Vestibular tributaries to the vein of the vestibular aqueduct.

CONCLUSION: The vein of the vestibular aqueduct drains blood from areas extensively lined by vestibular dark cells (VDCs). A possible involvement in the pathogenesis of an impaired endolymphatic homeostasis can be envisioned at the level of the dark cells area. OBJECTIVES: The aim of this study was to investigate the vascular relationship between the vein of the vestibular aqueduct and the vestibular apparatus, with focus on the VDCs. METHODS: Sixteen male Wistar rats were divided into groups of 6 and 10. In the first group, 2 µm thick sections including the vein of the vestibular aqueduct, utricle, and crista ampullaris of the lateral ampulla were examined by light microscopy and computer-generated three-dimensional imaging. In the second group, ultrathin sections including venules and VDCs were examined by transmission electron microscopy. RESULTS: A microvascular network was observed in close relation to the VDCs in the utricle and the crista ampullaris of the lateral semicircular canal in the vestibular apparatus. One major vein emanated from these networks, which emptied into the vein of the vestibular aqueduct. Veins draining the saccule and the common crus of the superior and posterior semicircular canals were likewise observed to merge with the vein of the vestibular aqueduct.

The intravestibular source of the vestibular aqueduct. III: Osseous pathology of Ménière's disease, clarified by a developmental study of the intraskeletal channels of the otic capsule.

CONCLUSION: Review of the histopathological changes in the vestibular arch in Ménière's disease, after a study of development of the otic capsule, indicated a severe apoptotic loss of osteoblasts with consequent denudation of these cells from and damage to the osseous canal structure of the arch. OBJECTIVE: To review previously reported histological findings in the inner layer of the vestibular aqueduct and its intravestibular source in Ménière's disease, using newer knowledge of otic capsule development. METHODS: Temporal bone histological sections from the vestibular arch region of eight patients with Ménière's disease were reviewed in our London-based material. RESULTS: Minute granules suggesting apoptotic bodies were found in the arch in the majority of cases, giving support for the concept of an apoptotic loss of osteoblasts. Explanation for the previously described appearance of proliferation of atypical channels and of small, finely outlined empty areas in the bone was provided by the observation of denudation of osteoblasts from Volkmann's canals and microcanals. These canals had been recently described in a developmental study of the otic capsule. Dislocation of dead microcanals into blood vessels of Volkmann's canals was seen in two of the cases.

The intravestibular source of the vestibular aqueduct. II: its structure and function clarified by a developmental study of the intra-skeletal channels of the otic capsule.

CONCLUSION: A developmental histologic study of the otic capsule indicates that it grows a system of lamellar bone with abundant interconnecting intraosseous channels. These include the 'cartilage canals' in the cartilage model, the chondro-osseous and Haversian-like (Volkmann's) canals in the ossified otic capsule, the fissula ante fenestram, which seems to function as a lifelong manufacturer of the latter two channels, and the inner layer (vestibular arch) of the vestibular aqueduct, which is a complex series of Volkmann's canals and microcanals. Chemical changes, possibly produced by breakdown of cells within the channels, may provide a homeostatic environment for the functions of hearing and balance that take place in the endolymphatic fluid. OBJECTIVES: We studied the development of the otic capsule to clarify the cellular appearances that we had previously described in the normal vestibular arch and the changes in that structure in Ménière's disease. METHODS: Step sections from 84 temporal bones, including those from fetuses, children and adults from a variety of ages were examined histologically. RESULTS: Cartilage canals, bringing blood vessels and mesenchymal cells from perichondrium to the depths of the cartilage model to mediate ossification, are found early in fetal life and disappear when ossification is complete at about 24 weeks. The otic capsule is formed of chondro-osseous canals, which are composed of trabeculae of mineralized cartilage lacunae containing mesenchymal cells that undergo ossification (globuli ossei); also Volkmann's canals (like Haversian canals in long bones but multidirectional), which are produced from osteoblasts. The lumina of the latter frequently link up with chondro-osseous canals. Lamellar bone forms the background of the otic capsule. The fissula ante fenestram is present from early in the cartilage model and then throughout life. It appears to mediate bone production and the new formation of chondro-osseous channels and Volkmann's canals. The internal layer of the vestibular aqueduct (vestibular arch) is seen in the cartilage model of the otic capsule (present in early fetal life) as a vascular layer of perichondrally derived connective tissue (not cartilage) surrounding the endolymphatic duct. When endochondral ossification starts, the bone from the adjoining cochlear and vestibular sides embrace this connective tissue layer to form the outer bony layer of the vestibular aqueduct. Osteoblasts then fill the inner layer with lamellar bone and macro- and mini-Volkmann's canals. At 1 year osteoblasts in the walls of macro-Volkmann's canals, proliferating thereafter throughout life, produce large numbers of microcanals. It is possible that slow breakdown of these osteoblasts and of similar cells in the canals of the otic capsule proper may contribute to the homeostasis of the endolymphatic duct and that of the rest of the membranous labyrinth, respectively.

