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PubMed2003

Steroidogenic enzyme expression in the rat cochlea.

OBJECTIVE: Steroid hormones, and particularly mineralocorticoids, are candidates for controlling the homeostasis of endolymph as steroid receptors are widely expressed in the cochlea. In contrast, experiments on adrenalectomized animals have shown that an absence of steroids had little effect on the ionic composition of endolymph and hearing ability. We thus hypothesized that local production of steroids in the inner ear may regulate cochlear fluid exchanges. RESULTS: Using reverse transcription-polymerase chain reaction techniques, we showed that transcripts encoding the P450 side-chain cleavage, the 3beta hydroxysteroid dehydrogenase (3beta HSD) and the 17alpha hydroxylase (P450 C17) were expressed in the lateral wall, organ of Corti and modiolus. The mRNA encoding aldosterone synthase was expressed in the modiolus and lateral wall while the P450 11beta1 hydroxylase was not detected at all in any of these tissues. In situ hybridization experiments on cochlear sections confirmed that the 3beta HSD transcripts were expressed in the spiral ligament and modiolus and possibly in the hair cells of the organ of Corti. However, it was not possible to detect P450 C17 transcripts. Immunohistochemistry performed with an antibody raised against the various 3beta HSD isoforms confirmed the localization found using in situ hybridization. CONCLUSIONS: These results suggest that enzymes of the steroid pathway leading to mineralocorticoids and sex steroid hormones, but not glucocorticoids, may be expressed in the rat cochlea. This work is in line with the recent description of local production of steroid hormones in the brain (neurosteroids), heart and skin. In the cochlea, the local production of mineralo and sex steroids could account for a paracrine role and could induce specific gene expression through steroid receptors or act via a non-genomic mechanism on membrane receptors or ionic exchangers. Experiments aimed at demonstrating enzymatic activities within cochlea tissues are in progress.

3-Hydroxysteroid Dehydrogenases↗

PubMed2002

Differences in endolymphatic sac mitochondria-rich cells indicate specific functions.

OBJECTIVE/HYPOTHESIS: The purpose of the study was to examine the specific involvement of endolymphatic sac mitochondria-rich cells in endolymph homeostasis. STUDY DESIGN: Transmission electron microscopy and immunohistochemistry were performed on the endolymphatic sac of young adult rats, and two important developmental stages were also investigated. METHODS: Ultrastructural characteristics of endolymphatic sac mitochondria-rich cells were studied more concisely and compared with renal mitochondria-rich cells (i.e., the intercalated cells). In addition, expression of cytokeratins 7 and 19 was determined. RESULTS: Until birth, only one type of mitochondria-rich cell is observed in the rat endolymphatic sac. In young adult animals, distinct differences in mitochondria-rich cell ultrastructure in the endolymphatic sac enables classification into subtypes or configurations. Comparison of endolymphatic sac mitochondria-rich cells with renal intercalated cells reveals striking similarities and provides additional information on their specific function in endolymph homeostasis. Furthermore, differences in cytokeratin expression are determined in endolymphatic sac mitochondria-rich cells. CONCLUSIONS: Differences in morphology of endolymphatic sac mitochondria-rich cells develop after birth and may reflect a distinct functional or physiological state of the cell. In analogy to renal intercalated cells, the distribution patterns of H+-adenosine triphosphatase and Cl-/HCO3- exchanger may differ between subtypes. We propose that subtype A mitochondria-rich cells, from which protruding A mitochondria-rich cells are the activated state, are involved in proton secretion (apical H+-adenosine triphosphatase) and thus are potential candidates for hearing loss accompanying renal tubular acidosis. Subtype B mitochondria-rich cells are the most likely candidates to be affected in Pendred syndrome because of the assumed function of pendrin as apical Cl-/HCO3- exchanger.

Animals↗

PubMed2001

In vitro growth of human endolymphatic sac cells: a transmission electron microscopic and immunohistochemical study in patients with vestibular schwannoma and Ménière's disease.

