Hypothetical mechanism for vertigo in Meniere's disease.

Past theories that have been proposed to account for the attacks of vertigo during the course of Meniere disease are reviewed. In the past, vascular theories and theories of perilymph and endolymph mixing due to ruptures or leakages were proposed. Recent research concerning the basic mechanisms of the inner ear anatomy and function cast doubt on these theories. The anatomy, physiology, and pathophysiology of the inner ear, and in particular of the endolymphatic sac and endolymphatic duct are reviewed. Recent studies suggest that in people the endolymph ionic content is replenished without any flow of fluid and that longitudinal endolymph flow only occurs in response to volume excess. Furthermore audiological and electrophysiological studies have revealed little or no change in the cochlear function during episodes of vertigo. The longitudinal drainage theory attempts to encompass the recent research findings. The theory hypothesizes that endolymph draining too rapidly from the cochlear duct (pars inferior) causes attacks of vertigo. The endolymph overfills the endolymphatic sinus and overflows into the utricle (pars superior), stretching the cristae of the semicircular canals, causing the attacks of vertigo.

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.

A Bast-like valve in the pigeon?

The first description of the presence of a utriculo-endolymphatic valve in human fetuses was given by Bast in 1928. Since then this valve-like structure is called Bast's valve. Its exact function has not yet been established. The general opinion is that it has a protective function by having the possibility to separate the superior endolymphatic compartments of the labyrinth from the inferior compartment. Phylogenetically seen birds are the first vertebrates with a cochlear duct and a distinct inferior and superior part of the labyrinth. A structure in the pigeon inner ear, resembling Bast's valve in mammals, is described.

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.

Morphology and function of Bast's valve: additional insight in its functioning using 3D-reconstruction.

The utriculo-endolymphatic valve was discovered by Bast in 1928. The function of Bast's valve is still unclear. By means of orthogonal-plane fluorescence optical sectioning (OPFOS) microscopy 3D-reconstructions of the valve and its surrounding region are depicted. The shape of the duct at the utricular side is that of a flattened funnel. In the direction of the endolymphatic duct and sac this funnel runs into a very narrow duct. The valve itself has a rigid 'arch-like' configuration. The opposing thin, one cell-layer thick, utricular membrane is highly compliant. We propose that opening and closure of the valve occurs through movement of the flexible base/utricular membrane away from and toward the relatively rigid valve lip.

Endolymphatic duct status during middle fossa dissection of the internal auditory canal: a human temporal bone radiographic study.

OBJECTIVE: Successful hearing preservation after acoustic neuroma resection is sometimes complicated by delayed hearing deterioration. The middle fossa approach appears to offer superior long-term hearing results when compared to the retrosigmoid surgical approach. The goal of this study is to investigate the hypothesis that internal auditory canal (IAC) drilling during middle fossa acoustic neuroma removal is associated with a lower incidence of endolymphatic duct (ELD) injury, a potential cause of delayed hearing loss (HL) known to accompany retrosigmoid hearing preservation dissection techniques. STUDY DESIGN: A human temporal bone anatomic and radiographic study complemented with a literature review. METHODS: Twenty human temporal bones were analyzed with high-resolution multislice computed tomography (HRMCT) and subjected to standard extended middle fossa IAC dissection with labyrinthine preservation and follow-up HRMCT for analyses of the ELD. RESULTS: Zero of 20 (0%) temporal bones were found to have violation of the ELD with preservation of the labyrinthine structures and the endolymphatic sac. Reviews of human and animal studies indicate that injury to the ELD may create endolymphatic hydrops, a known cause of hearing deterioration. CONCLUSION: The ELD is not vulnerable to injury during IAC dissection using the middle fossa approach. A previous radiographic study has shown that the ELD is violated in 24% of temporal bones during retrosigmoid dissection of the IAC. These findings support and may help explain other outcome studies that show that long-term hearing results are superior with the use of the middle fossa approach when compared to results following retrosigmoid dissection.

Suboccipital retrosigmoid approach for removal of vestibular schwannomas: facial nerve function and hearing preservation.

