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BackgroundVestibular migraine (VM) is one of the most common causes of recurrent vertigo and presents with a history of spontaneous or positional vertigo with a history of migraine headaches. While research has identified a high prevalence of migraine headaches and vestibular deficits among fragile X premutation carriers, there has been no discussion about VM within this population.ObjectiveThis case series and review seeks to describe the clinical characteristics and pathophysiology of VM among individuals with the fragile X premutation. We also seek to discuss treatment and future steps in addressing VM in this population.MethodsA review of the literature regarding vestibular migraine and presentation of migraine headaches and vestibular deficits among premutation carriers was performed. A detailed clinical history of migraine headaches and vertigo was obtained from three patients with the fragile X premutation seen by the senior author (RJH).ResultsAll three cases first developed symptoms of migraine headaches earlier in life, with the development of VM near menopause. Two of the three cases developed progressive balance issues following the development of VM. All three cases found that their VM episodes were improved or resolved with pharmacological and/or lifestyle interventions.ConclusionsIt is important to recognize VM among premutation carriers because beneficial treatments are available. Future studies are needed regarding the prevalence of VM and the relationship to subsequent FXTAS. The pathophysiology of VM remains uncertain but possibilities include mitochondrial abnormalities, cranial nerve VIII toxicity secondary to neurotoxic protein accumulation, and calcitonin gene-related peptide (CGRP) signaling dysfunction due to altered levels of fragile X messenger ribonucleoprotein (FMRP).

Purpose: This retrospective study aimed to analyze the symptom profile of Meniere's disease (MD) patients, particularly focusing on the cessation of episodic vertigo and the disease's longitudinal course and the impact of major symptoms on quality of life (QoL). Methods: The study employed a cross-sectional design and was conducted on 365 out of 560 individuals with definite MD from the Finnish Vestibular and Meniere Federation, utilizing an internet-based questionnaire. Participants were surveyed on vertigo attacks, vestibular drop attacks (VDA), balance issues, selective cognitive complaints, hearing loss, and their effects on overall quality of life (QoL). The study population comprised 79.5% females and 20.5% males, with a mean age of 63 years and an average disease duration of 15.2 years. Results: The onset of MD was characterized by simultaneous hearing loss, vertigo, and tinnitus in 38% of participants. There was a significant delay in diagnosis for many, with 20% experiencing a delay of over 5 years. The frequency and duration of vertigo attacks generally decreased over time, with attacks becoming shorter and less severe as the disease progressed. Spontaneous remission from episodic vertigo occurred in 34% of participants variably throughout the course of MD. Of the participants 65.5% reported balance issues, and 34% experienced mild VDAs, with severe falls occurring in 10%. VDAs were more common with longer disease duration. Bilateral hearing loss developed in 34.5% of participants over the long term, with a higher risk associated with younger onset age, migraines, and family history of MD. Fatigue, anxiety, and depression were prevalent, particularly among younger participants. Cognitive impairments were linked to the severity of these symptoms and the presence of constant dizziness. QoL was significantly lower among participants with constant dizziness, with factors like fatigue, depression, VDA, and hearing loss contributing to this reduction. Conclusions: The study highlights the complexity of MD. While vertigo may spontaneously remit, other symptoms such as VDAs, balance issues, cognitive complaints, and hearing loss often persist and worsen over time. Assessing MD solely on primary symptoms like vertigo and hearing loss is insufficient; a comprehensive evaluation is necessary for effective management. [ABSTRACT FROM AUTHOR]

Background: This research aimed to ascertain independent risk factors and the diagnostic value of vascular parameters in differentiating posterior circulation ischemic isolated vertigo (PCI-IV) from vestibular peripheral vertigo (VPV). Methods: This study involved 247 patients with acute-onset vertigo, categorized into two groups: PCI-IV and VPV. Multivariate logistic regression was conducted to pinpoint independent risk factors for PCI-IV. Results: The duration of vertigo, particularly episodes lasting more than a few hours, was a significant predictor of PCI-IV (OR = 2.183, p < 0.001). The presence of diabetes mellitus (OR = 1.746, p = 0.008) and hypertension (OR = 2.291, p = 0.004) also notably increased the likelihood of PCI-IV. Hemodynamic measurements such as the inner diameter and average blood flow velocity (Vmean) of the vertebral artery, as well as the resistive index (RI), were identified as significant predictive factors (p ≤ 0.033). The ROC analysis demonstrated the vertebral artery RI had the highest diagnostic accuracy with an area under the curve (AUC) of 0.78, indicating an optimal balance between sensitivity and specificity. Conclusion: Clinical features such as the duration of vertigo, diabetes mellitus, and hypertension, along with vascular hemodynamics, are crucial in assessing the risk of PCI-IV. The RI in the vertebral artery emerged as a particularly potent diagnostic parameter. These findings advocate a multifaceted diagnostic approach, combining clinical and vascular parameters for the effective identification and management of PCI-IV. [ABSTRACT FROM AUTHOR]

