Vestibular Aqueduct Morphology and Meniere's Disease-Development of the "Vestibular Aqueduct Score" by 3D Analysis.
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ABSTRACT:
SUBMITTER: Noyalet L
PROVIDER: S-EPMC8854222 | biostudies-literature | 2022
REPOSITORIES: biostudies-literature
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Vestibular Aqueduct Morphology and Meniere's Disease-Development of the "Vestibular Aqueduct Score" by 3D Analysis.
Noyalet Laurent L Ilgen Lukas L Bürklein Miriam M Shehata-Dieler Wafaa W Taeger Johannes J Hagen Rudolf R Neun Tilmann T Zabler Simon S Althoff Daniel D Rak Kristen K
Frontiers in surgery 20220204
Improved radiological examinations with newly developed 3D models may increase understanding of Meniere's disease (MD). The morphology and course of the vestibular aqueduct (VA) in the temporal bone might be related to the severity of MD. The presented study explored, if the VA of MD and non-MD patients can be grouped relative to its angle to the semicircular canals (SCC) and length using a 3D model. Scans of temporal bone specimens (TBS) were performed using micro-CT and micro flat panel volume ...[more]
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Project description:HypothesisThe vestibular aqueduct (VA) in Menière's disease (MD) exhibits different angular trajectories depending on the presenting endolymphatic sac (ES) pathology, i.e., 1) ES hypoplasia or 2) ES degeneration.BackgroundHypoplasia or degeneration of the ES was consistently found in inner ears affected by MD. The two etiologically distinct ES pathologies presumably represent two disease "endotypes," which may be associated with different clinical traits ("phenotypes") of MD. Recognizing these endotypes in the clinical setting requires a diagnostic tool.Methods1) Defining the angular trajectory of the VA (ATVA) in the axial plane. 2) Measuring age-dependent normative data for the ATVA in postmortem temporal bone histology material from normal adults and fetuses. 3) Validating ATVA measurements from normative CT imaging data. 4) Correlating the ATVA with different ES pathologies in histological materials and CT imaging data from MD patients.Results1) The ATVA differed significantly between normal adults and MD cases with ES degeneration, as well as between fetuses and MD cases with ES hypoplasia; 2) a strong correlation between ATVA measurements in histological sections and CT imaging data was found; 3) a correlation between the ATVA, in particular its axial trajectory in the opercular region (angle αexit), with degenerative (αexit < 120°) and hypoplastic ES pathology (αexit > 140°) was demonstrated.ConclusionWe established the ATVA as a radiographic surrogate marker for ES pathologies. CT-imaging-based determination of the ATVA enables endotyping of MD patients according to ES pathology. Future studies will apply this method to investigate whether ES endotypes distinguish clinically meaningful subgroups of MD patients.
| S-EPMC6554006 | biostudies-literature
Project description:Two histopathological subtypes of Meniere's disease (MD) were recently described in a human post-mortem pathology study. The first subtype demonstrated a degenerating distal endolymphatic sac (ES) in the affected inner ear (subtype MD-dg); the second subtype (MD-hp) demonstrated an ES that was developmentally hypoplastic. The two subtypes were associated with different clinical disease features (phenotypes), suggesting that distinct endotype-phenotype patterns exist among MD patients. Therefore, clinical endotyping based on ES pathology may reveal clinically meaningful MD patient subgroups. Here, we retrospectively determined the ES pathologies of clinical MD patients (n = 72) who underwent intravenous delayed gadolinium-enhanced inner ear magnetic resonance imaging using previously established indirect radiographic markers for both ES pathologies. Phenotypic subgroup differences were evidenced; for example, the MD-dg group presented a higher average of vertigo attacks (ratio of vertigo patterns daily/weekly/other vs. monthly, MD-dg: 6.87: 1; MD-hp: 1.43: 1; p = 0.048) and more severely reduced vestibular function upon caloric testing (average caloric asymmetry ratio, MD-dg: 30.2% ± 30.4%; MD-hp: 13.5% ± 15.2%; p = 0.009), while the MD-hp group presented a predominantly male sex ratio (MD-hp: 0.06:1 [f/m]; MD-dg: 1.2:1 [f/m]; p = 0.0004), higher frequencies of bilateral clinical affection (MD-hp: 29.4%; MD-dg: 5.5%; p = 0.015), a positive family history for hearing loss/vertigo/MD (MD-hp: 41.2%; MD-dg: 15.7%; p = 0.028), and radiographic signs of concomitant temporal bone abnormalities, i.e., semicircular canal dehiscence (MD-hp: 29.4%; MD-dg: 3.6%; p = 0.007). In conclusion, this new endotyping approach may potentially improve the diagnosis, prognosis and clinical decision-making for individual MD patients.
