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Laryngo- Rhino- Otologie Mar 2024Three forms of peripheral vestibular disorders, each with its typical symptoms and clinical signs, can be differentiated functionally, anatomically and...
Three forms of peripheral vestibular disorders, each with its typical symptoms and clinical signs, can be differentiated functionally, anatomically and pathophysiologically: 1. inadequate unilateral paroxysmal stimulation or rarely inhibition of the peripheral vestibular system, e. g., BPPV, Menière's disease, vestibular paroxysmia or syndrome of the third mobile windows; 2. acute unilateral vestibulopathy leading to an acute vestibular tone imbalance manifesting as an acute peripheral vestibular syndrome; and 3. loss or impairment of function of the vestibular nerve and/or labyrinth: bilateral vestibulopathy. For all of these diseases, current diagnostic criteria by the Bárány-Society are available with a high clinical and scientific impact, also for clinical trials. The treatment depends on the underlying disease. It basically consists of 5 principles: 1. Explaining the symptoms and signs, pathophysiology, aetiology and treatment options to the patient; this is important for compliance, adherence and persistence. 2. Physical therapy: A) For BPPV specific liberatory maneuvers, depending on canal involved. Posterior canal: The new SémontPLUS maneuver is superior to the regular Sémont and Epley maneuvers; horizontal canal: the modified roll-maneuver; anterior canal the modified Yacovino-maneuver; 3. Symptomatic or causative drug therapy. There is still a deficit of placebo-controlled clinical trials so that the level of evidence for pharmacotherapy is most often low. 4. Surgery, mainly for the syndrome of the third mobile windows. 5. Psychotherapeutic measures for secondary functional dizziness.
Topics: Humans; Vestibular Diseases; Vertigo; Meniere Disease; Bilateral Vestibulopathy; Acute Disease; Vestibule, Labyrinth
PubMed: 38134907
DOI: 10.1055/a-2144-3801 -
The Laryngoscope Jan 2024To explore the predictive factors between Meniere's disease (MD) and vestibular migraine (VM) by Gadolinium-enhanced Magnetic resonance imaging (MRI) of the inner ear...
OBJECTIVE
To explore the predictive factors between Meniere's disease (MD) and vestibular migraine (VM) by Gadolinium-enhanced Magnetic resonance imaging (MRI) of the inner ear and Clinical Features.
METHODS
Eighty-seven patients (50 MD and 37 VM) underwent intratympanic injection of gadolinium and MRI was performed 24 h later. All patients underwent pure tone audiometry and caloric tests.
RESULTS
In the MD group, 46 (92%) of 50 patients developed endolymphatic hydrops, although only 2 (5.4%) in the vestibular migraine (VM) group had positive results groups (p < 0.001). The incidence of migraine was 14% in the MD group and 67.7% in the VM group (p < 0.001). Multivariate logistic regression of the two groups of patients indicated that the greater the sum of the maximum slow phase velocity (SPV) of the ipsilateral ear, the higher the risk of VM occurrence (p = 0.009). The incidence of carsickness was positively correlated with the incidence of VM (p < 0.001) and asymmetric hearing loss (AHL) was negatively correlated with the diagnosis of VM (p = 0.045).
CONCLUSION
Gadolinium-enhanced MRI of the inner ear is helpful for the differential diagnosis of VM and MD. Carsickness, decreased AHL, and increased Sum of the maximum SPV in the ipsilateral side (SSPVI) may act as diagnostic predictors of VM.
LEVEL OF EVIDENCE
3 Laryngoscope, 134:426-432, 2024.
Topics: Humans; Meniere Disease; Gadolinium; Vertigo; Endolymphatic Hydrops; Motion Sickness; Magnetic Resonance Imaging; Migraine Disorders; Hearing Loss
PubMed: 37357969
DOI: 10.1002/lary.30858 -
Brazilian Journal of Otorhinolaryngology 2023To analyze, by means of a systematic review and meta-analysis, the proportion of patients with Meniere's disease who have altered caloric test and vHIT, as well as to... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To analyze, by means of a systematic review and meta-analysis, the proportion of patients with Meniere's disease who have altered caloric test and vHIT, as well as to determine the prevalence of altered caloric test and normal vHIT dissociation in the diagnosis of Meniere's disease.
