Unilateral Endolymphatic Hydrops and Associated Abnormalities

1976 ◽  
Vol 85 (3) ◽  
pp. 368-376 ◽  
Author(s):  
Isamu Sando ◽  
Lauren D. Holinger ◽  
Thomas J. Balkany ◽  
Raymond P. Wood
Keyword(s):  
Connective Tissue ◽  
Inner Ear ◽  
Endolymphatic Hydrops ◽  
Endolymphatic Sac ◽  
Brown Pigment ◽  
Epithelial Lining ◽  
Posterior Canal ◽  
Vestibular Aqueduct ◽  
Pigment Deposition ◽  
Associated Abnormalities

A case of unilateral endolymphatic hydrops is presented in which several significant histopathological findings are observed in the affected inner ear and are absent in the opposite ear. Moderate endolymphatic hydrops is present in all cochlear turns on the involved side. Atrophic changes in the cochlear and vestibular end-organs, partial collapse and infolding of Reissner's and the saccular membranes, and collapse of the posterior canal membrane are also seen. There is also marked narrowing of the proximal rugose portion of the endolymphatic sac, associated with a flattened epithelial lining, dense fibrotic connective tissue, brown pigment deposition, and poor vascularity in a relatively narrowed and straightened vestibular aqueduct. Fibrosis, loss of vascularity, and brown pigment deposition are also observed in the bony channels surrounding die vestibular aqueduct.

Hypoplasia of the Vestibular Aqueduct and Endolymphatic SAC in Endolymphatic Hydrops

1978 ◽  
Vol 86 (2) ◽  
pp. ORL-327-ORL-339 ◽  
Author(s):  
Tetsuya Egami ◽  
Isamu Sando ◽  
F. Owen Black
Keyword(s):  
Quantitative Study ◽  
Endolymphatic Hydrops ◽  
Distal Portion ◽  
Endolymphatic Sac ◽  
Anterior Dislocation ◽  
Posterior Canal ◽  
Vestibular Aqueduct ◽  
Temporal Bones ◽  
Air Cells ◽  
Medial View

Four temporal bones from three previously reported cases showing endolymphatic hydrops and pathology of the vestibular aqueduct (VA) and endolymphatic sac (ES) were investigated further. Pathology of the VA and ES was studied by measuring the sizes of the VA and ES, paying particular attention to the proximal rugose portions. A medial-view graphic reconstruction was created to delineate the course and size of the VA, as well as its correlation with neighboring structures (posterior canal and cochlea). In addition, 20 control temporal bones were selected and measured for quantitative study of the sizes of the VA and ES. The four pathologic temporal bones were shown to have small, simple, tube-like VA and ES, without surrounding bony pathology. This appeared to be congenital hypoplasia characterized by hypoplastic funnel-shaped dilatation of the VA and hypoplastic rugose portion of the ES. Anterior dislocation of the distal portion of the VA and poorly developed periaqueductal air cells were also noted in the pathologic bones. Susceptibility to endolymphatic hydrops, difficulty in radiologic visualization of the VA, and surgical exposure of the ES are discussed in relation to hypoplasia of the VA and ES.

Pathophysiologic Findings in the Human Endolymphatic Sac in Endolymphatic Hydrops: Functional and Molecular Evidence

2019 ◽  
Vol 128 (6_suppl) ◽  
pp. 76S-83S ◽  
Author(s):  
Sung Huhn Kim ◽  
Gi-Sung Nam ◽  
Jae Young Choi
Keyword(s):  
Inner Ear ◽  
Animal Studies ◽  
Endolymphatic Hydrops ◽  
Single Layer ◽  
Sensory Epithelium ◽  
Endolymphatic Sac ◽  
Molecular Evidence ◽  
Limited Opportunity ◽  
Basic Studies

Background: The endolymphatic sac (ES) is a cystic structure situated on the posterior fossa dura and is connected to the luminal space of the vestibular organ through the endolymphatic duct, which branches into the utricular and saccular ducts. Unlike the cochlea and vestibule, the ES does not contain sensory epithelium in its luminal space, and a single layer of epithelial cells line the luminal surface area. The ES in the inner ear is thought to play a role in the regulation of inner ear homeostasis, fluid volume, and immune reaction. If these functions of the ES are disrupted, dysfunction of the inner ear may develop. The most well-known pathology arising from dysfunction of the ES is endolymphatic hydrops, characterized by an enlarged endolymphatic space due to the accumulation of excessive endolymphatic fluid. Although, molecular identities and functional evidence for the roles were identified in animal studies, basic studies of the human ES are relatively uncommon compared with those using animal tissues, because of limited opportunity to harvest the human ES. Methods: In this study, molecular and functional evidence for the role of the human ES in the development of endolymphatic hydrops are reviewed. Results and Conclusions: Although evidence is insufficient, studies using the human ES have mostly produced findings similar to those of animal studies. This review may provide a basis for planning further studies to investigate the pathophysiology of disorders with the finding of endolymphatic hydrops.