In this report, we discuss the pertinent bony, arachnoid, and neurovascular anatomy of vestibular schwannomas that has an impact on the surgical technique for removal of these tumors, with the goal of facial nerve and hearing preservation. The surgical technique is described in detail starting with anesthesia, positioning, and neurophysiological monitoring and continuing with the exposure, technical nuances of tumor removal, hemostasis, and closure. Positive prognostic factors for hearing preservation are also highlighted.

3-T imaging of the cochlear nerve and labyrinth in cochlear-implant candidates: 3D fast recovery fast spin-echo versus 3D constructive interference in the steady state techniques.

BACKGROUND AND PURPOSE: High-resolution imaging of the internal auditory canal and labyrinth at 1.5 T is often performed by using three-dimensional (3D) fast spin-echo or T2* techniques. We evaluated both techniques at 3 T in the preoperative assessment of patients being considered for cochlear implants. METHODS: Sagittal 3D fast recovery fast spin-echo (FRFSE) and 3D constructive interference in the steady state (CISS) images were acquired in eight patients at 3.0 T by using dual surface coils. Contrast-to-noise ratios (CNRs) for the intracanalicular nerve and CSF were measured in the internal auditory canal. Two neuroradiologists reviewed the images to determine whether the techniques provided images of diagnostic quality. RESULTS: CNRs for 3D CISS were twice those obtained with 3D FRFSE. Both techniques provided images of diagnostic quality, though spurious signal intensity loss at the apex of the superior semicircular canals was encountered on 3D FRFSE images in four of eight patients. CONCLUSION: Both 3D FRFSE and 3D CISS provide high-resolution images of the internal auditory canal and labyrinth at 3.0 T. We predict that the superior CNRs obtained with 3D CISS will prove advantageous as we move to smaller fields of view at higher field strength.

Estimation of the endolymphatic sac and vestibular aqueduct using magnetic resonance imaging.

OBJECTIVE: To evaluate the diagnostic accuracy of magnetic resonance imaging for assessment of the endolymphatic sac and vestibular aqueduct. STUDY DESIGN: Imaging and histological study of the cadaver. METHODS: Five cadavers were studied by a 1.5-T magnetic resonance imaging system with a 3-inch-diameter surface coil. Magnetic resonance imaging scans were obtained with proton density-weighted and T2-weighted fast spin-echo sequences. Histological sections were made with an epoxy resin-embedding method and were compared with magnetic resonance imaging scans. RESULTS: The visibility of the endolymphatic sac on both sequences corresponded well to the presence of the endolymphatic sac on histological sections. On the histological sections, the width of the external aperture of vestibular aqueduct (endolymphatic sac including surrounding connective tissue) was 0.96 +/- 0.18 mm (mean +/- SD) and the width of lumen of endolymphatic sac at the same point was 0.47 +/- 0.17 mm. The width of the endolymphatic sac was 1.02 +/- 0.19 mm on proton density-weighted images and was 0.81 +/- 0.15 mm on T2-weighted images. The widths of endolymphatic sac measured on proton density-weighted image and those of vestibular aqueduct on histological section did not show statistically significant differences (P >.05). On the other hand, the endolymphatic sac as measured on T2-weighted image tended to be smaller than the vestibular aqueduct (P <.05) and tended to be larger than the lumen of the endolymphatic sac (P <.0005). CONCLUSION: Both sequences can precisely depict the endolymphatic sac; however, the proton density-weighted image is a more appropriate indicator of the actual anatomical configuration of the endolymphatic sac with surrounding connective tissue and vestibular aqueduct.

Volumetric and dimensional analysis of the guinea pig inner ear.

The objective of this study was to provide accurate volumetric data on the fluid spaces and soft tissue in the guinea pig inner ear by measuring all histologic serial sections by means of Metamorph Imaging Software at 400x to 1,000x magnification. The total endolymph volume of the inner ear was 4.691 mm3, of which 1.501 mm3 was in the cochlea, 3.090 mm3 in the vestibular labyrinth, and 0.100 mm3 in the endolymphatic duct and sac. The total perilymph volume was 15.938 mm3, of which 8.867 mm3 was in the cochlea and 7.071 mm3 in the vestibular labyrinth. The volume of the organ of Corti per millimeter length increased toward the apex, but the volumes of the stria vascularis, spiral ligament, and spiral limbus decreased. The volume of the macula utriculi was larger than that of the macula sacculi. The measurement of the luminal surface area of the stria vascularis was 3.944 mm2, and that of the vestibular dark cells was 5.772 mm2.