METHODS
The literature search had no restriction regarding the period of publication on the following indexed data platforms: PubMed, PubMed PMC, BVS-Bireme, Web of Science, Embase and Cochrane Library. Articles that evaluated patients with Meniere's disease who underwent caloric test and vHIT were included. Two researchers independently conducted the analysis of the articles, promoting the selection and capture of data, following the recommendations of the PRISMA method, and complying with the criteria for articles inclusion and exclusion defined in the research protocol. In case of disagreement during the selection process, a third researcher was included for analysis.
RESULTS
From a total of 427 initial studies, the researchers selected 12 articles, published between 2014 and 2021, with a total of 708 patients evaluated, with a mean age of 52.72 years old. The prevalence of patients with Meniere's disease with altered caloric reflex test was 64% (95% CI 57%‒71%), while the prevalence of altered vHIT was only 28% (95% CI 16%-40%). The prevalence of the altered caloric test + normal vHIT dissociation was 47% (95% CI 37%-57%).
CONCLUSION
The video head impulse test and the caloric test are valuable tools for vestibular assessment. The dissociation of findings between these two tests in patients with Meniere's disease was more prevalent in this meta-analysis and may be a result of the tonotopy of specialized hair cells in the ampullary crest. The prevalence of altered caloric test was 64% and anormal vHIT was 28%. The dissociation caloric asymmetry and normal vHIT was observed in 47% of the patients.
Topics: Humans; Middle Aged; Meniere Disease; Head Impulse Test; Caloric Tests; Reflex, Vestibulo-Ocular; Hair Cells, Auditory
PubMed: 37354884
DOI: 10.1016/j.bjorl.2023.101279 -
Scientific Reports Oct 2023Ménière's disease (MD) is associated with functional reorganization not only in the auditory or sensory cortex but also in other control and cognitive areas. In this...
Ménière's disease (MD) is associated with functional reorganization not only in the auditory or sensory cortex but also in other control and cognitive areas. In this study, we examined intranetwork and internetwork connectivity differences between 55 MD patients and 70 healthy controls (HC) in 9 well-defined resting-state networks. Functional connectivity degree was lower in MD compared to HC in 19 brain areas involved in the somatomotor, auditory, ventral attention, default mode, limbic, and deep gray matter networks. In addition, we observed lower intranetwork connectivity in the auditory, ventral attention, and limbic networks, as well as lower internetwork connectivity between the somatomotor and limbic networks, and between the auditory and somatomotor, deep gray matter, and ventral attention networks, and between the deep gray matter and default mode network. Furthermore, we identified 81 pairs of brain areas with significant differences in functional connectivity between MD patients and HC at the edge level. Notably, the left amygdala's functional connectivity degree was positively correlated with MD's disease stage, and the ventral attention network's intranetwork connectivity was positively correlated with the healthy side vestibular ratio. Our findings suggest that these functional network reorganization alterations may serve as potential biomarkers for predicting clinical progression, evaluating disease severity, and gaining a better understanding of MD's pathophysiology. Large-scale network studies using neuroimaging techniques can provide additional insights into the underlying mechanisms of MD.
Topics: Humans; Brain Mapping; Meniere Disease; Magnetic Resonance Imaging; Brain; Neuroimaging; Neural Pathways
PubMed: 37798378
DOI: 10.1038/s41598-023-44090-x -
Otology & Neurotology Open Sep 2023Vestibular migraine represents a growing public health problem, imposing enormous societal burdens in the form of patient suffering, loss of productivity, and direct...
Vestibular migraine represents a growing public health problem, imposing enormous societal burdens in the form of patient suffering, loss of productivity, and direct healthcare costs. This raises the question of how we developed our ideas about vestibular migraine and how these ideas shape how we treat it. This review walks through the history of how our conceptualization of migraine and vestibular symptoms evolved, starting with clinical observations in ancient times, inclusion under the umbrella of Meniere's disease, and then separation from Meniere's disease with its own identity. Tradition, clinical observations, and diagnostic criteria developed by professional societies have played prominent roles in building our current concept of vestibular migraine. A review of the ideas that have shaped our current conception of vestibular migraine may help us to see which ones have stood the test of time and which ones should continue to evolve. As in other disciplines, we study history in medicine to be inspired, warned, and sometimes, to be freed.
