d = 0.37), although not significant. VM patients with more frequent headaches had significantly higher initial MSQ (p = 0.008). VM patients with more frequent headaches or higher initial MSQ tended to require increased number and longer duration of VR (small/medium effect size although not statistically significant after Bonferroni correction)., Conclusion: VR is an effective treatment for both VM and non-VM pediatric patients. VM patients, especially those with severe motion sensitivity or poorly controlled headaches, may be less responsive to VR and may require increased frequency and duration of VR. Our findings propose the importance of counseling pediatric patients with severe motion sensitivity or uncontrolled migraines regarding realistic expectations of their VR course., Competing Interests: Disclosures/conflicts of interest: The authors disclose no conflicts of interest., (Copyright © 2024, Otology & Neurotology, Inc.)

Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is an hereditary autosomal recessive disease. Recent studies propose including chronic cough (CC) as a symptom of CANVAS. For 10 patients with CANVAS as genetically confirmed by biallelic expansion of the AAGG repeat motif (AAGGGexp) in intron 2 of replication factor C subunit 1 (RFC1), our aim was, as a multidisciplinary team, to describe clinical and functional characteristics and possible causes of CC following European Respiratory Society (ERS) recommendations, and to evaluate CC impact on quality of life (QoL) using self-administered questionnaires (Cough Severity Diary, Leicester Cough Questionnaire, Discrete Emotions Questionnaire, and EQ-5D-5L). In all 10 patients, the CC was a dry cough that developed several years prior to the neurological symptoms (mean 14.2 years); 7 patients had symptoms compatible with gastroesophageal reflux (GER), 5 with pathological GER diagnosed by 24-h esophageal pH testing, and 6 patients had impaired esophageal motility diagnosed by high-resolution esophageal manometry, most frequently ineffective peristalsis. Although further studies are required for confirmation, we conclude that CC may be a characteristic prodrome of CANVAS and may be related to GER and esophageal disorders. Furthermore, CC affects patients' QoL, especially in the psychosocial sphere., (© 2023. The Author(s).)

Background and Purpose: Vigilance towards balance has been proposed to underpin various chronic dizziness disorders, including persistent postural-perceptual dizziness (PPPD). The objective of this study was to develop (through patient input) a validated balance-specific measure of vigilance that comprehensively assesses the varied ways in which this construct may manifest., Methods: We developed the Balance Vigilance Questionnaire (Balance-VQ) through patient and clinician feedback, designed to assess vigilance towards balance. We then validated the questionnaire in 497 participants consisting of patients diagnosed with chronic dizziness disorders (including 97 individuals diagnosed with PPPD) and healthy controls., Results: The final six-item Balance-VQ was shown to be a valid and reliable way to assess vigilance towards balance. Scores were significantly higher in individuals diagnosed with PPPD compared to controls. Although scores were also higher in the PPPD group compared to individuals with diagnosed vestibular disorders other than PPPD, Balance-VQ scores did not discriminate between the two groups when confounding factors (including dizziness severity) were controlled for. Scores did, however, independently discriminate between the PPPD group and individuals who experience dizziness in daily life, but who have not been diagnosed with a neuro-otological disorder., Conclusions: Our findings confirm that the Balance-VQ is a valid and reliable instrument for assessing vigilance towards balance. As symptom vigilance has been identified as a key risk factor for developing chronic dizziness following acute vestibular symptoms or balance disruption, we recommend using the Balance-VQ as a screening tool in people presenting with such symptoms., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Superior canal dehiscence syndrome (SCDS) is a vestibular disorder in which the presence of a pathological third window in the labyrinth causes several vestibular and cochlear symptoms. Herein, we review the diagnostic criteria and treatment of SCDS. The cause of SCDS is considered to be congenital or acquired; however, its etiology is not well known. Symptoms: Vertigo and/or oscillopsia induced by loud sounds (Tullio phenomenon) or stimuli that change the middle ear or intracranial pressure (fistula symptoms) with vestibular symptoms and hyperacusis and aural fullness with cochlear symptoms are characteristic clinical complaints of this syndrome. Neurological tests: Vertical-torsional eye movements can be observed when the Tullio phenomenon or fistula symptoms are induced. Conductive hearing loss with both a decrease in the bone conduction threshold at lower frequencies and an increase in the air conduction threshold at lower frequencies may be present on audiometry. Cervical and/or ocular vestibular evoked myogenic potentials are effective in strongly suspecting the presence of a pathologic third window in the labyrinth. Computed tomography (CT) imaging: High-resolution CT findings with multiplanar reconstruction in the plane of the superior semicircular canal consistent with dehiscence indicate SCDS. The Pöschl view along the plane of the superior semicircular canal and the Stenvers view perpendicular to it are recommended as CT imaging conditions. Findings from all three major diagnostic categories (symptoms, neurological tests, and/or CT imaging) are needed to diagnose SCDS. The surgical approaches for SCDS are as follows: the 1) middle cranial fossa approach, 2) transmastoid approach, and 3) round window and oval window reinforcement. Each technique has advantages and disadvantages., Competing Interests: Declaration of Competing interest The authors declare that there is no conflict of interest., (Copyright © 2023. Published by Elsevier B.V.)

