Endolymphatic Duct Obstruction from a Jugular Bulb Diverticulum

Herniation of the jugular bulb into the middle ear is uncommon. Extension of a jugular bulb diverticulum into the petrous pyramid is rare. In ten previously reported cases in the literature, only the radiographic appearance of the lesion was stressed. Of interest to otolaryngologists is the fact that in seven of the cases there was an ipsilateral sensorineural hearing loss. Other symptoms included tinnitus and vertigo. One patient had been diagnosed as having Menière's disease. We document an additional case (a patient who had “classical Menière's disease”) and report the first known surgical exploration of this venous anomaly. The diverticulum extended high into the petrous bone and was found to obliterate the distal segment of the endolymphatic duct. To our knowledge, this is the first time that a cause and effect relationship has been recognized between endolymphatic hydrops and a jugular bulb diverticulum. We propose that the diverticulum, by obstructing the endolymphatic duct, was directly responsible for the Menière's-like symptoms.

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Computer-Aided and Graphic Reconstruction of the Human Endolymphatic Duct and Sac

Annals of Otology Rhinology & Laryngology ◽

10.1177/00034894800896s202 ◽

1980 ◽

Vol 89 (6_suppl2) ◽

pp. 23-32 ◽

Cited By ~ 21

Author(s):

Juan-Carlos M. Antunez ◽

Fred H. Linthicum ◽

Frank R. Galey ◽

Gilbert D. McCann

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Computer Aided ◽

Endolymphatic Duct ◽

Ménière's Disease ◽

Histopathological Evidence

The anatomy of the human endolymphatic duct system of three cases — one with Menière's syndrome (with no histopathological evidence of endolymphatic hydrops), one with Menière's disease (with histopathological evidence of endolymphatic hydrops), and one with no evidence of disease — has been studied by means of computer-aided and graphic reconstruction from serial sections of temporal bones. The reconstructions have revealed a canalicular arrangement of the epithelial lining of the rugose portion of the human endolymphatic sac. Accordingly, new nomenclature, “pars canalicularis,” is suggested for this portion. The computer produced lateral and medial views of the endolymphatic duct and sac of the patient with Menière's syndrome. The computer also calculated the luminal volume, the surface area of the epithelium, and the surface-to-volume ratio of the rugose portion of the sac. In addition, the authors graphically reconstructed the rugose portion of the sac of all three cases. Measurements of the dimensions of these reconstructions were made, from which the luminal volume was estimated. The reconstructed images and the calculations allowed quantitative comparison of the microscopic anatomy of the rugose portion of the sac in the Menière's syndrome and Menière's disease ears with that in an ear without evidence of disease. Significant differences were found in the volumes of the rugose portion of the sac of the three cases. The results suggest that the luminal volume of the rugose portion of the sac of Menière's disease patients may be significantly reduced. The authors speculate that endolymphatic hydrops may be the result of a loss of epithelial surface area in the canalicular portion, thus diminishing its resorptive function. The computer and graphic reconstruction techniques in conjunction with their analytical capabilities provide a new method for studying inner ear structures. These techniques have the potential to expand the capabilities of quantitative morphology and provide the means for deriving clinically useful data from histopathological and ultrastructural material.

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A high jugular bulb and poor development of perivestibular aqueductal air cells are not the cause of endolymphatic hydrops in patients with Ménière’s disease

Auris Nasus Larynx ◽

10.1016/j.anl.2017.09.014 ◽

2018 ◽

Vol 45 (4) ◽

pp. 693-701 ◽

Cited By ~ 2

Author(s):

Ryohei Oya ◽

Takao Imai ◽

Takashi Sato ◽

Atsuhiko Uno ◽

Yoshiyuki Watanabe ◽

...

Keyword(s):

Endolymphatic Hydrops ◽

Jugular Bulb ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

High Jugular Bulb ◽

Ménière's Disease ◽

Air Cells

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Ménière's Disease

Journal of the Royal Society of Medicine ◽

10.1177/014107688107400304 ◽

1981 ◽

Vol 74 (3) ◽

pp. 183-189 ◽

Cited By ~ 17

Author(s):

Andrew W Morrison

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Cellular Level ◽

Menière’S Disease ◽

Prognostic Indicators ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Endolymphatic Duct ◽

Ménière's Disease

The otological practice at The London Hospital provides very large numbers of patients with Ménière's disease from a busy department. This clinical material has been utilized as much as possible, without research assistants or funds, to examine some clinical and pathological aspects of the disease which might have a bearing on aetiology. The investigation procedures which are likely to lead to an earlier and more certain diagnosis are outlined and prognostic indicators are mentioned. It would appear that there may be an hereditary predisposition to progressive idiopathic endolymphatic hydrops. Potential sufferers tend to have migraine, a family history of migraine, minor degrees of basilar impression associated with an abnormally located endolymphatic duct and sac, a more emotional personality, and possibly an association with blood group A. The experimental and clinicopathological evidence supports the view that the endolymphatic duct and sac have an important role in the pathogenesis of both idiopathic and secondary hydrops. Ultrastructural examination is continuing and may shed more light on the pathology at a cellular level. There is, as yet, no cure for Ménière's disease and sufferers will be incapacitated in varying degrees, often severely, for the rest of their days.

