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Surface aspects of the developing human organ of Corti.

Thirteen cochleas from human fetuses ranging in age from week 9 to week 22 were observed by scanning electron microscopy. The classical 'base-to-apex' and 'internal-to-external' gradients of maturation were confirmed by surface observations of the developing organ of Corti. The tectorial membrane begins to be secreted around week 9, i.e. 2 weeks before the onset of the ciliogenesis. Its structure appears to be first amorphous and then fibrillar. The surface of the organ of Corti looks mature around week 22, but, in fact, its complete maturation, especially at outer hair cell level, probably occurs during the last trimester of pregnancy.

Cell Differentiation↗

Alcian blue staining of cochlear hair cell stereocilia and other cochlear tissues.

Using whole-mount surface preparations of the chinchilla organ of Corti, the anionic characteristics of cochlear tissues was investigated with the polycationic dye Alcian Blue. Our primary goal was to specifically stain the hair cell stereocilia for the purpose of producing cytocochleograms. To specifically stain hair cell stereocilia we investigated the effects of different fixatives and the 'critical electrolyte concentration' [Scott and Dorling (1965) Histochemie 5, 221-233] of MgCl2 on staining specificity. The stereocilia and the tectorial membrane were the most intensely stained cochlear structures and presumably have a strong negative charge. The most important factor in producing specific staining was fixation in osmium tetroxide rather than the addition of an electrolyte to the Alcian Blue staining solution. The use of Alcian Blue produced intense staining of hair cell stereocilia against an unstained background using standard, brightfield light microscopy.

Alcian Blue↗

IHC-TM connect-disconnect in relation to sensitization and masking of a HF-tone burst by a LF tone. IV.

Evidence continues to accumulate that although the outer hair cells (OHCs) of the cochlea are firmly bonded to the tectorial membrane (TM), the inner hair cells (IHCS) are not. This is the fourth in a series of papers that explores how the idea of a set of disconnected hair cells that "impact" the TM is consistent with psychophysical data. The paper extends the exploration to the masking of high-frequency (HF) tone bursts by low-frequency (LF) tones and shows that the model can explain the important features of these complex data.

Auditory Threshold↗

A quantitative light microscopic study of the bullfrog amphibian papilla tectorium: correlation with the tonotopic organization.

The height, width and cross-sectional area of the bullfrog amphibian papilla tectorial membrane were quantitatively analysed from frontal serial sections. The cross-sectional area (which is a measure of mass) of the tectorium does not appear to be linearly graded along the length of the papilla, but rather spatial gradations occur in more or less a step-wise manner. The spatially graded area (or mass) is well correlated with the width of the tectorium and their relationships with the tonotopic organization of the amphibian papilla are discussed.

Animals↗

IHC-TM connect-disconnect and efferent control V.

Four previous papers in this series have explored how the idea of a set of disconnected inner hair cells (IHCs) that can "impact" the tectorial membrane (TM) is consistent with psychophysical data. This paper extends the model and explores the potential for mechanical interaction between the IHCs and outer hair cells (OHCs). In particular, it is speculated that the advantage of IHC-TM disconnect is extended dynamic range, and that movement of the movement of the OHCs and TM, under efferent control, constitutes a mechanical servo system for adjusting IHC-TM spacing along the cochlear partition to achieve this extended range.

Afferent Pathways↗

Stereociliary dysfunction, a case of sensory hearing loss, recruitment, poor speech discrimination and tinnitus.

The following hypothesis is presented: A dysfunction of the hair cell cilia, either in the form of ciliary pathology or as a temporary loss of ciliary stiffness, both of which are documented, ought to lead to a partial decoupling of the involved hair cells from the tectorial membrane. Consequently, 1) energy transmission should be attenuated (= hearing loss), 2) the noise level at the hair cell input should be increased (= tinnitus) and, owing to the concomitant center-clipping of the signal waveform, 3) the input/output function should become steeper (= recruitment) and 4) the formant structure of speech should be largely destroyed (= relatively poor speech discrimination). The above signs and symptoms are characteristic of a number of acute cochlear disorders and would thus find a common explanation.

Animals↗

Low-voltage field-emission scanning electron microscopy of non-coated guinea-pig hair cell stereocilia.

The stereociliar structures of the guinea-pig cochlear organ of Corti were studied at low-voltage (1-5 kV) with field-emission scanning electron microscope (SEM) using various pre- and post-fixation methods, such as OTOTO (OsO4/thiocrbohydrazide/OsO4/thiocarbohydrazide/OsO4) and TAO (tannic acid/arginine/OsO4), and different dissection procedures of the cochlea. A perfusion and immersion pre-fixation with glutaraldehyde, in combination with removal of the bony wall and stria vascularis from the cochlea, followed by the TAO non-coating treatment gave the best result at 2 kV acceleration voltage. Due to these new technique, several interesting delicate structures of the stereocilia, in particular fine surface structures, were detected for the first time using SEM. These findings include the different types of cross-links and tip links, i.e., the fine surface morphology of the stereocilia and their attachments and imprints in the tectorial membrane (TM). One of the most interesting findings in this study is a network of long filamentous structures, which has been identified mainly at the top of the longest stereocilia and the undersurface of the TM and which may represent the glycocalyx. These findings and their possible implications in the process of mechanoelectrical transduction will be discussed.