Background: The relationship between young-onset dementia and peripheral vestibular disorders remained largely unknown although this association was observed in the older population., Objective: This case-control study aims to investigate the association of young-onset dementia with a pre-existing diagnosis of peripheral vestibular disorders using a population-based data from Taiwan's Longitudinal Health Insurance Database 2010., Methods: This study included 989 patients with young-onset dementia and 2967 propensity-score-matching controls. Differences in baseline characteristic between patients with young-onset dementia and controls were investigated using chi-square tests or t-tests. Multiple logistic regression models were employed to assess the association of young-onset dementia (outcome) with pre-existing peripheral vestibular disorders (predictor)., Results: Compared to patients without young-onset dementia, those affected by this condition exhibited a statistically significantly higher rate of peripheral vestibular disorders (18.3% versus 8.2%, p < 0.001). Furthermore, our analysis found notable between-group disparities in the rates of Meniere's Disease (3.5% versus 2.0%, p= 0.015), benign paroxysmal positional vertigo (2.4% versus 1.1%, p= 0.006), and vestibular neuritis (2.4% versus 1.1%, p= 0.003). Multiple logistic regression analysis showed that the presence of prior peripheral vestibular disorders increased the odds of young-onset dementia [2.603 (95% CI = 2.105∼3.220)] after adjusting for age, sex, monthly income, geographic location, urbanization level, hyperlipidemia, diabetes, coronary heart disease, hearing loss, and hypertension., Conclusions: The study findings demonstrate a notable association between young-onset dementia and pre-existing peripheral vestibular disorders, suggesting that vestibular malfunction could play a role in the development of young-onset dementia.

Dizziness is a common symptom among patients in primary care, general neurology, and headache clinic practices. Vestibular migraine is conceptualized as a condition of recurrent attacks of vestibular symptoms attributed to migraine. It is now considered the most common cause of spontaneous episodic vertigo. Persistent postural-perceptual dizziness (PPPD) has more recently been defined based on four previous clinical entities as a syndrome of chronic daily dizziness, unsteadiness, or nonspinning vertigo that fluctuates and is exacerbated by postural, motion, or visual factors. Although PPPD is more often precipitated by other conditions causing vertigo, unsteadiness, or dizziness, it is discussed at length in this chapter because vestibular migraine is among the most common triggers for development of PPPD. Pathophysiology of each is incompletely understood, and with lack of biomarkers, the diagnosis of each rests on consensus-derived, symptom-based criteria. Areas of uncertainty exist regarding some overlapping symptoms that may create potential diagnostic confusion between the conditions. This chapter provides a comprehensive review of the current state of vestibular migraine and PPPD, including diagnostic and management guidance for when they occur separately, together, or along with other common comorbidities., (Copyright © 2024 Elsevier B.V. All rights reserved, including those for text and data mining, AI training, and similar technologies.)