Introduction: Knowledge of the natural and temporal course of a disease is important when deciding if an intervention is appropriate. In the case of Ménière's disease (MD), there is some evidence that attacks diminish over time, but the topic remains controversial. A conservative approach to surgery is usually followed in northern Europe, and leads to strict patient selection before considering surgery. Here, we describe the evolution of vertigo attacks among a group of intractable MD patients in whom surgery was considered. Methods: Retrospective cohort study in a Ménière's disease expert center. Patients with definite unilateral Ménière's disease and persisting vertigo attacks despite treatment with intratympanic steroid injections were included. All patients had been waitlisted for participation in a planned trial assessing non-ablative surgery. They were waitlisted between June 2016 and June 2021 without undergoing the surgical intervention. In September 2022, data were collected from patient's files and follow-up telephone interviews were conducted to assess the evolution of their vertigo attacks. Results: Thirty-five patients (54% male, mean age of onset 52 years, 51% right sided) were included in the analysis. Twenty-five patients (71%) eventually declined surgery. Of the 33 patients with complete information on vertigo attacks, 21 (64%) were free of vertigo attacks upon data collection, after a median disease duration of 5.3 years. Patients who did undergo surgery, had longer duration of disease than patients who did not. Discussion: Even in a population with intractable MD, most patients will experience relief of symptoms over time. On one hand, active treatment may accelerate relief of symptoms, but on the other hand, non-ablative therapies are of debatable effect and ablative intervention carries a risk of life long side effects. Therefore, any active intervention should be carefully considered. [ABSTRACT FROM AUTHOR]

The document discusses a case study of a 45-year-old woman with bilateral cupulolithiatic benign paroxysmal positional vertigo (BPPV) involving both posterior semicircular canals. The patient experienced vertigo and nystagmus during specific head movements, which were successfully treated with canalith repositioning maneuvers. The study highlights the diagnostic challenges and implications of set-point adaptation in cupulolithiasis. The findings suggest that bilateral BPPV, including cupulolithiasis, can occur spontaneously and may require specific diagnostic maneuvers for accurate diagnosis. [Extracted from the article]

Martin Luther (1483–1546) reported attacks of headache and of vertigo in his letters and in his lectures. The symptomatology of his headache attacks fulfilled, at least in part, the diagnostic semiological criteria of migraine. However, because we cannot be sure about the time pattern and the exclusion of other disorders that might explain the headache, the diagnosis of migraine is not final. The vertigo attacks sometimes fulfilled the criteria of Ménière's disease. Vertigo also occurred together with headache attacks fulfilling the International Classification of Headache Disorders, 3rd edition criteria for vestibular migraine; however, we do not know exactly the pattern of attack frequency and duration. In summary, it is possible that Martin Luther suffered from attacks of vestibular migraine and had in addition a comorbidity of migraine and Ménière's disease. [ABSTRACT FROM AUTHOR]

Vestibular compensation is a physiological response of the vestibular organs within the inner ear. This adaptation manifests during consistent exposure to acceleration or deceleration, with the vestibular organs incrementally adjusting to such changes. The molecular underpinnings of vestibular compensation remain to be fully elucidated, yet emerging studies implicate associations with neuroplasticity and signal transduction pathways. Throughout the compensation process, the vestibular sensory neurons maintain signal transmission to the central equilibrium system, facilitating adaptability through alterations in synaptic transmission and neuronal excitability. Notable molecular candidates implicated in this process include variations in ion channels and neurotransmitter profiles, as well as neuronal and synaptic plasticity, metabolic processes, and electrophysiological modifications. This study consolidates the current understanding of the molecular events in vestibular compensation, augments the existing research landscape, and evaluates contemporary therapeutic strategies. Furthermore, this review posits potential avenues for future research that could enhance our comprehension of vestibular compensation mechanisms. [ABSTRACT FROM AUTHOR]

