Detection and Grading of Endolymphatic Hydrops in Menière Disease Using MR Imaging

Baráth, Krisztina; Schuknecht, Bernhard; Naldi, Arianne Monge; Schrepfer, Thomas; Bockisch, Christopher J.; Hegemann, Stefan

doi:10.3174/ajnr.a3856

Cited by 179 publications

(197 citation statements)

References 30 publications

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“…The ability to visualize hydrops with this technique has been confirmed by multiple groups on different continents. [6][7][8]13 To date, however, no strong clinical application has been established for this technique, and existing research is mostly limited to showing a connection between a clinical diagnosis of MD and imaging evidence of hydrops.…”

Section: Discussionmentioning

confidence: 99%

“…4,5 Recent advances in MR imaging technology have allowed in vivo imaging of endolymphatic hydrops, which has the potential to advance our understanding of the natural history of the disease and assess changes in response to treatment. Several groups have confirmed the ability of delayed intravenous contrast-enhanced MR imaging to detect hydrops, [6][7][8] but no prior studies have assessed serial changes in hydrops during diuretic treatment by using delayed intravenous contrast-enhanced MR imaging, to our knowledge. One study that followed patients treated with betahistine by using intratympanic contrast-enhanced MR imaging did not show hydrops reversal despite symptomatic improvement.…”

mentioning

confidence: 94%

“…The images were evaluated with respect to the presence of endolymphatic hydrops. The qualitative criteria described by Baráth et al 7 were used to assess the 2D images for the presence of hydrops in the vestibule. 3D MIPs were also reviewed, and the criteria described by Sepahdari et al 8 were used to assess these images for hydrops in the vestibule.…”

Section: Imagingmentioning

confidence: 99%

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Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI

Sepahdari¹,

Vorasubin²,

Ishiyama

et al. 2015

AJNR Am J Neuroradiol

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SUMMARY: Endolymphatic hydrops, the primary pathologic alteration in Menière disease, can be visualized by using delayed intravenous contrast-enhanced 3D-FLAIR MR imaging. It is not known whether MR imaging-demonstrable changes of hydrops fluctuate with disease activity or are fixed. We describe the results of baseline and posttreatment MR imaging studies in a group of subjects with Menière disease with hydrops who were treated with acetazolamide. Seven subjects with untreated Menière disease with MR imaging evidence of hydrops had repeat MR imaging during acetazolamide treatment. Symptoms and imaging findings were assessed at each time point. Five subjects showed symptom improvement, of whom 3 had improvement or resolution of hydrops. One subject had recurrent symptoms with recurrent hydrops after discontinuing therapy. Two had unchanged hydrops despite symptom improvement. Subjects with unchanged symptoms had unchanged hydrops. Hydrops reversal may be seen with acetazolamide treatment in Menière disease. MR imaging may provide an additional biomarker of disease.ABBREVIATIONS: hT2WI-FLAIR ϭ heavily T2-weighted 3D FLAIR; MD ϭ Menière disease; SPACE ϭ sampling perfection with application-optimized contrasts by

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 94%

Section: Imagingmentioning

confidence: 99%

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Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI

Sepahdari¹,

Vorasubin²,

Ishiyama

et al. 2015

AJNR Am J Neuroradiol

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“…Thus, experimental and clinical MRI investigations of the inner ear have established that, when administered intravenously or intratympanically, the Gd complex readily penetrates the perilymphatic space, but not the endolymphatic space of the entire vestibulo-cochlea membranous labyrinth [Naganawa et al, 2012, Counter et al, 1999, Counter et al, 2000, Duan et al, 2004. Indeed, MRI in combination with Gd contrast agents has been used to visualize EH in patients with Ménière's disease [Naganawa et al, 2014, Zou et al, 2009, Baráth et al, 2014, Jerin et al, 2014 and in animal models developed to simulate human inner ear disease , Niyazov et al, 2001.…”