HYPOTHESIS: Human endolymphatic sac cells have been notoriously difficult to maintain in culture. It was hypothesized that an in vitro environment intended for growth of keratinocytes would also be suitable for human endolymph sac cells. BACKGROUND: Studies on cell physiology of human endolymphatic sac cells have been hampered by difficulties in maintaining them in culture. METHODS: Human endolymphatic sac cells were taken from 10 patients during translabyrinthine skull base surgery for vestibular schwannoma, one of whom also had Ménière's disease. Cell lines of proliferating epithelial cells were obtained after trypsinization and growth in a 3:1 mixture of Dulbecco's modified Eagle medium and Ham's F12 medium supplemented with 10% fetal calf serum. Fibroblast overgrowth was counteracted by the use of so-called cloning rings. During various stages, cells were investigated with transmission electron microscopy and/or immunohistochemistry. RESULTS: Proliferation took place after 2 to 3 days of primary cell culture. The cells were cytokeratin-positive and pleomorphic, and they had abundant polarized microvillus-like projections, numerous coated cytoplasmic pits and vesicles, and a well-developed rough endoplasmic reticulum. CONCLUSION: Cell lines of proliferating human endolymphatic sac cells can be produced with the technique described here and may be a valid tool in studies of human endolymph sac physiology.

Cell Movement↗

PubMed2005

Inner ear abnormalities in a Kcnq1 (Kvlqt1) knockout mouse: a model of Jervell and Lange-Nielsen syndrome.

HYPOTHESIS: Mice lacking functional KCNQ1 (previously known as KvLQT1) channels exhibit functional and structural abnormalities that indicate disturbed production of endolymph. BACKGROUND: Congenital deafness associated with cardiac conduction abnormalities (Jervell and Lange-Nielsen syndrome) is associated with dysfunctional KCNQ1/KCNE1 channel complex. This potassium channel plays a critical role in the production and homeostasis of endolymph by the stria vascularis. A preliminary report documented severe abnormalities of the scala media and vestibular compartments of a single mouse lacking functional KCNQ1 alleles. METHODS: Hearing thresholds were measured in three Kcnq1 knockout mice, two heterozygous mice, and one wild-type mouse by auditory brainstem response recordings using clicks, after which the temporal bones were removed. After fixation and dehydration, the ears were embedded in araldite, sectioned at 20-microm thickness, stained with toluidine blue on glass slides, and examined with the light microscope. RESULTS: Kcnq1 knockout mice were deaf and demonstrated circling behavior. They exhibited a marked atrophy of the stria vascularis, contraction of the endolymphatic compartments, and collapse and adhesion of surrounding membranes. There was a complete degeneration of the organ of Corti and an associated degeneration of the spiral ganglion. CONCLUSION: Kcnq1 knockout mice exhibit histopathologic findings that are comparable to those reported in human temporal bone cases of Jervell and Lange-Nielsen syndrome, and provide further evidence that KCNQ1 channel dysfunction can lead to congenital deafness in this syndrome.

Animals↗

PubMed1999

Insights into ototoxicity. Analogies to nephrotoxicity.

The most common ototoxic compounds in clinical practice are aminoglycoside antibiotics, cisplatin, and loop diuretics (ethacrynic acid and furosemide). These agents also have substantial renal effects in the form of nephrotoxicity or diuresis. The understanding of these renal effects may provide insight into ototoxic mechanisms. For aminoglycosides, the renal proximal tubule cell is susceptible due to high concentration achieved and slow clearance with direct effects on phosphoinositide binding and mitochondrial bioenergetics. Pathogenesis appears to involve iron-induced free-radical formation, since iron chelators prevent nephrotoxicity. Analogous effects of aminoglycosides on the inner and outer hair cells have been observed. Cisplatin is also highly concentrated in the proximal tubule cell. Less is known about the direct toxic effects of this agent on renal cells. Insights into mechanisms or renal tubule cells could be directly relevant to the inner ear. The loop diuretics are direct inhibitors of the Na(+)-K(+)-2Cl- cotransport system, which also exists in the marginal and dark cells of the stria vascularis, which are responsible for endolymph secretion. The ototoxicity of these agents may be indirect, due to changes in ionic composition and fluid volume within the endolymph.

Aminoglycosides↗

PubMed1971

Ethacrynic acid effect on the composition of cochlear fluids.

Ethacrynic acid, when administered to six dogs, caused a fall in the concentration of potassium in endolymph from 145 to 21 milliequivalents per liter and a rise in the concentration of sodium from 5.9 to 146 milliequivalents per liter. The composition of perilymph is unaffected. Perilymph and endolymph are slightly hypertonic as compared to plasma.

Animals↗

PubMed2006

Gastric type H+,K+-ATPase in the cochlear lateral wall is critically involved in formation of the endocochlear potential.