[The vascular pattern of the endolymphatic duct, endolymphatic sac and its anatomic differences in guinea pigs].

In order to study the vascular pattern of the endolymphatic duct and sac, endolymphatic duct and sac were examined with vascular Indian ink injection and image analysis. The Results were as follows: 1. In the 20 temporal bones, 17 (85%) had posterior meningeal artery (PMA) and posterior vestibular artery (PVA) supply and the rest (3 specimens, 15%) had no PVA supply; 2. The distribution frequency of PMA in the endolymphatic sac was much higher than that of PVA(P < 0.01), but the distribution of PMA and PVA in the endolympatic duct were not different (P > 0.05). The conclusions is that there are anatomic differences in vascular supply and pattern of the endolymphatic duct and sac, PMA is the main vascular structure in the endolymphatic sac.

Morphometric evaluation of the infralabyrinthine approach to the internal auditory canal.

Morphometric evaluation of the infralabyrinthine approach to the internal auditory canal (IAC) was performed using 20 fresh human temporal bones in order to assess the exposure limitations, inherent risks and technical difficulties that may arise due to common anatomic variations of this region. While performing the infralabyrinthine approach to the IAC, minor problems such as an anteriorly placed sigmoid sinus were easily managed. However, in 50% of the specimens, this approach was limited due to variations of the jugular bulb, restricting access to the IAC. Sacrificing the endolymphatic duct in these specimens did not significantly improve the surgical access to the eighth nerve. Furthermore, it was noted that this approach puts the facial nerve and cochlea under the risk of inadvertent damage during drilling. The authors conclude that vestibular nerve sectioning using the infralabyrinthine approach may be performed only in few selected cases and extreme care is needed in order not to damage the structures that limit this approach.

High-resolution T2-weighted MR imaging of the inner ear using a long echo-train-length 3D fast spin-echo sequence.

The purpose of this study was to assess the value of a long echo-train-length 3D fast spin-echo (3D-FSE) sequence in visualizing the inner ear structures. Ten normal ears and 50 patient ears were imaged on a 1.5T MR unit using a head coil. Axial high-resolution T2-weighted images of the inner ear and the internal auditory canal (IAC) were obtained in 15 min. In normal ears the reliability of the visualization for the inner ear structures was evaluated on original images and the targeted maximum intensity projection (MIP) images of the labyrinth. In ten normal ears, 3D surface display (3D) images were also created and compared with MIP images. On the original images the cochlear aqueduct, the vessels in the vicinity of the IAC, and more than three branches of the cranial nerves were visualized in the IAC in all the ears. The visibility of the endolympathic duct was 80%. On the MIP images the visibility of the three semicircular canals, anterior and posterior ampulla, and of more than two turns of the cochlea was 100%. The MIP images and 3D images were almost comparable. The visibility of the endolymphatic duct was 80% in normal ears and 0% in the affected ears of the patients with Meniere's disease (p < 0.01). In one patient ear a small intracanalicular tumor was depicted clearly. In conclusion, the long echo train length T2-weighted 3D-FSE sequence enables the detailed visualization of the tiny structures of the inner ear and the IAC within a clinically acceptable scan time. Furthermore, obtaining a high contrast between the soft/bony tissue and the cerebrospinal/endolymph/ perilymph fluid would be of significant value in the diagnosis of the pathologic conditions around the labyrinth and the IAC.

High-resolution magnetic resonance imaging of the endolymphatic duct and sac.

An anatomical study was carried out to determine the extent to which magnetic resonance imaging (MRI) could delineate inner ear structures. Anatomical preparations of human petrous temporal bone were examined and compared with the results of MRI in 20 healthy subjects to see whether the structures of the inner ear could be visualized. Imaging of the subjects was carried out in a 1.0-T MRI scanner (Siemens Magnetom Impact). Two strongly T2*-weighted sequences were used: a 3D-PSIF sequence and a 3D-CISS sequence. The 3D data sets were postprocessed using a Maximum Intensity Projection (MIP) program. Our investigations show that it is possible to obtain accurate visualization of structures with a diameter of under 1 mm. In all 20 subjects it was possible to identify both the endolymphatic duct and the endolymphatic sac.