PubMed: 38515642
DOI: 10.1097/ONO.0000000000000040 -
Frontiers in Molecular Neuroscience 2023Hearing loss is one of the 10 leading causes of disability worldwide. No drug therapies are currently available to protect or restore hearing. Inner ear auditory hair...
Hearing loss is one of the 10 leading causes of disability worldwide. No drug therapies are currently available to protect or restore hearing. Inner ear auditory hair cells and the blood-labyrinth barrier (BLB) are critical for normal hearing, and the BLB between the systemic circulation and stria vascularis is crucial for maintaining cochlear and vestibular homeostasis. BLB defects are associated with inner ear diseases that lead to hearing loss, including vascular malformations, inflammation, and Meniere's disease (MD). Antibodies against proteins in the inner ear and cytokines in the cochlea, including IL-1α, TNF-α, and NF-kβ, are detected in the blood of more than half of MD patients. There is also emerging evidence of inner ear inflammation in some diseases, including MD, progressive sensorineural hearing loss, otosclerosis, and sudden deafness. Here, we examined the effects of TNF-α, IL6, and LPS on human stria vascularis-derived primary endothelial cells cultured together with pericytes in a Transwell system. By measuring trans-endothelial electrical resistance, we found that TNF-α causes the most significant disruption of the endothelial barrier. IL6 had a moderate influence on the barrier, whereas LPS had a minimal impact on barrier integrity. The prominent effect of TNF-α on the barrier was confirmed in the expression of the major junctional genes responsible for forming the tight endothelial monolayer, the decreased expression of and . We further tested permeability using 2 μg of daptomycin (1,619 Da), which does not pass the BLB under normal conditions, by measuring its passage through the barrier by HPLC. Treatment with TNF-α resulted in higher permeability in treated samples compared to controls. LPS-treated cells behaved similarly to the untreated cells and did not show differences in permeability compared to control. The endothelial damage caused by TNF-α was confirmed by decreased expression of an essential endothelial proteoglycan, syndecan1. These results allowed us to create an inflammatory environment model that increased BLB permeability in culture and mimicked an inflammatory state within the stria vascularis.
PubMed: 37808472
DOI: 10.3389/fnmol.2023.1243370 -
Journal of Biomedical Informatics Jul 2023The diagnosis of rare genetic diseases is often challenging due to the complexity of the genetic underpinnings of these conditions and the limited availability of... (Review)
Review
BACKGROUND
The diagnosis of rare genetic diseases is often challenging due to the complexity of the genetic underpinnings of these conditions and the limited availability of diagnostic tools. Machine learning (ML) algorithms have the potential to improve the accuracy and speed of diagnosis by analyzing large amounts of genomic data and identifying complex multiallelic patterns that may be associated with specific diseases. In this systematic review, we aimed to identify the methodological trends and the ML application areas in rare genetic diseases.
METHODS
We performed a systematic review of the literature following the PRISMA guidelines to search studies that used ML approaches to enhance the diagnosis of rare genetic diseases. Studies that used DNA-based sequencing data and a variety of ML algorithms were included, summarized, and analyzed using bibliometric methods, visualization tools, and a feature co-occurrence analysis.
FINDINGS
Our search identified 22 studies that met the inclusion criteria. We found that exome sequencing was the most frequently used sequencing technology (59%), and rare neoplastic diseases were the most prevalent disease scenario (59%). In rare neoplasms, the most frequent applications of ML models were the differential diagnosis or stratification of patients (38.5%) and the identification of somatic mutations (30.8%). In other rare diseases, the most frequent goals were the prioritization of rare variants or genes (55.5%) and the identification of biallelic or digenic inheritance (33.3%). The most employed method was the random forest algorithm (54.5%). In addition, the features of the datasets needed for training these algorithms were distinctive depending on the goal pursued, including the mutational load in each gene for the differential diagnosis of patients, or the combination of genotype features and sequence-derived features (such as GC-content) for the identification of somatic mutations.