Cochlear endolymph has a highly positive potential of approximately +80 mV known as the endocochlear potential (EP). The EP is essential for hearing and is maintained by K(+) circulation from perilymph to endolymph through the cochlear lateral wall. Various K(+) transport apparatuses such as the Na(+),K(+)-ATPase, the Na(+)-K(+)-2Cl(-) cotransporter, and the K(+) channels Kir4.1 and KCNQ1/KCNE1 are expressed in the lateral wall and are known to play indispensable roles in cochlear K(+) circulation. The gastric type of the H(+),K(+)-ATPase was also shown to be expressed in the cochlear lateral wall (Lecain E, Robert JC, Thomas A, and Tran Ba Huy P. Hear Res 149: 147-154, 2000), but its functional role has not been well studied. In this study we examined the precise localization of H(+),K(+)-ATPase in the cochlea and its involvement in formation of EP. RT-PCR analysis showed that the cochlea expressed mRNAs of gastric alpha(1)-, but not colonic alpha(2)-, and beta-subunits of H(+),K(+)-ATPase. Immunolabeling of an antibody specific to the alpha(1) subunit was detected in type II, IV, and V fibrocytes distributed in the spiral ligament of the lateral wall and in the spiral limbus. Strong immunoreactivity was also found in the stria vascularis. Immunoelectron microscopic examination exhibited that the H(+),K(+)-ATPase was localized exclusively at the basolateral site of strial marginal cells. Application of Sch-28080, a specific inhibitor of gastric H(+),K(+)-ATPase, to the spiral ligament as well as to the stria vascularis caused prominent reduction of EP. These results may imply that the H(+),K(+)-ATPase in the cochlear lateral wall is crucial for K(+) circulation and thus plays a critical role in generation of EP.

Animals↗

PubMed1987

Facilitated transfer of glucose from blood into perilymph in the rat cochlea.

The transport of glucose into cochlear endolymph, perilymph of scala vestibuli and perilymph of scala tympani, and cerebrospinal fluid (CSF) was studied after intravenous administration of tracers of D-glucose, L-glucose, and 3-O-methyl-D-glucose in anesthetized rats. The data showed that D-glucose concentrations in perilymph of scala vestibuli, perilymph of scala tympani, and CSF were approximately 50%, and in endolymph less than 10%, that in plasma; D-glucose concentration in perilymph of scala vestibuli, perilymph of scala tympani, and CSF increased as a linear function of that in plasma; D-glucose entry into perilymph of scala vestibuli, perilymph of scala tympani, and CSF was more rapid than that of L-glucose; after infusion of 3-O-methyl-D-glucose, but not after that of mannitol, both the D-glucose concentration ratio of perilymph over plasma and D-glucose transfer into perilymph were lowered. These results indicate that D-glucose enters into perilymph of scala vestibuli by a facilitated transport, possibly located at the blood-perilymph barrier.

3-O-Methylglucose↗

PubMed2005

WTC deafness Kyoto (dfk): a rat model for extensive investigations of Kcnq1 functions.

KCNQ1 forms K+ channels by assembly with regulatory subunit KCNE proteins and plays a key role in the K+ homeostasis in a variety of tissues. In the heart, KCNQ1 is coassembled with KCNE1 to produce a cardiac delayed rectifier K+ current. In the inner ear, the KCNQ1/KCNE1 complex maintains the high concentration of K+ in the endolymph. In the stomach, KCNQ1 is coassembled with KCNE2 to form the K+ exflux channel that is essential for gastric acid secretion. In the colon and small intestine, KCNQ1 is coassembled with KCNE3 to play an important role in transepithelial cAMP-stimulated Cl- secretion. For further understanding of Kcnq1 function in vivo, an animal model has been required. Here we reported the identification of a coisogenic Kcnq1 mutant rat, named deafness Kyoto (dfk), and the characterization of its phenotypes. WTC-dfk rats carried intragenic deletion at the Kcnq1 gene and showed impaired gain of weight, deafness, and imbalance resulting from the marked reduction of endolymph, prolonged QT interval in the electrocardiogram (ECG), and gastric achlorhydria associated with hypertrophic gastric mucosa. Surprisingly, WTC-dfk rats showed hypertension, which suggested that Kcnq1 might be involved in the regulation of blood pressure. These findings suggest that WTC-dfk rats could represent a powerful tool for studying the physiological functions of KCNQ1 and for the establishment of new therapeutic procedures for Kcnq1-related diseases.

Alleles↗

PubMedDate not supplied

K(+) cycling and its regulation in the cochlea and the vestibular labyrinth.