Animals↗

Sudden deafness in relapsing polychondritis. A scanning electron microscopy study.

The left ear of a 57-year-old female who suffered sudden deafness during the course of relapsing polychondritis was examined under a scanning electron microscope. Marked degeneration of the organ of Corti in all turns and dislocated and encapsulated tectorial membrane were found in the cochlea. Marked decrease in number of the sensory cells in the utricular and saccular maculae and total loss of sensory hair bundles in the ampullary cristae of the semicircular canals were seen in the vestibule. These findings strongly suggest that the cause of sudden deafness in this case might be viral. The usefulness of scanning electron microscopy in human temporal bone pathology is stressed.

Acoustic Maculae↗

Acute measles infection in the hamster cochlea.

Measles virus has been implicated in sudden sensorineural hearing loss (SNHL) in adults as well as in children. Furthermore, sensorineural hearing loss following live measles virus vaccination has been reported. As of yet, however, there has been only few reports on human temporal bone pathology due to measles, and on experimental animal models of measles infection. This study was undertaken to examine acute measles infection in adult hamster cochlea morphologically and immunohistochemically for precise understanding of this viral infection. Atrophy of the stria vascularis, loss of the organ of Corti, "rolled-up" tectorial membrane, and cell infiltration with a positive immunofluorescent reaction primarily within the scale media indicating endolymphatic labyrinthitis were the principal findings. These results were generally consistent with previous ones on human temporal bone pathology not only due to measles but also to SNHL. We consider measles virus to be one of the possible pathogens, even if low in frequency, causing profound and irreversible hearing loss, including SNHL. Completion of measles vaccination without complication and selective or mass revaccination may be necessary to prevent such hearing loss.

Acute Disease↗

Collagen changes in the cochlea of aged Fischer 344 rats.

Hearing function in the Fischer 344 (F344) albino inbred strain of rats deteriorates with aging faster than in other strains, in spite of the small hair cell loss in old F344 animals [Popelar, J., Groh, D., Pelanova, J., Canlon, B., Syka, J., 2005. Age-related changes in cochlear and brainstem auditory function. Neurobiol. Aging, in press.]. This study was aimed at elucidating the structural changes in the inner ear of this rat strain during aging. Cochlear histopathology was examined in 20-24-month-old F344 rats and compared with that of young F344 rats (4 months) and of old rats of the Long-Evans (LE) strain. Hematoxylin/eosin staining in aged F344 rats showed degenerative changes in the organ of Corti, consisting of a damaged layer of marginal cells, reduced vascularization of the stria vascularis and a distorted tectorial membrane detached from the organ of Corti. Age-related changes in collagen distribution were observed with Masson's trichrome staining in the spiral ligament of old F344 rats. The results of immunohistochemical staining for type II collagen revealed a marked decrease in collagen fibers in the area connecting the spiral ligament and stria vascularis and a decrease in area IV fibrocytes in old F344 but not in LE rats. These findings may contribute to an explanation of the substantial hearing loss found in old F344 rats.

Aging↗

Acute cochlear disorders: the combination of hearing loss, recruitment, poor speech discrimination, and tinnitus.

The combination of hearing loss, recruitment, poor speech discrimination and tinnitus, which is characteristic of acute cochlear disorders, can be accounted for on the basis of a decoupling of hair cells from their drive system, the tectorial membrane. Decoupling may either be caused by a temporary reduction of ciliary stiffness (shown to occur during periods of noise-induced temporary threshold shifts) or by temporary and/or chronic ciliary pathology (demonstrated to exist in cases of antibiotic ototoxicity and in endolymphatic hydrops). Since ciliary coupling is elastic in nature, the decoupling is only partial. The hearing loss and the tinnitus are manifestations of the reduced coupling per se, the magnitude of the loss depending on the degree of decoupling and the number of hair cells involved. Recruitment and poor speech discrimination result from center-clipping of the signal waveform applied to an involved hair cell, the direct corollary of partial, ciliary decoupling.

Acute Disease↗

Development of the organ of Corti in horseshoe bats: scanning and transmission electron microscopy.