Background: With the increasing clinical focus on the safety of bilateral cochlear implantation (CI) and the potential risk of bilateral vestibular dysfunction, evaluating vestibular end-organ function in patients with vestibular malformations with accompanying abnormalities has been strongly recommended., Objectives: To identify the vestibular-evoked myogenic potential (VEMP) values among children with sensorineural hearing loss (SNHL) with vestibular malformation and assess the effectiveness of VEMP testing for inner ear malformations (IEM) diagnosis., Methods: This study included 96 children (192 ears), including those with vestibular malformations (48 ears), large vestibular aqueduct syndrome (LVAS) (50 ears), and SNHL without IEM (94 ears; control group). All groups underwent ocular and cervical VEMP (oVEMP and cVEMP, respectively) testing. The response rates, VEMP parameters, and wave characteristics were compared., Results: The cVEMP response rates were 37.5 %, 64 %, and 58.51 % and the oVEMP response rates were 42.86 %, 78.95 %, and 77.27 % in the vestibular malformation, LVAS, and control groups, respectively, and significantly differed between groups (cVEMP: X 2  = 18.228, P<0.001) (oVEMP: X 2  = 7.528, P = 0.023). Significant inter-group differences were observed for the cVEMP and oVEMP latency and amplitude (P < 0.05). The LVAS group's waveform exhibited a prolonged latency and increased amplitude compared with that of the other groups., Conclusion: Patients with SNHL were highly susceptible to otolith dysfunction, regardless of comorbid vestibular malformation presence. Measuring VEMPs is an effective and rapid evaluation technique for vestibular function and could provide a basis for vestibular rehabilitation training., Competing Interests: Declaration of competing interest There are no conflicts of interest, financial or otherwise, to declare., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Objective: To assess vestibular (i.e., passive self-motion) perception in patients diagnosed with persistent postural-perceptual dizziness (PPPD)., Study Design: Case-controlled, cross-sectional, observational investigation., Setting: Single-center laboratory-based study., Patients: Thirteen patients with PPPD, 13 age-matched healthy control volunteers. Of those with PPPD, eight had co-occurring vestibular migraine (VM)., Interventions: All participants completed a vestibular threshold test battery reflecting perception with predominant inputs from ( a ) the otoliths (1-Hz interaural y -axis translation, 1-Hz superior-inferior z -axis translation), ( b ) the semicircular canals (2-Hz yaw rotation, 2-Hz tilts in the planes of the vertical canal pairs), and ( c ) and canal-otolith integration (0.5-Hz roll tilt)., Main Outcome Measures: Direction-recognition thresholds for each vestibular threshold test condition., Results: Across all patients with PPPD, higher thresholds for superior-inferior z -translations thresholds in comparison to age-matched healthy control participants were identified ( p < 0.001). Those patients with co-occurring VM and PPPD (PPPD/+VM) displayed significantly higher z -translation thresholds ( p = 0.006), whereas patients with PPPD without VM (PPPD/-VM) displayed significantly higher roll tilt thresholds ( p = 0.029)., Conclusions: Patients with PPPD did not display a global worsening of passive self-motion perception as quantified by vestibular perceptual thresholds. Instead, patients with PPPD displayed elevated thresholds for only roll tilt and z -translation thresholds, with the relative change in each threshold impacted by the co-occurrence of VM. Because both z -translation and roll tilt motions are reliant on accurate gravity perception, our data suggest that patients with PPPD may exhibit impaired processing of graviceptive cues., Competing Interests: Sources of support and disclosure of funding: This research was supported by an Ohio State Alumni Grants for Graduate Research and Scholarship (M.J.K.) and the Department of Defense Congressionally Directed Medical Research Programs Award Number W81XWH192000. No personal or professional conflicts of interest are reported by the authors., (Copyright © 2023, Otology & Neurotology, Inc.)

Background and Purpose: Vestibular symptoms are common in emergency department (ED) patients and have various causes, including stroke. Accurate identification of stroke in patients with vestibular symptoms is crucial for timely management. We conducted a prospective cross-sectional study from 2015 to 2019 to determine stroke prevalence and associated symptoms in ED patients with vestibular symptoms, aiming to improve diagnosis and outcomes., Methods: As part of the DETECT project, we screened 1647 ED patients with acute vestibular symptoms. Following a retrospective analysis of 961 head and neck magnetic resonance imaging (MRI) scans, we included 122 confirmed stroke cases and assessed them for vestibular signs and symptoms., Results: Stroke prevalence in dizzy patients was 13% (122/961 MRI scans). Most patients (95%) presented with acute vestibular symptoms with or without nystagmus, whereas 5% had episodic vestibular syndrome (EVS). Nystagmus was present in 50% of stroke patients. Eighty percent had a purely posterior circulation stroke, and nystagmus was absent in 46% of these patients. Seven patients (6%) had lesions in both the anterior and posterior circulation. Vertigo was experienced by 52% regardless of territory., Conclusions: A stroke was identified in 13% of ED patients presenting with acute vestibular symptoms. In 5%, it was EVS. Most strokes were in the posterior circulation territory; vertigo occurred with similar frequency in anterior and posterior circulation stroke, and absence of nystagmus was common in both., (© 2023 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Purpose: To determine if Persistent Postural-Perceptual Dizziness (PPPD) is associated with increased burden of dizziness and quality of life. Secondly, if this association is present, to determine if it can be explained by differences in anxiety and/or depression between patients with PPPD and dizzy patients without PPPD., Methods: Cross-sectional study performed in an outpatient otolaryngology clinic, including patients 18-67 years referred from primary care for suspected vestibular disease with chronic dizziness. Patients underwent clinical examination and completed the following questionnaires: Dizziness Handicap Inventory (DHI), RAND-12 Health Status Inventory and Hospital Anxiety and Depression Scale (HADS). Scores in DHI and RAND-12 were compared between patients diagnosed with PPPD and patients without PPPD., Results: 202 patients were included. 150 (74%) were women and 37 (18%) were diagnosed with PPPD. Patients in the PPPD group had increased burden of dizziness and reduced quality of life (QoL) as shown by a higher mean DHI score (49.2 vs. 30.8; p < 0.001) and reduced mean RAND-12 physical score (39.0 vs. 44.6; p = 0.004). After adjusting for age, gender and HADS, PPPD was associated with a 15.3 (p < 0.001) points increase in DHI score, and a 4.0 (p = 0.020) points decrease in RAND-12 physical score., Conclusion: Patients with PPPD have a higher burden of dizziness and a lower physical health-related quality of life (HRQoL) compared to other dizzy patients. The difference was evident also after adjusting for anxiety and depression, illustrating how PPPD is a different entity than these common psychiatric conditions., (© 2023. The Author(s).)