Potassium (K(+)) plays a very important role in the cochlea. K(+) is the major cation in endolymph and the charge carrier for sensory transduction and the generation of the endocochlear potential. The importance of K(+) handling in the cochlea is marked by the discovery of several forms of hereditary deafness that are due to mutations of K(+) channels. Deafness results from mutations of KCNQ4, a K(+) channel in the sensory hair cells, as well as from mutations of the gap junction proteins GJB2, GJB3 and GJB6 that may facilitate cell-to-cell movements of K(+). Deafness results also from mutations of KCNQ1 or KCNE1, subunits of a K(+) channel that carries K(+) from strial marginal cells and vestibular dark cells into endolymph. Further, deafness results from mutations of KCNJ10, a K(+) channel that generates the endocochlear potential in conjunction with the high K(+) concentration in strial intermediate cells and the low K(+) concentration in the intrastrial fluid spaces. This review details recent advances in the understanding of K(+) transport and its regulation in the cochlea and the vestibular labyrinth.

Cochlea↗

PubMed1981

Electron microscopic studies of the endolymphatic sac in Ménière's disease.

Five specimens of the endolymphatic sac taken from patients with Ménière's disease during epidural drainage surgery were examined under the electron microscope. The clinical history and histopathological findings are presented. The most common and prominent finding was perisaccular fibrosis of the endolymphatic sac which appears to disturb the free absorption of endolymph in the endolymph in the endolymphatic sac resulting in endolymphatic hydrops. Well-preserved epithelial cells of the endolymphatic sac could only be found in one of the specimens.

Aged↗

PubMed1989

Effects of ethyl alcohol and heavy-water administration on vestibulo-ocular reflex in rabbits.

The effects of ethyl alcohol and heavy water on the long-time constant (T1) of the vestibulo-ocular reflex, an index of the dynamics of the cupula-endolymph system of the semicircular canal, were investigated in rabbits. Ethyl alcohol with a specific gravity of 0.793 caused two types of direction-changing positional nystagmus. In the early period after its administration, geotropic type positional nystagmus was induced. In this period, ethyl alcohol reduced T1. In the later period after its administration, apogeotropic type positional nystagmus was induced. In this period, T1 was again prolonged. Heavy water with a specific gravity of 1.10 caused apogeotropic type positional nystagmus and prolonged T1. These findings demonstrated that ethyl alcohol and heavy water directly affect the cupula-endolymph system, suggesting that the semicircular canal was responsible for the positional nystagmus.

Animals↗

PubMedDate not supplied

Endolymphatic hydrops in children.

Apart from clinical observations of Ménière's disease in children, there have only been a few histological descriptions of endolymphatic hydrops (EH) as an incidental finding in children. In this paper we report on the incidence of EH in temporal bones of children. One hundred eighteen temporal bones from 70 infants between newborn and 10 years (average age 12.9 months) were analyzed by light microscopy for EH of the cochlear duct, which was defined as a bulging of Reissner's membrane into the scala vestibuli in more than one turn of the cochlea. All endolymph spaces were carefully examined for factors such as an obliterated ductus reuniens which may have contributed to the development of EH. In 64 (54.2%) of the 118 temporal bones, a bulging of Reissner's membrane in the cochlear duct was found. In 65.6%, the bulging was confined to the apical turn and since the significance of isolated apical hydrops is controversial, we elected not to consider this group as having hydrops. Twenty (16.9%) bones met our criteria for a diagnosis of EH. Compared to the high incidence of bulging in the cochlear duct, fewer saccules and utricles were dilated. In approximately half of the temporal bones (49.2%), the ductus reuniens was collapsed. The ductus reuniens seems to be closed in its normal position and might open when pressure occurs due to an increasing volume of endolymph. If there is a permanent closure, EH may result. Our investigation does not indicate that EH is associated with a specific disease; however, where there were congenital anomalies the incidence of EH was higher.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

PubMed1989

Effects of nitrogen mustard-N-oxide on ionic activities of inner ear fluid and ionic permeabilities of the cochlear partition in the guinea pig.

The effect of nitrogen mustard-N-oxide (NMO) on the endocochlear potential (EP) was investigated from the aspect of the ion concentrations and permeabilities in the cochlea. Compared with the untreated animals, in NMO-treated animals 20 to 30 hours after administration, the EP was decreased (30.8 +/- 3.5 mV in NMO versus 82.4 +/- 1.6 mV in control), the K+ concentration in perilymph of the scala tympani was increased (8.2 +/- 1.0 mM versus 5.3 +/- 0.7 mM), the K+ concentration in endolymph was decreased (128.5 +/- 10.6 mM versus 157.9 +/- 7.9 mM), and the Na+ concentration in endolymph was increased (9.6 +/- 3.6 mM versus 2.5 +/- 0.4 mM). The permeability coefficient for Na+ of the cochlear partition in the NMO-treated animals significantly decreased, while that for Cl- significantly increased. The negative EP, which presumably exists in the normal state, diminished further (-2.7 mV versus -27.8 mV), and the calculated electrogenic potential of the EP was depressed remarkably (33.5 mV versus 110.2 mV). The results suggest that the effects of NMO involved changes in ion permeabilities of the partition and the inhibition of electrogenic transport processes in the cochlea.