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Modulation of hearing function following the downgrading or upgrading of endolymphatic hydrops in Meniere’s disease patients with endolymphatic duct blockage

PLoS ONE ◽

10.1371/journal.pone.0240315 ◽

2020 ◽

Vol 15 (10) ◽

pp. e0240315

Author(s):

Anquan Peng ◽

Junjiao Hu ◽

Qin Wang ◽

Wenqi Jiang ◽

Wei Liu ◽

...

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Hearing Function ◽

Endolymphatic Duct ◽

Ménière's Disease

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Dynamics in Endolymphatic Hydrops & Symptoms in Meniere's Disease After Endolymphatic Duct Blockage, Preliminary Results

Frontiers in Neurology ◽

10.3389/fneur.2020.622760 ◽

2021 ◽

Vol 11 ◽

Author(s):

Jun He ◽

Anquan Peng ◽

Junjiao Hu ◽

Zhiwen Zhang ◽

Yichao Chen ◽

...

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Menière's Disease ◽

Complete Control ◽

Significant Difference ◽

Endolymphatic Duct ◽

Ménière's Disease

Objective: The purpose of the present study was to evaluate the dynamics of endolymphatic hydrops (EH) and symptoms in a group of patients who underwent endolymphatic duct blockage (EDB) for treatment of intractable Meniere's Disease (MD), and to explore a metric for verifying the effectiveness of EDB procedure.Methods: A total of 22 patients with intractable MD patients who underwent EDB participated in the present study. EH was visualized using locally enhanced inner ear magnetic resonance imaging (MRI) prior to and following surgery. The vestibular hydrops ratio (VHR) in the second MRI examination was compared with the pre-surgery recordings.Results: Following EDB, 6 patients exhibited complete or partial reversal of EH, complete control of vertigo spells and reported improvement in hearing; 13 patients showed no changes in EH or hearing, but 5 of these patients exhibited complete control of vertigo attacks, and the other 8 patients exhibited improved control of vertigo attacks. The final 3 patients showed an increase in EH, but symptomatic worsening in 2 patients, and symptomatic improvement in 1 patient. There was a significant difference in the average VHR prior to and following EDB. Postoperative VHR was positively correlated with the frequency of vertigo spells in the latest 6 months of follow-up and improvement of postoperative average hearing threshold.Conclusion: The decreased EH accompanying the reduction in vertigo attacks and hearing preservation may provide a metric for verifying the effectiveness of EDB treatment in patients with MD.

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A Synchrotron and Micro-CT Study of the Human Endolymphatic Duct System: Is Meniere's Disease Caused by an Acute Endolymph Backflow?

Frontiers in Surgery ◽

10.3389/fsurg.2021.662530 ◽

2021 ◽

Vol 8 ◽

Author(s):

Hao Li ◽

Gunesh P. Rajan ◽

Jeremy Shaw ◽

Seyed Alireza Rohani ◽

Hanif M. Ladak ◽

...

Keyword(s):

Endolymphatic Hydrops ◽

Meniere’S Disease ◽

Menière’S Disease ◽

Meniere's Disease ◽

Ménière’S Disease ◽

Micro Ct ◽

Duct System ◽

Menière's Disease ◽

Endolymphatic Duct ◽

Ménière's Disease

Background: The etiology of Meniere's disease (MD) and endolymphatic hydrops believed to underlie its symptoms remain unknown. One reason may be the exceptional complexity of the human inner ear, its vulnerability, and surrounding hard bone. The vestibular organ contains an endolymphatic duct system (EDS) bridging the different fluid reservoirs. It may be essential for monitoring hydraulic equilibrium, and a dysregulation may result in distension of the fluid spaces or endolymphatic hydrops.Material and Methods: We studied the EDS using high-resolution synchrotron phase contrast non-invasive imaging (SR-PCI), and micro-computed tomography (micro-CT). Ten fresh human temporal bones underwent SR-PCI. One bone underwent micro-CT after fixation and staining with Lugol's iodine solution (I2KI) to increase tissue resolution. Data were processed using volume-rendering software to create 3D reconstructions allowing orthogonal sectioning, cropping, and tissue segmentation.Results: Combined imaging techniques with segmentation and tissue modeling demonstrated the 3D anatomy of the human saccule, utricle, endolymphatic duct, and sac together with connecting pathways. The utricular duct (UD) and utriculo-endolymphatic valve (UEV or Bast's valve) were demonstrated three-dimensionally for the first time. The reunion duct was displayed with micro-CT. It may serve as a safety valve to maintain cochlear endolymph homeostasis under certain conditions.Discussion: The thin reunion duct seems to play a minor role in the exchange of endolymph between the cochlea and vestibule under normal conditions. The saccule wall appears highly flexible, which may explain occult hydrops occasionally preceding symptoms in MD on magnetic resonance imaging (MRI). The design of the UEV and connecting ducts suggests that there is a reciprocal exchange of fluid among the utricle, semicircular canals, and the EDS. Based on the anatomic framework and previous experimental data, we speculate that precipitous vestibular symptoms in MD arise from a sudden increase in endolymph pressure caused by an uncontrolled endolymphatic sac secretion. A rapid rise in UD pressure, mediated along the fairly wide UEV, may underlie the acute vertigo attack, refuting the rupture/K+-intoxication theory.

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