Sequential Imaging in Patient With Suspected Menière's Disease Identifies Endolymphatic Sac Tumor

Kirsh, Elliana; Kozin, Elliott D.; Knoll, Renata M.; Wong, Kevin; Faquin, William C.; Reinshagen, Katherine L.; Kieff, David A.; Rauch, Steven D.; Jung, David

doi:10.1097/mao.0000000000001952

Cited by 8 publications

(10 citation statements)

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“…* Considering the anticipated low chance of intraoperatively identifying the operculum (25) .(†) equal to the overall risk of developing MD with degenerative ES pathology in the general population [i.e., 0.15% based on the MD prevalence of 0.2% (26) and a prevalence of degenerative ES pathology of 76.4% among MD patients in the present study] .(‡) upon initial presentation with clinically unilateral symptoms .(§) due to degenerative changes of extraosseous ES in these patients (6), we consider the rationale for ES surgery questionable. (||) according to Kirsh et al (27). In MD-hp patients, ES hypoplasia sufficiently explains the etiology .…”

Section: Discussionmentioning

confidence: 99%

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Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

et al. 2019

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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.

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Section: Discussionmentioning

confidence: 99%

“…(§) due to degenerative changes of extraosseous ES in these patients (6), we consider the rationale for ES surgery questionable. (||) according to Kirsh et al (27). In MD-hp patients, ES hypoplasia sufficiently explains the etiology .…”

Section: Discussionmentioning

confidence: 99%

Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

et al. 2019

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“…3.4.2.1 Clinical manifestation and imaging Endolymphatic sac tumors (ELSTs) are low-grade malignant papillary neoplasms (low-grade adenocarcinomas) that originate from the epithelium of the endolymphatic duct or sac in the area of the bony vestibular aqueduct (▶ Fig. 9) [271][272][273]. They are characterized by a locally destructive and infiltrating growth pattern, whereas metastases are very rare (only three cases with spinal or cerebellar metastases reported so far) [274,275].…”

Section: Endolymphatic Sac Tumormentioning

confidence: 99%

“…Currently, less than 200 cases of ELSTs have been described in the medical literature [276]. The clinical manifestation with fluctuating, progressive or chronic unilateral (audio-)vestibular hypofunction resembles that of Menièreʼs disease [273,275,277]. Accordingly, a -most likely secondary -endolymphatic hydrops has been visualized on inner ear MRI of ELST patients [278].…”

Section: Endolymphatic Sac Tumormentioning

confidence: 99%

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

Dlugaiczyk

2021

Laryngorhinootologie

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ZusammenfassungDie Differenzialdiagnose von Erkrankungen des vestibulären Labyrinths stellt eine grosse Herausforderung dar, da sich hinter dem sehr häufigen Leitsymptom «Schwindel» viele verschiedene und v. a. seltene Erkrankungen verbergen können. Der vorliegende Beitrag gibt einen Überblick über die für den HNO-Arzt wichtigen seltenen Erkrankungen des vestibulären Labyrinths ausgehend von ihrer klinischen Präsentation als akutes (AVS), episodisches (EVS) oder chronisches vestibuläres Syndrom (CVS). Der Schwerpunkt liegt dabei auf den EVS, sortiert nach ihrer Dauer und dem Vorhandensein von Triggern (Sekunden, ohne Trigger: Vestibularisparoxysmie, Tumarkin-Krise; Sekunden, lärm- und druckinduziert: Syndrome des «dritten Fensters»; Sekunden bis Minuten, positionsabhängig: seltene Varianten und Differenzialdiagnosen des benignen paroxysmalen Lagerungsschwindels; Stunden bis Tage, spontan: intralabyrinthäre Schwannome, Tumoren des endolymphatischen Sacks, Autoimmunerkrankungen des Innenohres). Des Weiteren werden seltene Differenzialdiagnosen eines AVS (Neuritis vestibularis inf., Otolithenfunktionsstörungen, vaskuläre Ursachen, akute bilaterale Vestibulopathie) und eines CVS (bilaterale Vestibulopathie) erläutert. Dabei werden insbesondere die entscheidenden diagnostischen Massnahmen für die Identifikation der einzelnen Krankheitsbilder und die Warnzeichen für potentiell gefährliche Ursachen (z. B. Labyrinthinfarkt/-blutung) dargelegt. Somit dient dieser Beitrag dem HNO-Arzt in Klinik und Praxis als eine Art «Vademecum» für die zügige Identifikation und zeitnahe Therapie seltener Erkrankungen des Gleichgewichtsorgans.

