The Vestibular Aqueduct and Endolymphatic Sac and Duct in Endolymphatic Hydrops

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.

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Unilateral Endolymphatic Hydrops and Associated Abnormalities

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348947608500307 ◽

1976 ◽

Vol 85 (3) ◽

pp. 368-376 ◽

Cited By ~ 11

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.

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

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348947708600412 ◽

1977 ◽

Vol 86 (4) ◽

pp. 518-524 ◽

Cited By ~ 33

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.

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Endolymphatic Hydrops with Absence of Vein in Paravestibular Canaliculus

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948008900212 ◽

1980 ◽

Vol 89 (2) ◽

pp. 157-161 ◽

Cited By ~ 7

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.

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The vestibular aqueduct and the endolymphatic sac and duct in endolymphatic hydrops

Clinical Otolaryngology ◽

10.1111/j.1365-2273.1979.tb01778.x ◽

1979 ◽

Vol 4 (6) ◽

pp. 469-474

Keyword(s):

Endolymphatic Hydrops ◽

Endolymphatic Sac ◽

Vestibular Aqueduct

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Idiopathic Endolymphatic Hydrops and the Vestibular Aqueduct

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348948109000120 ◽

1981 ◽

Vol 90 (1) ◽

pp. 77-79 ◽

Cited By ~ 7

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.

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