The utriculo-endolymphatic valve in pediatric temporal bones.

The utriculo-endolymphatic valve (UEV) is located in the posterior wall of the utricle at its junction with the utricular duct and was first described in a human fetus by Bast in 1928. Although different theories about its normal position and function have been postulated, the function of the UEV remains unclear. In the present investigation we studied 118 temporal bones from 70 children to determine whether there were differences in the position of the valve and by inference, its function between children and adults. Premortem ages ranged from newborn to 10 years (mean age, 11.6 months). All temporal bones were fixed in 10% formalin, decalcified and processed by the celloidin technique. Specimens were sectioned in a horizontal plane at a thickness of 20 microns. Every tenth section was stained with hematoxylin-eosin and studied by light microscopy. The position of the UEV was then classified as closed or open. Valves damaged by preparation or having an uncertain position were classified as artifact. The chi-square test was used to determine a correlation between the position of the valve and the status of the rest of the endolymphatic system and whether or not endolymphatic hydrops was present in the cochlear and vestibular systems. The UEV was closed in 39 temporal bones (33.1%) and open in 13 (11.0%). Artifacts were found in 66 bones (55.9%). In the group of patients with a collapsed ductus reuniens the UEV was closed in 38% of the specimens, suggesting that the UEV prevented loss of endolymph from the pars superior, but these findings were not statistically significant.

Immunolocalization of Na+, K(+)-ATPase, Ca(++)-ATPase, calcium-binding proteins, and carbonic anhydrase in the guinea pig inner ear.

The distribution of Na+, K(+)-ATPase, Ca(++)-ATPase, carbonic anhydrase, and calcium-binding proteins were investigated immunohistochemically in paraffin sections of guinea pig inner ears. Marginal cells of the stria vascularis, type II fibrocytes of the spiral ligament, and cells in supralimbal and suprastrial regions, were positive for Na+, K(+)-ATPase. Type I fibrocytes of the spiral ligament were positive for Ca(++)-ATPase, carbonic anhydrase, calmodulin and osteopontin. In the vestibular system, dark cells were positive for Na+, K(+)-ATPase. However, these cells and subepithelial fibrocytes were negative for Ca(++)-ATPase, carbonic anhydrase, and the calcium-binding proteins. In the endolymphatic sac, epithelial cells in intermediate and distal portions were positive for Na+, K(+)-ATPase, but the reaction was less than that in the stria. The same endolymphatic sac cells that were positive for Na+, K(+)-ATPase were also positive for Ca(++)-ATPase and calcium-binding proteins, but negative for carbonic anhydrase. The presence of Ca(++)-ATPase and calcium-binding proteins in the type I fibrocytes of the spiral ligament suggests that these cells are involved in mediating Ca++ regulation. Lower levels of Na+, K(+)-ATPase and the co-existence of Ca(++)-ATPase and calcium-binding proteins in the epithelial cells of the endolymphatic sac indicate that these cells have a distinctive role in ion transport that is different from that of the cells of the stria vascularis and vestibular dark cells.

Periductal vessels of the endolymphatic duct.

Light microscope was used to examine the rich vascular plexus surrounding the human endolymphatic duct, both in the periductal loose connective tissue and in the bony channels surrounding the bony vestibular aqueduct. We also performed computer-aided three-dimensional reconstruction on one serially sectioned region of the endolymphatic duct. We found an anastomotic and looping network of vessels residing in the loose connective tissue close to the epithelium of the endolymphatic duct. This network often received a vascular contribution from the vessels in the periaqueductal bony channels. These findings were verified by light microscopic examination of 50 temporal bone specimens. Concurrent with this finding, histologic examination also showed different characteristic features of the vascular system of the endolymphatic duct-proximal sac areas and of the more distal parts of the endolymphatic sac. These features include the arrangement, quantity, and contents of the periaqueductal bony channels, as well as the organization of the bone containing these periductal bony channels. Findings from this study help the understanding of the anatomy of the human endolymphatic duct. In addition, they support and supplement earlier observations of the structure of the endolymphatic duct. We suggest the possible existence of a periductal vasculature system, similar in pattern to that in the endolymphatic sac, but specialized to work with the duct to aid its function.