| S-EPMC6459933 | biostudies-literature
Project description:ObjectivesTo evaluate the diagnostic performance and reliability of MRI descriptors used for the detection of Ménière's disease (MD) on delayed post-gadolinium MRI. To determine which combination of descriptors should be optimally applied and whether analysis of the vestibular aqueduct (VA) contributes to the diagnosis.Materials and methodsThis retrospective single centre case-control study evaluated delayed post-gadolinium MRI of patients with Ménièriform symptoms examined consecutively between Dec 2017 and March 2023. Two observers evaluated 17 MRI descriptors of MD and quantified perilymphatic enhancement (PLE) in the cochlea. Definite MD ears according to the 2015 Barany Society criteria were compared to control ears. Cohen's kappa and diagnostic odds ratio (DORs) were calculated for each descriptor. Forward stepwise logistic regression determined which combination of MRI descriptors would best predict MD ears, and the area under the receiver operating characteristic curve for this model was measured.ResultsA total of 227 patients (mean age 48.3 ± 14.6, 99 men) with 96 definite MD and 78 control ears were evaluated. The presence of saccular abnormality (absent, as large as or confluent with the utricle) performed best with a DOR of 292.6 (95% confidence interval (CI), 38.305-2235.058). All VA descriptors demonstrated excellent reliability and with DORs of 7.761 (95% CI, 3.517-17.125) to 18.1 (95% CI, 8.445-39.170). Combining these saccular abnormalities with asymmetric cochlear PLE and an incompletely visualised VA correctly classified 90.2% of cases (sensitivity 84.4%, specificity 97.4%, AUC 0.938).ConclusionEither absent, enlarged or confluent saccules are the best predictors of MD. Incomplete visualisation of the VA adds value to the diagnosis.Clinical relevance statementA number of different MRI descriptors have been proposed for the diagnosis of Ménière's disease, but by establishing the optimally performing MRI features and highlighting new useful descriptors, there is an opportunity to improve the diagnostic performance of Ménière's disease imaging.Key points• A comprehensive range of existing and novel vestibular aqueduct delayed post-gadolinium MRI descriptors were compared for their diagnostic performance in Ménière's disease. • Saccular abnormality (absent, confluent with or larger than the utricle) is a reliable descriptor and is the optimal individual MRI predictor of Ménière's disease. • The presence of this saccule descriptor or asymmetric perilymphatic enhancement and incomplete vestibular aqueduct visualisation will optimise the MRI diagnosis of Ménière's disease.