Section: Introductionmentioning

confidence: 99%

Vasopressin induces endolymphatic hydrops in mouse inner ear, as evaluated with repeated 9.4 T MRI

et al. 2015

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“…Gadolinium, an agent used in magnetic resonance imaging (MRI), is taken up into the perilymph, presumably via perfusion through the BLB, specifically through the blood-perilymphatic barrier. Increased gadolinium enhancement in the perilymph on the ipsilaterally inner ear affected with Meniere's disease has been noted in multiple studies, indicative of breakdown of the BLB [36][37][38][39] . Of note, in the study of Tagaya et al 37 , a high signal intensity ratio correlated with a higher grade (none, mild, or significant) of hydrops in both the cochlea and the vestibule 37 .…”

Section: Discussionmentioning

confidence: 99%

Recent Research Trend for Ménièreʼs Disease

Nam¹

2017

Res Vestib Sci

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The ultrastructural organization of the blood labyrinthine barrier (BLB) was investigated in the human vestibular endorgan, the utricular macula, using postmortem specimens from individuals with documented normal auditory and vestibular function and surgical specimens from patients with intractable Meniere's disease. Transmission electron microscopic analysis of capillaries located in the normal human utricular stroma showed vascular endothelial cells with few pinocytotic vesicles, covered by a smooth and uniform basement membrane surrounded by pericyte processes. Meniere's disease specimens revealed differential ultrastructural pathological changes in the cellular elements of the microvasculature. With moderate degeneration of the BLB, there were numerous vesicles within the vascular endothelial cells (VECs), with increased numbers at the abluminal face, pericyte process detachment and disruption of the perivascular basement membrane surrounding the VECs. With severe degeneration of the BLB, there was severe vacuolization or frank apparent necrosis of VECs and loss of subcellular organelles. A higher severity of BLB degenerative changes was associated with a higher degree of basement membrane thickening and edematous changes within the vestibular stroma. This study presents the first ultrastructural analysis of the capillaries constituting the BLB in the human vestibular macula utricle from normal and Meniere's disease.In the inner ear, the term blood labyrinthine barrier (BLB) refers to the barrier between the vasculature and the inner ear fluids, either endolymph or perilymph 1, 2 . The BLB is critical for the maintenance of the inner ear fluid ionic homeostasis and for the prevention of the entry of deleterious substances into the inner ear 3 . By studying the penetration of dyes and drugs from the systemic circulation into the fluids of the inner ear, it can be shown that the BLB is selective and that the composition of the inner ear fluids is regulated and markedly different from that of blood or of other fluids such as cerebrospinal fluid 2 . Understanding the dynamics of the BLB is important to develop therapeutic drug delivery systems to the inner ear to block or enhance the BLB inflammatory response. The BLB in the cochlea is well studied in animal models 2,4,5 . Initial transmission electron microscopy (TEM) studies demonstrated that the BLB in the guinea pig stria vascularis and spiral ligament is composed of vascular endothelial cells (VECs) surrounded by a basement membrane 6 . More recent studies have noted that, in addition, the intrastrial BLB is composed of pericytes and perivascular-resident macrophage-like melanocytes [7][8][9][10][11] . The VECs line the interior surface of the blood vessels, connected by tight junctions, forming an interface between circulating blood and the rest of the vessel wall. Pericytes are the only cell type to intimately connect with VECs as they lie embedded within the endothelial basement membrane. The perivascular-resident macrophage-like melanocytes have foot pr...

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Detection and Grading of Endolymphatic Hydrops in Menière Disease Using MR Imaging

Abstract: BACKGROUND AND PURPOSE:Endolymphatic hydrops has been recognized as the underlying pathophysiology of Menière disease. We used 3T MR imaging to detect and grade endolymphatic hydrops in patients with Menière disease and to correlate MR imaging findings with the clinical severity.

Cited by 179 publications

References 30 publications

Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI

Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI

Vasopressin induces endolymphatic hydrops in mouse inner ear, as evaluated with repeated 9.4 T MRI

Recent Research Trend for Ménièreʼs Disease

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