CONCLUSIONS
ML algorithms based on sequencing data are mainly used for the diagnosis of rare neoplastic diseases, with random forest being the most common approach. We identified key features in the datasets used for training these ML models according to the objective pursued. These features can support the development of future ML models in the diagnosis of rare genetic diseases.
Topics: Humans; Rare Diseases; Machine Learning; Algorithms; Genomics; Prognosis
PubMed: 37352901
DOI: 10.1016/j.jbi.2023.104429 -
The Laryngoscope Oct 2023Despite its relatively high prevalence, our understanding of the natural clinical course of acute low-tone hearing loss (ALHL) without vertigo remains incomplete. The... (Review)
Review
OBJECTIVE
Despite its relatively high prevalence, our understanding of the natural clinical course of acute low-tone hearing loss (ALHL) without vertigo remains incomplete. The purpose of this study is to summarize the findings of studies that evaluated recovery from hearing loss (HL), recurrence and/or fluctuation of HL, and progression to Meniere's Disease (MD) of patients presenting with ALHL without vertigo.
METHODS
A scoping review of the English literature was performed. On May 14, 2020 and July 6, 2022, MEDLINE, Embase, and Scopus were searched to identify articles related to the prognosis of ALHL. To be included, articles had to present outcomes that were clearly distinguishable for patients with ALHL without vertigo. Two reviewers evaluated articles for inclusion and extracted data. Disagreements were adjudicated by a third reviewer.
RESULTS
Forty-one studies were included. There was extensive heterogeneity between studies in regard to defining ALHL, treatment methods, and time of follow-up. Most of the cohorts (39 out of 40) reported partial or complete recovery of hearing in the majority (>50%) of patients, although reports of recurrence were relatively common. Progression to MD was infrequently reported. Shorter time from onset of symptoms to treatment predicted better hearing outcomes in 6 of 8 studies.
CONCLUSION
The literature suggests that although the majority of patients with ALHL experience hearing improvement, recurrence and/or fluctuation are common, and progression to MD occurs in a minority of patients. Additional trials utilizing standardized inclusion and outcome criteria are needed to determine the ideal treatment for ALHL.
LEVEL OF EVIDENCE
NA Laryngoscope, 133:2457-2469, 2023.
Topics: Humans; Hearing Loss, Sensorineural; Hearing Loss; Meniere Disease; Vertigo; Prognosis; Deafness
PubMed: 36880419
DOI: 10.1002/lary.30630 -
Otology & Neurotology : Official... Oct 2023To identify key risk factors for the development of bilateral Ménière's disease. (Observational Study)
Observational Study
OBJECTIVE
To identify key risk factors for the development of bilateral Ménière's disease.
STUDY DESIGNS
Observational study.
SETTING
Four NHS Trusts and four independent hospitals or clinics, within three distinct urban and rural regions within the United Kingdom (Norfolk, Leicestershire, and London).
METHODS
Patients with Ménière's disease were identified at ENT or audiovestibular medicine secondary/tertiary care and specialist private clinics. A range of patient-reported data, questionnaire data, and clinical data (audiometric, radiological, and specialist balance testing data) was inputted into a bespoke database. A logistic regression model was used to identify potential risk factors for bilateral Ménière's disease compared with unilateral Ménière's disease.
RESULTS
A total of 411 participants were recruited into this study, 263 from NHS Trusts and 148 from independent hospitals or clinics. In our cohort of patients, 22% of individuals were identified as having bilateral Ménière's disease. Two statistically significant independent variables were identified as risk factors for the development of bilateral Ménière's disease: the presence of psoriasis and a history of ear infections.
CONCLUSIONS
Psoriasis and a history of ear infection have been identified as key risk factors for the development of bilateral Ménière's disease. It is anticipated that further work based on this finding will allow a better understanding of the underlying pathophysiological mechanisms that predispose to the development of Ménière's disease symptoms.
Topics: Humans; Meniere Disease; Databases, Factual; Logistic Models; Psoriasis; Risk Factors
PubMed: 37590874
DOI: 10.1097/MAO.0000000000003984