The intravestibular source of the vestibular aqueduct: Its structure and pathology in Ménière's disease.

CONCLUSION: We describe a thin, highly vascular layer of mineralized cartilage, which surrounds most of the endolymphatic duct. In the normal ear this may act in helping to control the chemical composition of endolymph. In Ménière's disease (MD) there is a marked apoptotic change among the mineralized cartilage cells of this layer, which seems to be associated also with the deposition of a pathological substance in the walls of many blood vessels. This may lead to serious chemical change in the nearby endolymph and so provoke the symptoms of MD. OBJECTIVES: Endolymphatic hydrops is found in all cases of MD, but is not specific for that condition. We sought a cellular change in the vicinity of the saccule that might be more specific than the lesion of endolymphatic hydrops and thus lead to a more successful management of the disease. MATERIALS AND METHODS: We examined stained step sections of 33 autopsy temporal bones from 20 cases of MD, particularly in the region of the vestibule, and compared the changes with those found in a similar region of 65 temporal bones taken from randomly selected cases of non-Ménière conditions. RESULTS: In all temporal bones there was a well-demarcated region of the posterior vestibule, which formed a skeletal arch around the opening of the tunnel of the vestibular aqueduct into which the endolymphatic duct entered from the vestibule. This 'vestibular arch' was composed mainly of blood vessels and mineralized chondrocytes. The inner skeletal layer surrounding the course of most of the endolymphatic duct in the tunnel of the vestibular aqueduct was composed of the same tissue and was in fact continuous with the vestibular arch. In the non-Ménière temporal bones the mineralized chondrocytes were congregated around normal thin-walled blood vessels and small numbers of them seemed to be undergoing apoptosis in this vicinity. In all of the MD temporal bones, except five in which the vestibular arch was either absent or atrophic, we found pronounced changes of apoptosis among the mineralized cartilage cells and these were associated with proliferative changes in blood vessels in which a bluish-staining translucent deposit, possibly mineralization of the vascular wall, was prominent.

The vein of the vestibular aqueduct with potential pathologic perspectives.

HYPOTHESIS: Pathologic changes around the vein of the vestibular aqueduct (VVA) may cause obstruction to the flow of blood toward the sigmoid sinus. Furthermore, a distal obstruction of this vessel may be responsible for a development of a retrograde flow of blood with concomitant drainage of endolymphatic sac (ES) substances to the inner ear. BACKGROUND: The VVA is responsible for the venous drainage of the vestibular apparatus and endolymphatic duct and ES. Previous studies have linked the VVA to Ménière's disease. The aim of the present article was a 3-dimensional perspective study of the VVA with its adjacent anatomic structures. METHODS: In 14 rats, the VVA was examined by 3-dimensional reconstruction of 2-microm serial sections, corrosion cast technique, and scanning electron microscopy. RESULTS: From the external aperture of the vestibular aqueduct, the VVA is interposed between the ES and the operculum. Three to 4 collecting venules from the ES drain into the VVA. The VVA merges at an oblique angle with the sigmoid sinus. CONCLUSION: The VVA courses near the ES, operculum, and sigmoid sinus and is potentially vulnerable to expanding structures in the cranial posterior fossa. The possible role of the VVA for the function of the ES under normal and pathologic conditions is discussed.

A potential portal flow in the inner ear.

OBJECTIVES/HYPOTHESIS: The aim of the present study was to visualize the flow direction of blood in the extraosseous part of the vein of the vestibular aqueduct (VVA) and to explore the effect of an induced obstruction in the distal part of the VVA before it merges with the sigmoid sinus. The endolymphatic sac has been implicated as a potential endocrine gland, which venules drain to the VVA. A reversal of the direction of flow in the VVA toward the inner ear could, through vestibular arteriovenous anastomosis, cause portal circulation in the inner ear. STUDY DESIGN: The authors conducted an experimental animal study using in vivo fluorescence microscopy. RESULTS: Obstructing the distal part of the VVA just before it empties into the sigmoid sinus immediately reverses the flow of blood in the VVA toward the inner ear. CONCLUSIONS: After an obstruction of the VVA, the drained venous blood from the endolymphatic sac may enter a portal circulation in the inner ear, which could cause disturbances in the endolymph homeostasis and potentially symptoms as seen in Meniere disease.