Objectives: The Epley maneuver (EM) shows immediate effect, wherein disappearance of positional nystagmus occurs soon after the EM. Our previous study showed that setting interval times during the EM reduced the immediate effect. The purpose of this study is to identify the head position for which interval time reduces the immediate effect. Methods: Fifty-one patients with posterior canal type of benign paroxysmal positional vertigo (BPPV) were randomly assigned to the following three groups: 10 min interval time set at the first head position of the EM in group A, at the third head position in group B, and at the fourth head position in group C. The primary outcome measure (POＭ) was the ratio of maximum slow-phase eye velocity of positional nystagmus soon after the EM, compared with that measured before the EM. A large ratio value indicates a poor immediate effect of the EM. Results: The POＭ in group A (0.07) was smallest (B: 0.36, C: 0.49) (p < 0.001). Discussion: The interval times at the third and fourth head positions reduced the immediate effect of the EM. Our previous study showed that the effect of BPPV fatigue is continued by maintaining the first head position of the EM. BPPV fatigue constitutes fatigability of positional nystagmus with repeated performance of the Dix-Hallpike test. Our findings may be interpreted in accordance with the theory that the immediate effect of the EM is BPPV fatigue itself, because we observed that the effect of BPPV fatigue is strongest in group A. [ABSTRACT FROM AUTHOR]

Purpose: This study introduces and evaluates the sitting lateral canal maneuver (SLCM), a novel seated repositioning technique for treating geotropic lateral canal benign paroxysmal positional vertigo (BPPV). Methods: We conducted a retrospective chart review at the Hospital of Salerno, focusing on 26 patients diagnosed with geotropic LC-BPPV between 2021 and 2022. The SLCM was applied, and its efficacy was assessed based on the resolution of nystagmus and vertigo symptoms. A 95% confidence interval was calculated to estimate the success rate. Results: The SLCM demonstrated a high success rate, with 22 out of 26 patients (approximately 85%, 22/26 patients) showing positive outcomes. The 95% confidence interval for the success rate ranged from approximately 65.02–100%. These findings suggest that SLCM is a potentially effective intervention for LC-BPPV, especially beneficial for patients who find traditional supine or lateral maneuvers uncomfortable. Conclusion: The SLCM represents a promising alternative to traditional BPPV maneuvers, especially for patients requiring a seated approach. While the initial results are encouraging, further research with larger sample sizes and longer follow-up periods is needed to validate its efficacy and explore its full potential in the management of LC-BPPV. Level of evidence: This study represents a Level IV source of evidence, as defined by the evidence-based practice guidelines. It is a retrospective chart review that involves a moderate cohort of patients diagnosed with geotropic horizontal positional nystagmus consistent with lateral canal benign paroxysmal positional vertigo (LC-BPPV). While the study provides valuable insights into the efficacy of the sitting lateral canal maneuver (SLCM) and contributes to the existing literature on BPPV management, it is important to note the inherent limitations associated with this level of evidence. [ABSTRACT FROM AUTHOR]

Introduction: Benign positional paroxysmal vertigo (BPPV) stands as the commonest cause for vertigo. It accounts for 20% of all cases of vertigo, even with its high prevalence rate it often goes underdiagnosed and undertreated. Development of the consensus document by the Bárány society's International Classification of Vestibular Disorders (ICVD)significantly facilitates the diagnosis of BPPV and its variants. This study assesses the utilisation of ICVD criteria for managing BPPV. Methodology: This is a cross-sectional descriptive study conducted at a tertiary care hospital in Northern India spanning from November 1, 2022, to November 30, 2023. A total of 110 participants diagnosed with BPPV were enrolled consecutively. All participants underwent Dix-Hallpike and supine log roll positional maneuvers. Diagnosis was made based on the history and type of nystagmus seen, and classified as per the ICVD criteria. Results: Posterior semicircular canalolithiasis (pc-BPPV) accounted for 25.45% of cases and horizontal canal canalolithiasis (hc-BPPV) accounted for 20.91% of cases. Probable BPPV, spontaneously resolved (pBPPVsr) was diagnosed in 16.36% of participants and possible BPPV(pBPPV) was diagnosed in 18.18% of participants. Multiple canal BPPV (mc-BPPV) accounted for 17.27% of cases. One participant was diagnosed with horizontal canal cupulolithiasis and anterior canal canalolithiasis respectively. No participant was diagnosed with posterior canal cupulolithiasis. Conclusion: The most common type of BPPV was pc-BPPV followed by hc-BPPV. The affected canal in possible BPPV, can be identified, and appropriate repositioning maneuvers are effective in treating them as well as aids in confirming the diagnosis. The diagnostic clarity provided by ICVD, aids in effective management of BPPV. More studies with larger sample size are required to further validate its clinical utility. [ABSTRACT FROM AUTHOR]

