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.

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.

Effect of Transverse Temporal Bone Fracture on the Fluid Compartment of the Inner Ear

1979 ◽  
Vol 88 (6) ◽  
pp. 741-748 ◽  
Author(s):  
Syed S. Rizvi ◽  
Kevin P. Gibbin
Keyword(s):  
Hair Cells ◽  
Endolymphatic Hydrops ◽  
Membranous Labyrinth ◽  
Temporal Bone Fracture ◽  
Complete Obstruction ◽  
Vestibular Hair Cells ◽  
Vestibular Aqueduct ◽  
Fluid Compartment ◽  
Temporal Bones ◽  
Transverse Fractures

Five temporal bones exhibiting transverse fractures were studied with a view to determining whether such fractures could lead to symptomatic endolymphatic hydrops. Four out of the five temporal bones showed the fracture line traversing the vestibular aqueduct. Two of these four showed complete obstruction of the endolymphatic duct. One of these had an intact membranous labyrinth and severe endolymphatic hydrops. The other had ruptures of the membranous labyrinth and no hydrops. Three ears exhibited partial preservation of auditory and vestibular hair cells and neurons. These findings are consistent with the concept that a transverse fracture may produce endolymphatic hydrops by obstructing the vestibular aqueduct while preserving enough audiovestibular epithelium and neurons to present as symptomatic Menière's disease.

Cochlear Hydrops in Association with Collapsed Saccule and Ductus Reuniens

1982 ◽  
Vol 91 (1) ◽  
pp. 5-13 ◽  
Author(s):  
Ken KitamurA ◽  
Harold F. Schuknecht ◽  
Robert S. Kimura
Keyword(s):  
Endolymphatic Hydrops ◽  
Endolymphatic Sac ◽  
Longitudinal Flow ◽  
Anatomical Distribution ◽  
Temporal Bones ◽  
Histopathological Evidence

Histopathological evidence from five human temporal bones with endolymphatic hydrops limited to the cochlea shows pathological obliteration of their saccules and/or ductus reunientes. We suggest that this finding supports the theory of longitudinal flow of endolymph. The anatomical distribution of endolymphatic hydrops is related to the location of the blockage which may occur anywhere from the ductus reuniens to the endolymphatic sac. Atrophy of sensorineural structures of the cochlea is severe in these five temporal bones and is presumed to be caused by the original disorders rather than occurring secondary to the 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.

Expression of inducible nitric oxide synthase (iNOS/NOS II) in the hydropic vestibule after injection of keyhole limpet hemocyanin into the endolymphatic sac of guinea pigs

2001 ◽  
Vol 11 (2) ◽  
pp. 67-71
Author(s):  
Ken-ichi Watanabe ◽  
Shunichi Tomiyama ◽  
Ken Jinnouchi ◽  
Alexander Hess ◽  
Olaf Michel ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Nitric Oxide Synthase ◽  
Inducible Nitric Oxide Synthase ◽  
Guinea Pigs ◽  
Endolymphatic Hydrops ◽  
Keyhole Limpet Hemocyanin ◽  
Endolymphatic Sac ◽  
Temporal Bones ◽  
Oxide Synthase ◽  
Inducible Nitric Oxide

This study was undertaken to examine the expression of inducible nitric oxide synthase (iNOS / NOS II) in the hydropic vestibule of guinea pigs. Animals were systemically sensitized with 500 μg of keyhole limpet hemocyanin. Two weeks after the first injection, keyhole limpet hemocyanin (100 μg/5 μl) was injected into the endolymphatic sac following the intradural approach, and the next day temporal bones were removed for the immunohistochemical examination. Endolymphatic hydrops was evidenced by the expansion of the Reissner's membrane in the cochlea after direct injection of keyhole limpet hemocyanin into the endolymphatic sac. Inducible nitric oxide synthase expression was increased in the sensory cells, supporting cells and vestibular ganglion cells, while temporal bones, where only phosphate buffered saline was injected, did not show any inducible nitric oxide synthase immunoreactivity. High levels of inducible nitric oxide synthase-catalyzed nitric oxide were detected prior to the development of the inner ear dysfunction. Our results suggest that the occurrence of inducible nitric oxide synthase immunoreactivity parallels the inner ear disturbance as seen in endolymphatic hydrops.

The utriculo-endolymphatic valve: its functional significance

1975 ◽  
Vol 89 (10) ◽  
pp. 985-996 ◽  
Author(s):  
Harold F. Schuknecht ◽  
A. Aziz Belal
Keyword(s):  
Functional Significance ◽  
Endolymphatic Hydrops ◽  
Normal Structure ◽  
Anatomical Structure ◽  
Endolymphatic Sac ◽  
Developmental Defects ◽  
Cochlear Duct ◽  
Anatomical Features ◽  
Temporal Bones ◽  
Excessive Accumulation

AbstractA STUDY was made of the anatomical structure and functional significance of the ultriculo-endolymphatic (UE) valve in man. The material consists of 170 human temporal bones of which 75 were chosen to show normal structure through nine decades of life, 29 with endolymphatic hydrops, 22 with developmental defects and 44 with pertinent staining characteristics of the endolymphatic fluid. The studies show that the UE valve is ideally suited to preserve the humoral and anatomical features of the pars superior (utricle and canals) from the developmental, disease, and traumatic susceptibilities of the pars inferior (cochlear duct and saccule). Its function probably is to permit the occasional egress of excessive accumulation of endolymph to be processed in the endolymphatic sac and to accomplish this while preserving the normal endolymph volume and membrane anatomy of the utricle and canals.

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