| S-EPMC11364795 | biostudies-literature
Project description:(1) Background: Vestibular migraine (VM) and Menière's disease (MD) share multiple features in terms of clinical presentations and auditory-vestibular functions, and, therefore, more accurate diagnostic tools to distinguish between the two disorders are needed. (2) Methods: The study was of retrospective design and examined the data of 69 MD patients, 79 VM patients and 72 MD with migraine patients. Five vestibular autorotation test (VAT) parameters, i.e., horizontal gain/phase, vertical gain/phase and asymmetry were subjected to logistic regression. The receiver operating characteristic (ROC) curves were generated to determine the accuracy of the different parameters in the differential diagnosis of MD and VM. (3) Results: Our results showed that the horizontal gain of VAT significantly outperformed other parameters in distinguishing MD and VM. In addition, the sensitivity, specificity and accuracy of the horizontal gain were 95.7%, 50.6% and 71.6%, respectively, for the differentiation between VM and MD. In most MD patients, the horizontal gain decreased in the range of 3-4 Hz, while in most VM patients, horizontal gain increased in the range between 2-3 Hz. More MD with migraine patients had an increased horizontal gain when the frequency was less than 5.0 Hz and had a decreased horizontal gain when the frequency was greater than 5.0 Hz. (4) Conclusion: Our study suggested the VAT, especially the horizontal gain, as an indicator, may serve as a sensitive and objective indicator that helps distinguish between MD and VM. Moreover, VAT, due to its non-invasive and all-frequency nature, might be an important part of a test battery.
| S-EPMC9688433 | biostudies-literature
Project description:The diagnosis of "definite" Méniére's disease (MD) relies upon its clinical manifestations. MD has been related with Endolymphatic Hydrops (EH), an enlargement of the endolymphatic spaces (ES) (cochlear duct, posterior labyrinth, or both). Recent advances in Magnetic Resonance (MR) imaging justify its increasing role in the diagnostic workup: EH can be consistently recognized in living human subjects by means of 3-dimensional Fluid-Attenuated Inversion-Recovery sequences (3D-FLAIR) acquired 4 h post-injection of intra-venous (i.v.) Gadolinium-based contrast medium, or 24 h after an intratympanic (i.t.) injection. Different criteria to assess EH include: the comparison of the area of the vestibular ES with the whole vestibule on an axial section; the saccule-to-utricle ratio ("SURI"); and the bulging of the vestibular organs toward the inferior 1/3 of the vestibule, in contact with the stapedial platina ("VESCO"). An absolute link between MD and EH has been questioned, since not all patients with hydrops manifest MD symptoms. In this literature review, we report the technical refinements of the imaging methods proposed with either i.t. or i.v. delivery routes, and we browse the outcomes of MR imaging of the ES in both MD and non-MD patients. Finally, we summarize the following imaging findings observed by different researchers: blood-labyrinthine-barrier (BLB) breakdown, the extent and grading of EH, its correlation with clinical symptoms, otoneurological tests, and stage and progression of the disease.
| S-EPMC8419327 | biostudies-literature
Project description:(1) Background: Vestibular migraine (VM) and Meniere's disease (MD) share multiple features in terms of clinical presentations and auditory-vestibular dysfunctions, e.g., vertigo, hearing loss, and headache. Therefore, differentiation between VM and MD is of great significance. (2) Methods: We retrospectively analyzed the medical records of 110 patients with VM and 110 patients with MD. We at first established a regression equation by using logistic regression analysis. Furthermore, sensitivity, specificity, accuracy, positive predicted value (PV), and negative PV of screened parameters were assessed and intuitively displayed by receiver operating characteristic curve (ROC curve). Then, two visualization tools, i.e., nomograph and applet, were established for convenience of clinicians. Furthermore, other patients with VM or MD were recruited to validate the power of the equation by ROC curve and the Gruppo Italiano per la Valutazione degli Interventi in Terapia Intensiva (GiViTI) calibration belt. (3) Results: The clinical manifestations and auditory-vestibular functions could help differentiate VM from MD, including attack frequency (X5), phonophobia (X13), electrocochleogram (ECochG) (X18), head-shaking test (HST) (X23), ocular vestibular evoked myogenic potential (o-VEMP) (X27), and horizontal gain of vestibular autorotation test (VAT) (X30). On the basis of statistically significant parameters screened by Chi-square test and multivariable double logistic regression analysis, we established a regression equation: P = 1/[1 + e-(-2.269× X5 - 2.395× X13 + 2.141× X18 + 3.949 × X23 + 2.798× X27 - 4.275× X30(1) - 5.811× X30(2) + 0.873)] (P, predictive value; e, natural logarithm). Nomographs and applets were used to visualize our result. After validation, the prediction model showed good discriminative power and calibrating power. (4) Conclusions: Our study suggested that a diagnostic algorithm based on available clinical features and an auditory-vestibular function regression equation is clinically effective and feasible as a differentiating tool and could improve the differential diagnosis between VM and MD.