Objective: Investigate the relationship between Vestibular Migraine Patient Assessment Tool and Handicap Inventory (VM-PATHI) scores and daily dizziness symptoms., Study Design: Prospective cohort analysis of 52 patients with vestibular migraine (VM)., Setting: Tertiary referral center., Patients: Fifty-two patients diagnosed with VM or probable VM according to Barany Society criteria., Interventions: Subjects reported their dizzy symptoms (on a scale of 0 [no symptoms], 1 [mild], 2 [moderate], and 3 [severe]) every day for 1 month via automated text messaging linked to a cloud-based research database. Subjects completed VM-PATHI and Dizziness Handicap Inventory (DHI) scores at the end of the month. We examined the correlation between a composite of daily dizziness scores with VM-PATHI and DHI scores through linear regression and correlation analysis., Main Outcome Measures: Pearson correlation coefficient, R2 value., Results: VM-PATHI showed a moderate correlation with daily dizziness symptoms (correlation coefficient, 0.51). DHI showed a lower correlation with daily dizziness (correlation coefficient, 0.38). VM-PATHI score was a strong predictor of daily dizziness with univariate linear regression ( R2 = 0.26, p = 0.001). In a multiple linear regression model with age, history of anxiety and/or depression, and VM-PATHI, the VM-PATHI score was the only statistically significant predictor of daily dizziness ( p < 0.001)., Conclusions: Daily dizziness symptoms are better correlated with VM-PATHI score than the DHI, providing further validation of VM-PATHI as a disease-specific outcome measure for patients with VM., Competing Interests: Sources of support and disclosure of funding: Dr. Sharon receives research support from Advanced Bionics and Eli Lilly and is a paid consultant for Spiral Therapeutics. All other authors have no conflicts of interest or sources of funding to disclose., (Copyright © 2023, Otology & Neurotology, Inc.)

Persistent postural-perceptual dizziness (PPPD) is a functional neuro-otologic (vestibular) disorder manifesting dizziness, unsteadiness, or nonspinning vertigo lasting 3 months or more and exacerbated by upright posture, active or passive motion, and complex visual stimuli. PPPD is the most common cause of chronic vestibular symptoms. Early pathophysiologic models of PPPD emphasized the adverse effects of anxiety on postural control and spatial orientation. More recent concepts added predictive processing of sensory inputs and alterations in motion perception. Herein, a third-generation model incorporates prioritization of postural stability over fluid locomotion to explain symptoms, physiologic and neuroimaging data, and effects of current treatments., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: There is increasing evidence for close interrelations between vestibular and emotional brain networks. A study in patients with bilateral peripheral vestibulopathy (BVP) showed relatively low vertigo-related anxiety (VRA), despite high physical impairment. The current working hypothesis proposes the integrity of the peripheral vestibular system as a prerequisite for development of VRA. Here we contribute by evaluating VRA and vestibular-related handicap in central vestibular disorders., Methods: Of 6396 patients presenting in a tertiary vertigo centre, 306 were identified with four clear central vestibular disorders: pure cerebellar ocular motor disorder (COD; 61), cerebellar ataxia (CA; 63), atypical parkinsonian syndromes (APS; 28), vestibular migraine (VM; 154). Their results of the Vertigo Handicap Questionnaire (VHQ), with its subscales for anxiety and handicapped activity, were compared to those of 65 BVP patients. Postural instability was measured on a force-plate. Multivariate linear regression was used to adjust for patient demographics., Results: Patients with chronic central vestibular disorders (COD, CA, APS) had relatively low VRA levels comparable to those in BVP, independent of increased handicapped activity or postural instability. Only VM patients showed significantly higher VRA, although their activity impairment and postural instability were lowest. No significant differences within chronic central vestibular disorders were found for VRA and subjective activity impairment., Conclusions: Subjective and objective vestibular-related impairment are not necessarily correlated with vestibular-related anxiety in central vestibular disorders. Our findings rather support the hypothesis that, in addition to an intact peripheral, an intact central vestibular system could also serve as a prerequisite to develop specific VRA., (© 2023. The Author(s).)