The late prenatal and early postnatal development of the organ of Corti were studied in the horseshoe bat (Rhinolophus rouxi) by using scanning and transmission electron microscopy. Arrangements and dimensions of stereocilia bundles, together with their contacts with the tectorial membrane, were found to be adult-like shortly before birth, and thus before the biological onset of hearing (3-5 days after birth). During the first postnatal week, there were baso-apical gradients in disappearing kinocilia on inner hair cells (IHC), microvillis of supporting cells, and marginal pillars. The lower basal cochlear turn was mature with respect to these regressing structures at 3 days after birth, the apical turn at 10 days after birth. At birth, cytodifferentiation was found to be completed, and the tunnel of Corti and innermost spaces of Nuel had opened. The ultrastructure of IHCs was not markedly different from that at later ages. In outer hair cells (OHC), the adult-like regular arrangement of a single layer of subsurface cisternae and pillars was seen as soon as protrusions of supporting cells had withdrawn from the lateral wall of OHCs (basal turn at birth and throughout the cochlea 2 days after birth). Numerous efferent endings contacted the somata of IHCs up to the second postnatal week. Since the medial olivocochlear system is absent in horseshoe bats, the adult-like innervation pattern of OHCs was established at the biological onset of hearing. During the first 2 postnatal weeks, the cytoskeleton of pillar and Deiters cells, and the specialized Deiters cups developed. The organ of Corti appeared adult-like at 14 days, apart from the persistence of a reduced tympanic cover layer attached to the basilar membrane. Morphological data support physiological findings that the first broadly tuned auditory responses arise from the basal turn. The distinct low to high frequency gradient in development of sensitivity during the first 2 postnatal weeks of the horseshoe bat was not, however, matched by morphological gradients, and it would appear that the development of the cytoskeleton of supporting cells contributed to the establishment of tuning in the auditory fovea. Adult-like morphology of the organ of Corti coincided with the emergence of sharply tuned responses from the auditory fovea, but there was no clear-cut correlate for the shift in tuned foveal frequency representation that occurred during the following 3 weeks.

Animals↗

Neck injuries: I. Occipitoatlantal dislocation--a pathologic study of twelve traffic fatalities.

Twelve of 155 persons killed in traffic crashes had occipitoatlantal dislocations. Nine were vehicular occupants, 2 were cyclists, and one was a pedestrian. The dislocations involved various combinations of lacerations of the alar ligaments, the occipitoatlantal joint capsules, the dura mater, the tectorial membrane, the rectus capitis muscles, and the suboccipital muscles. In 2 instances, an occipital condyle failed instead of the corresponding alar ligament, producing condyle fractures. Atlas ring fractures occurred in 3 instances. Axial and subaxial cervical trauma were uncommon. Facial or mandibular fractures occurred in a majority of cases, vault skull fractures were uncommon, and basilar fractures were absent. Pontomedullary brainstem lacerations occurred in 9 of the 12, and 4 had midbrain lacerations. The majority of the victims succumbed to acute neurogenic shock as the sole or the major mechanism of death. The biomechanical basis for occipitoatlantal dislocation is discussed, and the author suggests that distraction, in concert with variable combinations of extension, rotation, and posterior translation is responsible for occipitoatlantal dislocations.

Accidents, Traffic↗

Incorporation of D-[3H]-glucosamine and L-[3H]-fucose into the developing rat cochlea.

The uptake of two tritiated carbohydrates, D-[3H]-glucosamine and L-[3H]-fucose, to the developing rat cochlea was examined using light and electron microscopic radioautography. Both carbohydrates, administered to in vitro developing rat cochleas, shared a similar ultrastructural labeling pattern on the microvilli and apical cell region and on the tectorial membrane (TM) fibrils. On embryonic day 18, the radiolabeling appeared on the apical surface of the undifferentiated epithelium that will develop into both spiral limbus and Kölliker's organ (KO), while on postnatal day (PD) 1, it was only located on the apical surface of the KO. When D-[3H]-glucosamine was administered in vivo to newborn rats, the radiolabeling was observed in the TM covering the KO at PD 3. Lastly, D-[3H]-glucosamine administered in vivo to PD 7 rats, appeared at PD 9 in the TM region lying just above the organ of Corti. The present findings support the previously suggested leading role of the spiral limbus and KO in the secretion of the TM during cochlear development. The secretion of carbohydrates, and probably of other matrix components, starts on the spiral limbus and KO region and progressively extends to the organ of Corti.

Animals↗

Delayed inner ear maturation and neuronal loss in postnatal Igf-1-deficient mice.