Inner ear Hemorrhage and Endolymphatic Hydrops in a Leukemic Patient with Sudden Hearing Loss

1977 ◽  
Vol 86 (4) ◽  
pp. 518-524 ◽  
Author(s):  
Isamu Sando ◽  
Tetsuya Egami
Keyword(s):  
Hearing Loss ◽  
Endolymphatic Hydrops ◽  
Stria Vascularis ◽  
Organ Of Corti ◽  
Sudden Hearing Loss ◽  
Lymphocytic Leukemia ◽  
Endolymphatic Sac ◽  
New Bone Formation ◽  
Vestibular Aqueduct ◽  
Endolymphatic Space

Several significant histopathological findings were noted in a case of sudden hearing loss in a patient with chronic lymphocytic leukemia. The major pathological findings were leukemic hemorrhage into both perilymphatic and endolymphatic spaces in the cochlear and vestibular systems, endolymphatic hydrops in the cochlea and sacculus, and a relatively narrowed and straightened vestibular aqueduct and endolymphatic sac. Additional interesting findings include: loss of hair cells in the organ of Corti and vestibular end-organs; destruction of the stria vascularis (possibly the origin of the blood); fibrosis in the perilymphatic spaces in the cochlea and the vestibule, and in the endolymphatic space in the vestibule; and new bone formation in the perilymphatic spaces in the vestibule. The leukemic infiltrate observed in both the cochlea and the vestibule was not considered to be significant. Hemorrhage into the cochlea is thought to be the most reasonable cause of the sudden hearing loss in this case. Also discussed are fibrosis and osteogenesis as a late consequence of hemorrhage, and the coexistence of endolymphatic hydrops with an anomaly of the vestibular aqueduct and endolymphatic sac.

Endolymphatic Hydrops with Absence of Vein in Paravestibular Canaliculus

1980 ◽  
Vol 89 (2) ◽  
pp. 157-161 ◽  
Author(s):  
Ruth Gussen
Keyword(s):  
Bone Formation ◽  
Venous Return ◽  
Endolymphatic Hydrops ◽  
Venous Drainage ◽  
Developmental Anomaly ◽  
Endolymphatic Sac ◽  
Functional Relationships ◽  
Chronic Rupture ◽  
Mild Degree ◽  
Vestibular Aqueduct

Unilateral endolymphatic hydrops is described associated with absence of the vein in the paravestibular canaliculus (PVC) and with decreased vascularity of the vestibular aqueduct and endolymphatic sac. The venous return from the vestibule was normal as far as the junction of the branches forming the PVC vein. At this junction, a blind venous loop was formed with no continuation of venous drainage through the PVC. This probably represents a developmental anomaly. The decreased vascularity of the endolymphatic sac may be related to the absence of the PVC vein. However, anatomical and functional relationships of these vessels are not clear and need further study. Perisac fibrosis and endosteal bone formation are possibly secondary to the decrease in vascularity. A large chronic rupture of the inferior saccule wall probably accounts for the absence of vertigo and the relatively mild degree of cochlear endolymphatic hydrops.

Idiopathic Endolymphatic Hydrops and the Vestibular Aqueduct

1981 ◽  
Vol 90 (1) ◽  
pp. 77-79 ◽  
Author(s):  
Syed S. Rizvi ◽  
Lee Eliott Smith
Keyword(s):  
Marked Reduction ◽  
Endolymphatic Hydrops ◽  
Endolymphatic Sac ◽  
Vestibular Aqueduct ◽  
Consistent Feature

The vestibular aqueduct (VA) and endolymphatic sac were examined topographically in 29 human ears with idiopathic endolymphatic hydrops. When compared with 34 control ears, there was found to be a slight but statistically significant reduction in the width ( P < 0.005) and length ( P < 0.05) of the vestibular aqueduct. A small hypoplastic endolymphatic sac was found in three of the ears with hydrops but was also present in one control ear. It would appear that a marked reduction in sac size is not a consistent feature in ears with endolymphatic hydrops.