Introduction: Vestibular symptoms, including vertigo, dizziness, and gait unsteadiness, are a frequent reason of urgent medical assistance. Their causes are numerous and diverse, including neurological, otorhinolaryngological, and systemic diseases. Therefore, following a systematic approach is essential to differentiate striking but benign conditions from others that can compromise the patient's life. This study is intended to review vestibular disorders from a practical perspective and provide guidance to physicians involved in the emergency care of patients with vestibular symptoms., Materials and Methods: A narrative review was performed, revisiting the main causes of vestibular disorders., Results: Based on the speed of onset, duration, and history of similar episodes in the past, vestibular disorders can be categorized into three syndromic entities (acute, recurrent, and chronic vestibular syndromes). The most representative conditions pertaining to each group were reviewed (including their diagnosis and treatment) and a practical algorithm was proposed for their correct management in the acute care setting., Conclusions: Carrying out a correct categorization of the vestibular disorders is essential to avoid diagnostic pitfalls. This review provides useful tools for clinicians to approach their patients with vestibular symptoms at the emergency room., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: To investigate the effect of early vestibular rehabilitation on physical function and dizziness in patients with acute vestibular disorders., Databases Reviewed: The electronic databases examined included PubMed (MEDLINE), Cochrane Central Register of Controlled Trials (CENTRAL), EMBASE (via Dialog), and PEDro., Methods: The inclusion criteria in terms of the study participants were patients 20 years and older with an acute unilateral peripheral vestibular disorder. We included individual randomized controlled trials (RCTs), cluster-RCTs, quasi-RCTs, and crossover trials. The outcomes were gait, balance (eyes open, eyes close), activities of daily living, dizziness, and vestibular function. Early vestibular rehabilitation was defined as rehabilitation within 14 days of vestibular disorder onset or surgery. Main outcome measures were gait, balance (eyes open, eyes close), activities of daily living, dizziness, and vestibular function., Results: Twelve trials involving 542 participants were included. Early vestibular rehabilitation improved the Dizziness Handicap Inventory by -7.18 (95% confidence interval [CI], -10.48 to -3.88), balance during eyes close by -1.40 (95% CI, -2.42 to -0.39), and dizziness by -1.47 (95% CI, -2.74 to -0.21) compared with no intervention or placebo., Conclusion: The present study demonstrated that early vestibular rehabilitation improved the Dizziness Handicap Inventory, balance (eyes close), and subjective dizziness in a patient with acute vestibular disorders. This result indicates that early vestibular rehabilitation can promote vestibular compensation., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Objective: Large vestibular aqueduct syndrome (LVAS) is one of the etiology of hearing loss. Clinically, we observed that the VA size of patients with idiopathic sudden sensorineural hearing loss (ISSNHL) did not meet the diagnostic criteria of VA enlargement, but there were individual variations. Through this study, we want to understand the VA development and explore its risk for suffering from ISSNHL., Methods: 74 patients with ISSNHL were retrospectively reviewed in our department from June 2018 to September 2021. Meanwhile, 57 people with no ear diseases were randomly selected as the control group. All their clinical information were systematically collected. The axial thin-slice CT images of temporal bone were used to observe and measure the VA in ISSNHL and controls. ISSNHL were classified as different types and grades according to pure tone audiometry and the degree of hearing loss, respectively. Logistic regression analysis was adopted to evaluate the risk factors of different types and grades of ISSNHL., Results: The operculum morphology could be funnel-shaped, tubular and invisible, but they had no statistical difference in the morbidity of ISSNHL. The operculum width of the affected sides in the case group was significantly wider than that of the matched sides in the control group (0.84±0.35mm vs 0.68±0.34mm, p=0.009), but the midpoint width had no statistical difference (p=0.447). The operculum width was an independent risk factor for the total hearing loss type (p=0.036, OR=4.49, 95% CI=1.10-18.29), moderate (p=0.013, OR=17.62, 95% CI=1.82-170.95) and profound (p=0.031, OR=4.50, 95% CI=1.14-17.67) grade of ISSNHL. Hypertension was an independent risk factor for the severe grade (p=0.004, OR=12.44, 95% CI=2.19-70.64) of ISSNHL. Both the operculum width (p=0.048, OR=7.14, 95% CI=1.02-50.26) and hypertension (p=0.014, OR=6.73, 95% CI=1.46-30.97) were the risk factors for the flat type of ISSNHL. The midpoint width of the VA, gender, age, diabetes mellitus, hyperlipidemia, and plasma fibrinogen concentration had no significant effect on the risk for suffering from ISSNHL., Conclusion: The development of the VA operculum is a risk factor for some types and grades of ISSNHL. Hypertension remained a risk factor for ISSNHL., Competing Interests: Declaration of competing interest The authors have no relevant financial interests to disclose., (Copyright © 2023. Published by Elsevier B.V.)