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“…Today, the relationship between ELH and MD symptoms (for review cp [3]), as well as the specificity of ELH for MD, has come under scrutiny. The underlying reason is that different ELH patterns can be found not only in MD [4,5], but also so far in 3.3-28% of healthy ears [6,7], various inner ear [8][9][10] and central [11][12][13][14] pathologies, as well as in anatomic or vascular abnormalities affecting endolymph resorption [15][16][17].…”

Section: Introductionmentioning

confidence: 99%

VOLT: a novel open-source pipeline for automatic segmentation of endolymphatic space in inner ear MRI

et al. 2020

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Background Objective and volumetric quantification is a necessary step in the assessment and comparison of endolymphatic hydrops (ELH) results. Here, we introduce a novel tool for automatic volumetric segmentation of the endolymphatic space (ELS) for ELH detection in delayed intravenous gadolinium-enhanced magnetic resonance imaging of inner ear (iMRI) data. Methods The core component is a novel algorithm based on Volumetric Local Thresholding (VOLT). The study included three different data sets: a real-world data set (D1) to develop the novel ELH detection algorithm and two validating data sets, one artificial (D2) and one entirely unseen prospective real-world data set (D3). D1 included 210 inner ears of 105 patients (50 male; mean age 50.4 ± 17.1 years), and D3 included 20 inner ears of 10 patients (5 male; mean age 46.8 ± 14.4 years) with episodic vertigo attacks of different etiology. D1 and D3 did not differ significantly concerning age, gender, the grade of ELH, or data quality. As an artificial data set, D2 provided a known ground truth and consisted of an 8-bit cuboid volume using the same voxel-size and grid as real-world data with different sized cylindrical and cuboid-shaped cutouts (signal) whose grayscale values matched the real-world data set D1 (mean 68.7 ± 7.8; range 48.9-92.8). The evaluation included segmentation accuracy using the Sørensen-Dice overlap coefficient and segmentation precision by comparing the volume of the ELS. Results VOLT resulted in a high level of performance and accuracy in comparison with the respective gold standard. In the case of the artificial data set, VOLT outperformed the gold standard in higher noise levels. Data processing steps are fully automated and run without further user input in less than 60 s. ELS volume measured by automatic segmentation correlated significantly with the clinical grading of the ELS (p < 0.01). Conclusion VOLT enables an open-source reproducible, reliable, and automatic volumetric quantification of the inner ears' fluid space using MR volumetric assessment of endolymphatic hydrops. This tool constitutes an important step towards comparable and systematic big data analyses of the ELS in patients with the frequent syndrome of episodic vertigo attacks. A generic version of our three-dimensional thresholding algorithm has been made available to the scientific community via GitHub as an ImageJ-plugin.

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Sequential Imaging in Patient With Suspected Menière's Disease Identifies Endolymphatic Sac Tumor

Abstract: A patient with a longstanding diagnosis of MD demonstrated interval development of an ELST. While ELSTs are rare, the study raises the question regarding whether interval imaging is indicated in patients with MD.

Cited by 8 publications

References 10 publications

Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

Endotype-Phenotype Patterns in Meniere's Disease Based on Gadolinium-Enhanced MRI of the Vestibular Aqueduct

Seltene Erkrankungen des vestibulären Labyrinths: von Zebras, Chamäleons und Wölfen im Schafspelz

VOLT: a novel open-source pipeline for automatic segmentation of endolymphatic space in inner ear MRI

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