Large vestibular aqueduct syndrome and endolymphatic hydrops: two presentations of a common primary inner-ear dysfunction?

2009 ◽  
Vol 123 (8) ◽  
pp. 919-921 ◽  
Author(s):  
J H Spiegel ◽  
A K Lalwani
Keyword(s):  
Inner Ear ◽  
World Literature ◽  
Endolymphatic Hydrops ◽  
Fluid Homeostasis ◽  
Vestibular Aqueduct ◽  
Primary Dysfunction ◽  
The World ◽  
Large Vestibular Aqueduct Syndrome ◽  
Inner Ear Dysfunction ◽  
Large Vestibular Aqueduct

AbstractObjective:To present the theory that large vestibular aqueduct syndrome (i.e. the recognised existence of an enlarged vestibular aqueduct with progressive sensorineural hearing loss) and endolymphatic hydrops are due to a common primary dysfunction of inner-ear fluid homeostasis.Method:Case report and review of the world literature concerning large vestibular aqueduct syndrome and endolymphatic hydrops.Results:We report a family in which one sibling suffered from large vestibular aqueduct syndrome while the other had classic Ménière's disease. This suggests that large vestibular aqueduct syndrome and endolymphatic hydrops, in some cases, may be due to a common primary dysfunction of inner-ear fluid homeostasis.Conclusion:To our knowledge, this is the first report in the world literature to postulate that variation in the relative compliance of inner-ear membranes could be the factor that determines the manifestation of the disorder as either endolymphatic hydrops or large vestibular aqueduct syndrome.

Expression of Intercellular Adhesion Molecule-1 during Inner Ear Inflammation

1995 ◽  
Vol 104 (1) ◽  
pp. 69-75 ◽  
Author(s):  
Masashi Suzuki ◽  
Jeffrey P. Harris
Keyword(s):  
Immune Response ◽  
Inner Ear ◽  
Adhesion Molecule ◽  
Endolymphatic Hydrops ◽  
Stria Vascularis ◽  
Intercellular Adhesion ◽  
Endolymphatic Sac ◽  
Intercellular Adhesion Molecule ◽  
Spiral Ligament ◽  
Intercellular Adhesion Molecule 1

This study was designed to investigate the expression of intercellular adhesion molecule-1 (ICAM-1) on the spiral modiolar vein (SMV) with its collecting venules (CVs) and the venules of the endolymphatic sac during inner ear inflammation. These data will further elucidate the role of adhesion molecules in extravasation of inflammatory cells from blood vessels during an inner ear immune response. Labyrinthitis was induced in rats by inoculation of keyhole limpet hemocyanin into the scala tympani of animals who had been systemically sensitized to it. Expression of ICAM-1 was examined with a mouse monoclonal antibody to rat ICAM-1 by immunohistochemistry. ICAM-1 was found weakly on the epithelium of SMVs and CVs as early as 6 hours postchallenge, reaching a maximum by day 2 and then fading away gradually. The maximum influx of immunocompetent cells into the cochlea was seen between days 3 and 7. Staining for ICAM-1 was observed on the epithelium of the endolymphatic sac and perisaccular region at 12 and 24 hours, respectively, and this was associated with infiltration of cells into these areas 3 days postchallenge. By day 28, the inner ear had developed endolymphatic hydrops, but at this time it showed almost no significant staining with anti-ICAM-1. The molecule was also expressed in the mesothelium of perilymph, the perineurium of cochlear nerves, the spiral ligament, and the basal cells of the stria vascularis following immunization. Our data provide evidence that endothelial cells of the SMV and its CVs, as well as other inner ear sites, have the potential to express ICAM-1. This expression precedes the influx of immune cells; therefore, it is possible that this ligand plays a pivotal role in the onset of inflammation in the inner ear. This study also confirmed that the immune response results in endolymphatic hydrops as a long-term consequence.

The Role of the Endolymphatic Sac in Inner Ear Immunity

1987 ◽  
Vol 103 (5) ◽  
pp. 182-188 ◽  
Author(s):  
Shunichi Tomiyama ◽  
Jeffrey Harris
Keyword(s):  
Inner Ear ◽  
Endolymphatic Sac
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