Objective: To assess changes in cognitive function in vestibular migraine patients undergoing treatment., Study Design: Prospective cohort., Setting: Single-institution tertiary-care center., Patients: Thirty-four patients with vestibular migraine were included in the study. Average age at diagnosis was 47.9 years. A majority of patients (91.2%) were female., Interventions: Vestibular therapies included pharmacologic treatment (67.6%), mindfulness-based stress reduction (58.8%), vestibular physical therapy (20.6%), and lifestyle changes only (2.9%)., Main Outcome Measures: Pretreatment and posttreatment questionnaires were collected including the Cognitive Failures Questionnaire (CFQ), Vestibular Migraine Patient Assessment Tool and Handicap Inventory (VM-PATHI), and Dizziness Handicap Inventory., Results: Median time between pretreatment and posttreatment questionnaire was 4.4 months (range, 2.8-15.6. mo). CFQ scores decreased in subjects who responded to treatment, as defined by those with a positive change in VM-PATHI score (average decrease, 6.5; p = 0.03). CFQ scores did not improve in subjects who had no improvement in their vestibular condition, as defined by no change or an increase in VM-PATHI score (average increase, 2.0; p = 0.53). Univariate linear regression showed that VM-PATHI score change was highly predictive of CFQ change ( p < 0.01, r2 = 0.36). Multivariate regression demonstrated that the VM-PATHI ( p = 0.03) and not the Dizziness Handicap Inventory ( p = 0.10) predicted changes in CFQ score., Conclusions: Self-reported cognitive dysfunction improves with successful treatment of vestibular migraine., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Introduction: The vestibular system - the sixth sense - allows humans to preserve balance, stable vision, and body orientation in space. This system performs a continuous integration of the activity of the vestibular end-organ in the inner ear, the sense of sight, and the proprioceptive sense. Damage to the components of the vestibular system causes dizziness, imbalance, and poor orientation in space. Following vestibular injury, central compensation mechanisms are recruited to reduce the symptoms and improve the functional ability of the dizzy patient. Dizziness is a subjective complaint. Yet, the development that has taken place in the field of vestibular function testing allows accurate diagnosis, targeted treatment, and disability assessment of the dizzy patient. The dizzy patient must be examined meticulously, taking into account all the levels on which the vestibular impairment can affect (physiological, behavioral, and functional). Meaning, clinical and laboratory evaluation of the vestibular function together with functional testing of the patient. In this article, the authors present the range of existing innovative tests of the vestibular system. Vestibular tests are accepted in advanced vestibular laboratories in Israel and around the world, and enable assimilation in vestibular clinics and medical committees in Israel.

Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognised but underdiagnosed cause of late-onset hereditary ataxia. Symptoms may vary, and differential diagnoses can span several specialties. We report the case of a man in his 60 s who presented with a 10 year history of imbalance and progressive gait disturbance associated with a chronic spasmodic cough that preceded these symptoms by almost 30 years. He had previously undergone extensive testing for acquired and genetic causes of ataxia without a conclusive diagnosis. Brain MRI revealed cerebellar atrophy, and nerve conduction tests suggested a sensory ganglionopathy. Vestibular function testing was crucial for diagnosis, identifying a severe bilateral vestibulopathy. This led to the consideration of CANVAS, which was finally confirmed by genetic testing. This case raises awareness of this novel genetic disease, highlighting the importance of objective vestibular function tests in establishing an early diagnosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Vestibular migraine (VM) is accepted as the most common cause of spontaneous episodic vertigo. In most patients, vestibular symptoms follow migraine headaches that begin earlier in life. The aim of this multicenter retrospective study was to find out the differences between migraine patients without any vestibular symptoms (MwoV) and VM patients and to delineate the specific clinical features associated with VM. MwoV and VM patients were compared regarding demographic features, migraine headache years, headache attack frequency, intensity, symptoms associated with headache and vertigo attacks, presence of menopause, history of motion sickness and family history of migraine. Four-hundred and forty patients with MwoV and 408 patients with VM were included in the study. Migraine with aura was more frequent in patients with MwoV (p = 0.035). Migraine headache years was longer (p < 0.001) and headache intensity was higher in patients with VM (p = 0.020). Aural fullness/tinnitus was more common in patients with VM (p < 0.001) when all other associated symptoms were more frequent in patients with MwoV (p < 0.001) as well as attack triggers (p < 0.05). Presence of menopause and motion sickness history was reported more frequently by VM patients (p < 0.001). Logistic regression analysis indicated that longstanding history of migraine with severe headache attacks, aural fullness/tinnitus accompanying attacks, presence of menopause, previous motion sickness history were the differentiating clinical features of patients with VM., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