| S-EPMC9410183 | biostudies-literature
Project description:Despite the huge progress in the definition and classification of vestibular disorders within the last decade, there are still patients whose recurrent vestibular symptoms cannot be attributed to any of the recognized episodic vestibular syndromes, such as Menière's disease (MD), vestibular migraine (VM), benign paroxysmal positional vertigo (BPPV), vestibular paroxysmia, orthostatic vertigo or transient ischemic attack (TIA). The aim of the present international, multi-center, cross-sectional study was to systematically characterize the clinical picture of recurrent vestibular symptoms not otherwise specified (RVS-NOS) and to compare it to MD and VM. Thirty-five patients with RVS-NOS, 150 patients with VM or probable VM and 119 patients with MD were included in the study. The symptoms of RVS-NOS had been present for 5.4 years on average before inclusion, similar to VM and MD in this study, suggesting that RVS-NOS is not a transitory state before converting into another diagnosis. Overall, the profile of RVS-NOS vestibular symptoms was more similar to VM than MD. In particular, the spectrum of vestibular symptom types was larger in VM and RVS-NOS than in MD, both at group comparison and the individual level. However, in contrast to VM, no female preponderance was observed for RVS-NOS. Positional, head-motion and orthostatic vertigo were reported more frequently by patients with RVS-NOS than MD, while external vertigo was more prevalent in the MD group. At group level, the spectrum of attack durations from minutes to 3 days was evenly distributed for VM, while a small peak for short and long attacks in RVS-NOS and a big single peak of hours in MD were discernible. In general, vertigo attacks and associated vegetative symptoms (nausea and vomiting) were milder in RVS-NOS than in the other two disorders. Some patients with RVS-NOS described accompanying auditory symptoms (tinnitus: 2.9%, aural fullness and hearing loss: 5.7% each), migrainous symptoms (photophobia, phonophobia or visual aura in 5.7% each) or non-migrainous headaches (14%), but did not fulfill the diagnostic criteria for MD or VM. Absence of a life time diagnosis of migraine headache and attack duration of <5 min were further reasons not to qualify for VM. In some RVS-NOS patients with accompanying ear symptoms, attack durations of <20 min excluded them from being diagnosed with MD. These findings suggest that RVS-NOS is a stable diagnosis over time whose overall clinical presentation is more similar to VM than to MD. It is more likely to be composed of several disorders including a spectrum of mild or incomplete variants of known vestibular disorders, such as VM and MD, rather than a single disease entity with distinct pathognomonic features.
| S-EPMC8248237 | biostudies-literature
Project description:Objective: Intravenous contrast agent enhanced, high-resolution magnetic resonance imaging of the inner ear (iMRI) confirmed that patients with Menière's disease (MD) and vestibular migraine (VM) could present with endolymphatic hydrops (EH). The present study aimed to investigate EH characteristics and their interrelation to neurotologic testing in patients with VM, MD, or VM with concurrent MD (VM-MD). Methods: Sixty-two patients (45 females, aged 23-81 years) with definite or probable VM (n = 25, 19 definite), MD (n = 29, 17 definite), or showing characteristics of both diseases (n = 8) were included in this study. Diagnostic workup included neurotologic assessments including video-oculography (VOG) during caloric stimulation and head-impulse test (HIT), ocular and cervical vestibular evoked myogenic potentials (o/cVEMP), pure tone audiometry (PTA), as well as iMRI. EH's degree was assessed visually and via volumetric quantification using a probabilistic atlas-based segmentation of the bony labyrinth and volumetric local thresholding (VOLT). Results: Although a relevant number of VM patients reported varying auditory symptoms (13 of 25, 52.0%), EH in VM was only observed twice. In contrast, EH in VM-MD was prevalent (2/8, 25%) and in MD frequent [23/29, 79.3%; χ2(2) = 29.1, p < 0.001, φ = 0.7]. Location and laterality of EH and neurophysiological testing classifications were highly associated (Fisher exact test, p < 0.005). In MD, visual semi-quantitative grading and volumetric quantification correlated highly to each other (r S = 0.8, p < 0.005, two-sided) and to side differences in VOG during caloric irrigation (vestibular EH ipsilateral: r S = 0.6, p < 0.05, two-sided). In VM, correlations were less pronounced. VM-MD assumed an intermediate position between VM and MD. Conclusion: Cochlear and vestibular hydrops can occur in MD and VM patients with auditory symptoms; this suggests inner ear damage irrespective of the diagnosis of MD or VM. The EH grades often correlated with auditory symptoms such as hearing impairment and tinnitus. Further research is required to uncover whether migraine is one causative factor of EH or whether EH in VM patients with auditory symptoms suggests an additional pathology due to MD.