Insulin-like growth factor-1 (IGF-1) has been shown to play a key role during embryonic and postnatal development of the CNS, but its effect on a sensory organ has not been studied in vivo. Therefore, we examined cochlear growth, differentiation, and maturation in Igf-1 gene knock-out mice at postnatal days 5 (P5), P8, and P20 by using stereological methods and immunohistochemistry. Mutant mice showed reduction in size of the cochlea and cochlear ganglion. An immature tectorial membrane and a significant decrease in the number and size of auditory neurons were also evident at P20. IGF-1-deficient cochlear neurons showed increased caspase-3-mediated apoptosis, along with aberrant expression of the early neural markers nestin and Islet 1/2. Cochlear ganglion and fibers innervating the sensory cells of the organ of Corti presented decreased levels of neurofilament and myelin P(0) in P20 mouse mutants. In addition, an abnormal synaptophysin expression in the somata of cochlear ganglion neurons and sensory hair cells suggested the persistence of an immature pattern of synapses distribution in the organ of Corti of these animals. These results demonstrate that lack of IGF-1 in mice severely affects postnatal survival, differentiation, and maturation of the cochlear ganglion cells and causes abnormal innervation of the sensory cells in the organ of Corti.

Aging↗

Functional structure of the organ of Corti: a review.

The mammalian auditory organs have a dual sensory system (inner vs. outer hair cells) with distinctly different cellular organizations and innervation patterns. However, the inner (IHCs) and outer (OHCs) hair cells are mechanoreceptors sharing similar general characteristics such as organization of stereocilia (including linkage system) and a gradation of stereociliary height along the length of the cochlea. This gradation of stereociliary height may be the single most important anatomic feature in the tuning capability of the sensory cell. Several lines of evidence suggest that the stereociliary stiffness may be modulated by the sensory cells themselves, most likely via the cuticular plate-rootlet complex. The stereociliary bundles of both types of hair cell are organized in a 'W' formation with a steplike arrangement. In the OHCs, the 'W' formation is sharply angulated and slanted toward the apex, coinciding with the slanted fiber arrangement of the overlying tectorial membrane, which is firmly coupled to the tips of the tallest row of the stereociliary bundles. However, in the IHCs, the 'W' formation is wide and its long axis is linear and arranged at a right angle to the radial axis of the organ of Corti; also, the ciliary bundles are freestanding (with a few exceptions in the basal turn). This arrangement in the IHCs would be best suited for deflection by the radial flow of the endolymph. Present evidence suggests that the subtectorial fluid space exists, is filled with endolymph, and freely communicates with endolymph. Because of the discovery of the phenomenon of 'cochlear emission', the possible motility of the sensory cells, particularly of the OHCs, has drawn intense interest in recent years. Recent investigations with dissociated sensory cells (OHCs) indicate some motile capability under various experimental conditions, although it has not been established that this motility is present in vivo. For this reason, the specialized cellular organization for motility and localization of contractile and cytoskeletal proteins have been investigated. These results support the possibility that the OHCs may have cellular facilities for this function.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

Longitudinal flow of macromolecules in the endolymphatic space of the rat. An autoradiographical study.

Secretion and resorption of sulphated glycoproteins by the epithelial lining of the endolymphatic space of the rat were studied autoradiographically with the use of 35SO4 as a selective marker. This isotope was found to be incorporated into the sensory area of cristae and maculae and a small area of epithelial cells adjacent to the sensory epithelium. Thereafter 35S-labelled glycoproteins were secreted into cupulae and otolithic membranes. No such process could be established in the tectorial membrane. From the gradual disappearance of 35S-labelled glycoproteins from cupulae and otolithic membrane coupled with an accumulation of this substance in the lumen of the endolymphatic sac, the existence of a longitudinal flow under physiological conditions could be concluded. Resorption of glycoproteins by the saccus epithelium was found to be very slow. Accumulation of 35S-labelled glycoproteins in the endolymphatic duct after saccus obstruction indicates that, in addition to the endolymphatic sac, also the duct contributes to the mechanism underlying the longitudinal flow of macromolecules.

Animals↗

Separate forms of pathology in the cochlea of congenitally deaf white cats.

Congenital deafness due to cochlear pathology can have an immediate or progressive onset. The timing of this onset could have a significant impact on the development of structures in the central auditory system, depending on the animal's hearing status during its critical period. In order to determine whether cats in our deaf white cat colony suffered from progressive hearing loss, they were tested repeatedly in 30-day intervals using standard auditory evoked brainstem response (ABR) methodology. ABR thresholds did not change over time, indicating that deafness in our colony was not progressive. Moreover, different forms of cochlear pathology were associated with deafness. One form (67% of the deaf ears) had a collapsed Reissner's membrane that obliterated the scala media, resembling what is called the Scheibe deformity in humans. A second form (18%) exhibited excessive epithelial growth within the bony labyrinth. A third form (15%) combined excessive epithelial growth in the apex and a collapsed Reissner's membrane in the base. Cochleae having an abnormally thin tectorial membrane and an outward bulging Reissner's membrane were associated with elevated thresholds (poor hearing).

Aging↗