We report a case of a woman presenting with unilateral right profound hearing loss accompanied by vertigo secondary to barotrauma-induced perilymph fistula during recreational skydiving. Video head impulse test demonstrated a reduced gain in both the right horizontal and right anterior semicircular canals accompanied by frequently gathered overt corrective saccades. High-resolution computed tomography revealed an enlarged vestibular aqueduct on the affected side, a predisposing factor for the development of perilymph fistula. An exploratory tympanotomy was performed during which a perilymph leak was visualized at the round window niche. Temporal fascia patches enforced by absorbable gelatin sponges were applied to both round and oval windows. During post-surgery follow-up, the patient remained free of vestibular symptoms. An audiogram displayed mild improvement in the right ear speech reception threshold, although her hearing remained non-serviceable. The video head impulse test showed a favorable dynamic with a stepwise return to normal gain values in all semicircular canals and the disappearance of overt corrective saccades. This is the first case in which video head impulse test was employed as a valuable diagnostic tool for the evaluation and post-surgery follow-up of vestibular function in a barotrauma-induced perilymph fistula. The demonstration of an enlarged vestibular aqueduct on high-resolution computed tomography and the risk of perilymph fistula recurrence are discussed.

This qualitative systematic review presents an overview of the state of the research relating to visual motion hypersensitivity (VMH) and offers a reference tool for future studies in the field. The study set out to identify and collate articles investigating risk groups with aberrant responses to visual motion as compared to healthy control groups, presenting evidence for risk factors associated with visual motion hypersensitivity. Data were synthesized into the state of the research and analyzed in the context of the clinical characteristics of each risk factor. Literature searches were performed on Medline Ovid, EMBASE, Web of Science, and Cinahl, identifying a total of 586 studies of which 54 were finally included. Original articles published between the dates of commencement for each database and 19th January 2021 were included. JBI critical appraisal tools were implemented for each corresponding article type. In total, the following number of studies was identified for each respective risk factor: age (n = 6), migraines (n = 8), concussions (n = 8), vestibular disorders (n = 13), psychiatric conditions (n = 5), and Parkinson's disease (n = 5). Several studies described VMH as the primary concern (n = 6), though these primarily included patients with vestibulopathies. There were considerable differences in the nomenclature employed to describe VMH, depending largely on the investigating group. An overview of investigated risk factors and their evaluation methods was presented in a Sankey diagram. Posturography was the most implemented methodology but due to diverse measurements meta-analyses were not possible. One may however note that while the easily implemented Vestibular Ocular Motor Screening (VOMS) was designed for concussed patients, it may prove useful for other risk groups., (© 2023. The Author(s).)

Background: Vertigo and dizziness in children can be multi-factorial. Vestibular function tests allow an improved differential diagnosis and treatment. Delay in diagnosis of the diverse etiologies causing dizziness can adversely affect the health of children and is a matter of concern for their families. This study analyzes the delay in diagnosis and the importance of establishing a diagnosis with detailed history and neuro-otological evaluation., Methods: A total of 241 children presenting with vertigo to a tertiary otoneurology clinic between January 2019 and April 2022 were analyzed for the duration between the onset of symptoms and diagnosis, presenting complaints, and characteristic findings., Results: Two hundred and forty-one patients with a mean age of 12.5 ± 3.02 years (range, 5-16 years) were evaluated. About 39.4% of patients were diagnosed after over a year (with some over 5 years) of suffering from vertigo and only 18.7% of patients were diagnosed correctly within 1 month of symptom onset. The presenting features were variable with 174 (72.2%) complaining of spinning, unsteadiness, and falls seen in 36+10+37 (34.4%). Vestibular migraine was the most common diagnosis (63.39%), followed by benign paroxysmal positional vertigo (24.48%), of which the posterior canal was most affected (50.85%) followed by horizontal (40.68%) and anterior canal (8.47%). Other etiologies noted were central (14.10%) and peripheral vestibulopathy (17.42%) and variable other causes (6.19%)., Conclusion: Many pediatric vertigo and dizziness patients do not reach the correct diagnosis for long durations and are treated as "unspecified dizziness." A detailed examination with a multidisciplinary approach including vestibular evaluation is advocated to give definitive treatment to these children.