Ménière disease caused by an anomalous vein of the vestibular aqueduct.

The precise cause of Ménière disease remains unclear. Multiple causes have been proposed with most experimental evidence pointing to impaired fluid resorption by the endolymphatic duct and sac as the final common pathway in development of hydrops. We report a unique case of Ménière disease secondary to compression of the endolymphatic duct and sac by an anomalous vein of the vestibular aqueduct. The resulting mechanical obstruction led to excessive accumulation of endolymph with clinical signs of Ménière disease. We review the literature and discuss proposed pathogenesis of disease. The finding of this anomalous vein provides further evidence that anatomical obstruction of the endolymphatic duct and sac may lead to Ménière-like symptoms. This unique example of an anatomical variant offers additional insight into the pathophysiology of endolymphatic hydrops.

Degeneration of vestibular sensory cells caused by ablation of the vestibular aqueduct in the gerbil ear.

The vestibular aqueduct of the gerbil has a unique anatomic feature that makes it possible to selectively obliterate the endolymphatic sac with or without interfering with its venous drainage. In animals in which only the endolymphatic sac was ablated, endolymphatic hydrops was slight in the cochlea and was absent in the vestibular labyrinth. The cochlear and vestibular sensory cells were normal. In animals in which both the endolymphatic duct and the vein were obliterated, hydrops was slight, with the exception of a few cochleas that showed moderate hydrops. The sensory cells of the posterior canal cristae had degenerated in all specimens, while varied pathologic changes in cochlear and vestibular sensory cells were present in some specimens. These results suggest that hydrops is primarily due to blockage of the endolymphatic duct and sac and that degeneration of sensory cells occurs when blood flow in the vestibular aqueduct is impeded. Pathologic changes in the endolymphatic sac, including the vascular plexus at the endolymphatic sac, may play an important role in the production of endolymphatic hydrops and vestibular symptoms in Meniere's disease.

Venous communications of the cochlea after acute occlusion of the vein of the cochlear aqueduct.

The vein of the cochlear aqueduct (VCAQ) is the principal drainage vein of the cochlea in the guinea pig. Morphological observations of the VCAQ and its adjacent structures were made by studying serial sections of the cochlea. We detected the presence of two collateral vessels from the mucoperiosteal veins of the middle ear which communicated with the VCAQ. Following acute occlusion of the VCAQ, marked dilatations of these vessels were observed in corrosion cast preparations. Our findings suggest that these vessels act as collateral veins following acute venous congestion of the inner ear.

Vascular mechanisms in Meniere's disease.

Normal venous drainage of the vestibular organs through the vein of the paravestibular canaliculus (PVC) may be crucial to inner ear fluid mechanics. It is proposed that increased venous pressure, with resultant venous insufficiency of the vestibular organs, may result in endolymphatic hydrops unless collateral venous circulation develops. Certain variations in pattern of venous drainage where the vestibular organs drain predominantly through the PVC vein may be a predisposing factor. In patients with Meniere's disease, different mechanisms can cause venous insufficiency. One suggested mechanism is morphologic change in the microcirculation of the intermediate portion of the endolymphatic sac. Microcirculation changes may be associated with fibrosis of the perisac tissues or shortening of the intermediate sac region or might be physiologically determined. Venous insufficiency may also result from anomalies of the PVC vein.

The fenestrated blood vessels of the endolymphatic sac. A freeze-fracture and transmission electron microscopic study.

The fenestrated blood vessels surrounding the endolymphatic sac in guinea pigs were investigated with the help of freeze-fracturing. The technique exposes the structure and distribution of vascular pores as well as interendothelial bridges or tight junctions between vascular endothelial cells. It is possible to get a three-dimensional comprehension of the vascular structure which can be compared with that of conventional transmission electron microscopy (TEM). Discontinuity in the junctional elements as seen in some endothelial layers and the high number of fenestrations organized in geometric patterns, as well as the abundant, randomly distributed micropinocytotic vesicles seem to bear out the theory that the endolymphatic sac is one of the most metabolically active parts of the inner ear and may be involved in the turnover of endolymph.