Purpose: This study aimed to investigate the hearing outcomes in patients with sudden sensorineural hearing loss without vertigo (SSNHLwoV). Methods: Patients with SSNHLwoV managed from December 2016 to March 2020 were prospectively enrolled in an academic tertiary referral center. Fifty-one patients with SSNHLwoV who completed high-dose steroid treatment. The hearing prognosis was analyzed using a multivariate Cox regression model. Results: The rates of complete, partial, and no hearing recovery were 52.9%, 17.6%, and 29.4% in patients with SSNHLwoV, respectively. The video head impulse test (vHIT) of the posterior semicircular canal (PSC), high-tone hearing loss (4–8 kHz) ≥ 30 dB, and average hearing threshold (0.5–1–2–4 kHz) were significantly associated with incomplete recovery of hearing after treatment. In multivariate analysis, the vHIT of the PSC (hazard ratio [HR], 14.502; 95% confidence interval [CI], 1.371–153.355) and high-tone hearing loss ≥ 30 dB (HR, 9.170; 95% CI, 2.283–36.830) remained robust. Conclusions: Abnormal vestibular function tests were performed in 80.4% of the patients with SSNHLwoV. Abnormal vHIT of the PSC and high-tone hearing loss ≥ 30 dB were independent factors resulting in incomplete recovery of hearing in patients with SSNHLwoV. In the SSNHLwoV cohort, the caloric test was not significantly associated with hearing prognosis, and vHIT was a feasible predictor of treatment outcome. [ABSTRACT FROM AUTHOR]

Potassium channel mutations play an important role in neurological diseases, such as spinocerebellar ataxia (SCA). SCA is a heterogeneous autosomal-dominant neurodegenerative disorder with multiple sub-entities, such as SCA13, which is characterized by mutations in the voltage-gated potassium channel Kv3.3 (KCNC3). In this study, we present a rare and atypical case of SCA13 with a predominant episodic central rotational vertigo, while the patient suffered only from mild progressive cerebellar symptoms, such as dysarthria, ataxia of gait and stand, and recently a cognitive impairment. In this patient, we identified a heterozygous variant in KCNC3 (c.2023G > A, p.Glu675Lys) by next-generation sequencing. This Kv3.3E675K variant was studied using voltage-clamp recordings in Xenopus oocytes. While typical SCA13 variants are dominant-negative, show shifts in the voltage-dependence of activation or an altered TBK1 regulation, the Kv3.3E675K variant caused only a reduction in current amplitude and a more pronounced cumulative inactivation. Thus, the differences to phenotypes observed in patients with classical SCA13 mutations may be related to the mechanism of the observed Kv3.3 loss-of-function. Treatment of our patient with riluzole, a drug that is known to also activate potassium channels, turned out to be partly beneficial. Strikingly, we found that the Kv3.3 and Kv3.3E675K inactivation and the frequency-dependent cumulative inactivation was antagonized by increased extracellular potassium levels. Thus, and most importantly, carefully elevated plasma potassium levels in the physiological range, or novel drugs attenuating Kv3.3 inactivation might provide novel therapeutic approaches to rescue potassium currents of SCA13 variants per se. In addition, our findings broaden the phenotypic spectrum of Kv3.3 variants, expanding it to atypical phenotypes of Kv3.3-associated neurological disorders. [ABSTRACT FROM AUTHOR]