| S-EPMC7991602 | biostudies-literature
Project description:The diagnosis of Meniere's disease (MD) and vestibular migraine (VM) is primarily based on clinical criteria and their differentiation is often difficult. Currently, there are no known definitive diagnostic tests that can reliably distinguish the two conditions. Patients with MD and patients with VM are treated differently, therefore improving the diagnosis of these two pathologies should avoid errors in management. A systematic review was conducted according to PRISMA guidelines. Medline-Ovid and Embase databases were used to conduct a thorough search of English-language publications dating from 1948 to March 2016. The primary search objective was to identify all papers explicitly comparing MD and VM in order to clarify and validate the diagnosis of these two diseases. A total of 13 articles out of 831 were reviewed. Among other differences, MD showed later age of onset, more hearing loss, tinnitus, aural fullness, abnormal nystagmus, abnormal caloric testing results, abnormal vestibular evoked myogenic potential and endolymphatic hydrops. VM showed more headaches, photophobia, vomiting and aura. Even though differences were noted between the two diseases, only one study focused on assessing the differences between VM, MD and patients fulfilling both diagnostic criteria (MDVM). This study showed no difference between the three groups. Since the introduction of the new International Headache Society and Barany Society criteria for VM, no studies have focused on comparing these three groups. We strongly encourage authors to focus on comparing MD and VM from MDVM in future studies to help adequately distinguish the diagnosis of both diseases.
| S-EPMC5544477 | biostudies-literature
Project description:The distribution of cochlin and its associated basement membrane proteins (collagen IV, collagen II, laminin-β2, and nidogen-1) were evaluated in the vestibular endorgans of subjects with Meniere's disease and compared with normal specimens. Cochlin mRNA expression in vestibular endorgans from Meniere's disease specimens was also investigated. Specimens were obtained from patients who had Meniere's disease and who were undergoing ablative labyrinthectomy. Control specimens were obtained both from autopsy specimens with documented normal audiovestibular function and from patients undergoing labyrinthectomy for acoustic neuroma excision. In the normal control specimens, cochlin immunoreactivity was found evenly distributed in the stroma of the cristae ampullaris and maculae of the utricle. In Meniere's specimens, cochlin immunoreactivity was markedly increased; this was associated with an increase in cochlin mRNA expression as shown by real-time reverse transcription with the polymerase chain reaction. Collagen IV and laminin-β2 immunoreactivity was significantly decreased in Meniere's specimens. Nidogen-1 and collagen II immunoreactivity was unchanged in Meniere's specimens when compared with normal samples. Cochlin upregulation has been implicated in the hereditary audiovestibulopathy, DFNA9. The increased expression of cochlin and decreased expression of collagen IV and laminin in Meniere's disease are suggestive that the overexpression of cochlin contributes to the dysfunctional inner ear homeostasis seen in this disease.
| S-EPMC4420027 | biostudies-literature