We aim to assess if genotype-phenotype correlations are present within ocular manifestations of Kabuki syndrome (KS) among a large multicenter cohort. We conducted a retrospective, medical record review including clinical history and comprehensive ophthalmological examinations of a total of 47 individuals with molecularly confirmed KS and ocular manifestations at Boston Children's Hospital and Cincinnati Children's Hospital Medical Center. We assessed information regarding ocular structural, functional, and adnexal elements as well as pertinent associated phenotypic features associated with KS. For both type 1 KS (KS1) and type 2 KS (KS2), we observed more severe eye pathology in nonsense variants towards the C-terminus of each gene, KMT2D and KDM6A, respectively. Furthermore, frameshift variants appeared to be not associated with structural ocular elements. Between both types of KS, ocular structural elements were more frequently identified in KS1 compared with KS2, which only involved the optic disc in our cohort. These results reinforce the need for a comprehensive ophthalmologic exam upon diagnosis of KS and regular follow-up exams. The specific genotype may allow risk stratification of the severity of the ophthalmologic manifestation. However, additional studies involving larger cohorts are needed to replicate our observations and conduct powered analyses to more formally risk-stratify based on genotype, highlighting the importance of multicenter collaborations in rare disease research., (© 2023 Wiley Periodicals LLC.)

Objective: Vestibular migraine (VM) is the most common cause of episodic vertigo afflicts 1% of the general population. The complexity of VM is owing to the migrainous, and vestibular components and much knowledge have been gained in recent years on VM in the adult population. Akin to that, numerous studies focusing on VM in children and adolescent has emerged. We reviewed the literature to understand the characteristics and diagnostic approach of VM in children and adolescents., Methods: A literature search was conducted over a period of one month (April 2022)., Results: 16 articles were selected based on our objective and selection criteria. A total of patients was included, with a median age of 10.9 years. 11 studies diagnosed VM based on diagnostic criteria. Caloric test and electro/videonystagmography are the most favoured investigation used (50%). Imaging was performed in 56.2% of included studies., Conclusion: Deciphering the ideal diagnostic approach for VM is prudent to ensure children and adolescents suffering from VM are treated earlier. VM can be diagnosed using the established diagnostic criteria, which requires thorough and meticulous history taking. The available oto-neurological examination aims to exclude other disorders as its significance in diagnosing VM is still debatable., (Copyright © 2022. Published by Elsevier B.V.)

Objective: To review Politzerization and describe a complication that has not been reported previously., Patients: Case report., Interventions: Clinical details of Politzerization and a complication that has not been reported previously, and a brief review of relevant literature from 1861 to 2022., Main Outcome Measures: Round window fistula was caused by Politzerization., Conclusions: Although it is extremely rare, transnasal eustachian tube Politzerization can result in perilymph fistula., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2023, Otology & Neurotology, Inc.)

Dizziness or vertigo is a common clinical symptom, and its underlying etiology is complex. Many clinicians are confused about its diagnosis and treatment. This article presents a case about chronic vestibular syndrome. And case appreciation and academic discussion are conducted by well-known domestic neurologists and otologists, so as to provide a good thinking model and basic ideas for the diagnosis and treatment of dizziness or vertigo, hoping to further improve the diagnosis and treatment level among clinicians., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Background: Patients with dizziness are severely affected in their daily life. The dizziness may be caused by vestibular neuritis and this condition may be severe and result in hospitalization. Qualified municipal rehabilitation services are warranted for these patients after edischarge from the hospital. However, very few specialized municipal initiatives in Denmark are targeting this patient group., Methods: This paper reports on the development of a clinically applicable municipality-based vestibular neuritis rehabilitation program and evaluates the acceptability of this initiative., Results: The study recognized the need for a rehabilitation program after hospital discharge. However, the program was not evaluated as acceptable for multiple reasons. The exercise program was applicable and feasible but was experienced as a limitation for the practitioner, when addressing other balance issues was needed. It proved challenging to inform both the administrative staff and the clinicians about the new rehabilitation service to allow for sufficient implementation., Conclusion: Although the rehabilitation program was not considered an unequivocal success, there were several derived valuable snowball effects of the program. This paper advocates that focus should not only lie on the success of a single program, but also explore the derived benefits for patients and organizations, as well as the practice-oriented knowledge these programs generate.