Course and contents of the paravestibular canaliculus.

The purpose of this study is to describe the anatomy of the paravestibular canaliculus (PVC) in detail, especially its course in relation to the vestibular aqueduct (VA) and its vascular contents. Serial horizontal sections of 20 normal human temporal bones were stained either with hematoxylin and eosin, by Verhoeff-van Gieson's method, or by Mallorys method, and studied under the light microscope. Graphic reconstruction of the VA and the PVC was performed in some of the cases. In this study, the following new anatomical information on the common course and vascular contents of the PVC was obtained: 1) two PVC are present in the area near the vestibular orifice of the VA; 2) the PVC merges with the VA in the area near its cranial orifice without any particular branching, and does not enter the posterior cranial fosa; 3) veins course through the entire length of the PVC whereas arteries course only through the posterior cranial fossa side of the PVC. It appears that veins are the major contents of the PVC and that these veins represent the main venous drainage system from the vestibule. It also appears that arteries in the PVC supply the blood from the posterior cranial fossa mainly to the PVC itself.

Endolymphatic hydrops with absence of vein in paravestibular canaliculus.

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.

Histological evidence of specialized microcirculation of the endolymphatic sac.

A specialized type of blood vessel is demonstrated within the dense soft tissue areas of the vestibular aqueduct and vascular channels of the surrounding bone, including the paravestibular canaliculus (PVC). The vessel wall is formed by the collagen-smooth muscle bundles of these areas. The lumen of these vessels is irregular, and segments appear to be closed by apposition of the bundles. The vessels are continuous with the capillaries of the endolymphatic sac. It is not clear whether they are on the venous side of the capillary system, or on the arterial side and might possibly be part of an arteriovenous anastomoses system. Their possible role in some cases of Meniere's disease must be considered.

Vein of the vestibular aqueduct.

The vein of the vestibular aqueduct (VVA) was investigated in a series of 40 human temporal bones. The processing included vascular injection with a colored medium, decalcification and cutting in serial, thick sections, which were put in a clear fluid and studied with a stereo-microscope. The labyrinthine roots of the VVA are the single veins of the ampulla and simple limbs of the semicircular canals and of the posterior wall of the utricle. They drain the rich capillary bed of the simple endolymphatic walls of the canals and the utricle, as well as a small peripheral area of the cristae and the utricular macula. The VVA leaves the vestibule through an individual bone canal running parallel to the vestibular aqueduct up to the dura of the posterior side of the petrosa in the area of the endolymphatic sac. It then opens in the inferior petrosal sinus or the jugular bulb. The vein receives other branches from the bone, dura and sac. Correct information on the course of this vein appears to be lacking in contemporary textbooks and articles, although it has been correctly described since the last century.

[The venous drainage of the vestibular labyrinth in man (author's transl)]. / Le drainage veineux du labyrinthe vestibulaire chez l'homme.

The venous drainage of the vestibular division of the inner ear presents two different and separated routes. One, draining the central part of the sensorial areas (maculae and cristae), ends up into the vein of the cochlear aqueduct. The other drains the perypheral part of the sensorial areas as well as the simple endolymphatic walls and ends into the vein of the vestibular aqueduct. The latter vein shows close relationships to the endolymphatic sac.

Anatomy of the para-vestibular canaliculus.

A histologic study of the para-vestibular canaliculus (PVC), its contents, and its relationship to the vestibular aqueduct (VA), is presented. 20 normal human temporal bones were fixed in 10% formalin solution, embedded in celloidin, and sectioned horizontally at intervals of 20 micrometers. Every tenth section was stained with hematoxylin and eosin (HE) and studied under a light microscope. Three significant observations were made. First, in 80% of the specimens, two rather than one PVC were found in the area of the vestibular orifice of the VA. Second, in 70% of the specimens, the PVC was found to merge with the VA rather than to enter the posterior cranial fossa (PCF) separately. Third, in all the specimens examined, a vein was seen to traverse the entire length of the PVC. However, in 17 specimens, no artery could be identified within the PVC. In the 13 (65%) specimens in which arteries could be identified in the PVC, the arteries extended only half the length of the PVC, from the PCF to the VA. In no specimen examined could arteries be seen extending the full length of the PVC from the PCF to the vestibule.