Animals↗

PubMed1995

Disturbance of regulation of sodium by cis-diamminedichloroplatinum in perilymph of the guinea pig cochlea.

We studied the acute effects of cis-diamminedichloroplatinum (CDDP) on the cochlear partition and inner ear fluid in the guinea pig. At 48 hours after the administration of a single intramuscular injection of CDDP, 12.5 mg/kg of body weight, the endocochlear resting potential (EP) was significantly decreased to 32.1 +/- 1.8 mV in the treated animals, versus 80.6 +/- 1.0 mV in the control animals. There was a significant rise in potassium (K+), sodium (Na+), and chlorine (Cl-) in the endolymph of the animals treated with CDDP as compared with the control animals. Only Na+ was found to increase significantly in the perilymph, reaching more than twice the level of the control animals; both K+ and Cl- remained within the normal range. Serum electrolytes also remained within the normal range. Evaluation of modified ionic permeabilities across the endolymph-perilymph barrier showed an apparent increase in Na+ permeability and a normal range of K+ and Cl- permeabilities. Histopathologic examination of the cochlea showed a moderate collapse of the endolymphatic space, with atrophy of the stria vascularis and destruction of the outer hair cells. The findings suggest that the acute changes produced in the cochlea by administration of CDDP were attributable to a breakdown in the regulation of Na+ metabolism in the perilymph.

Animals↗

PubMed2003

Network organization of interstitial connective tissue cells in the human endolymphatic duct.

The human endolymphatic duct (ED) and sac of the inner ear have been suggested to control endolymph volume and pressure. However, the physiological mechanisms for these processes remain obscure. We investigated the organization of the periductal interstitial connective tissue cells and extracellular matrix (ECM) in four freshly fixed human EDs by transmission electron microscopy and by immunohistochemistry. The unique surgical material allowed a greatly improved structural and epitopic preservation of tissue. Periductal connective tissue cells formed frequent intercellular contacts and focally occurring electron-dense contacts to ECM structures, creating a complex tissue network. The connective tissue cells also formed contacts with the basal lamina of the ED epithelium and the bone matrix, connecting the ED with the surrounding bone of the vestibular aqueduct. The interstitial connective tissue cells were non-endothelial and non-smooth muscle fibroblastoid cells. We suggest that the ED tissue network forms a functional mechanical entity that takes part in the control of inner ear fluid pressure and endolymph resorption.

Connective Tissue Cells↗

PubMedDate not supplied

Element content of intracochlear fluids, outer hair cells, and stria vascularis as determined by energy-dispersive roentgen ray analysis.

Roentgen ray spectrometry was used to obtain element spectra from isolated samples of perilymph, endolymph, outer hair cells, and stria vascularis obtained by microdissection from the freeze-dried inner ears of chinchillas. The spectra of perilymph and endolymph residues indicated that no cross-contamination of the two cochlear fluids occurs during freeze-drying or sampling. The spectra of outer hair cells suggested that the extracellular fluid in the organ of Corti spaces is similar to perilymph in its ionic content. The spectra of stria vascularis samples indicated low sodium and high phosphorus contents. Energy-dispersive roentgen ray analysis of freeze-dried inner ears appears to be a promising method for low-contamination measurement of ion distribution in the cochlea.

Animals↗

PubMed1992

Hyaluronan synthesis in the adult guinea pig endolymphatic sac.

The endolymphatic sac is believed to play a major role in membranous labyrinth homeostasis by controlling the volume of endolymph, removing debris, and participating in the immune response of the inner ear. The endolymphatic sac is postulated to absorb endolymph and to synthesize and secrete high-molecular-weight and osmotically active glycosaminoglycans (GAGs). The present study examines the ability of in vitro adult guinea pig endolymphatic sac cells to synthesize complex proteins and polysaccharides. The intent is to characterize the nature of these compounds by studying carbon-14 (14C) glucose incorporation in tissue cultured endolymphatic sac specimens using autoradiographic and specific enzymatic digestion techniques. Our results suggest that sac cells can synthesize GAGs and proteins in vitro in proportionately larger amounts than surrounding connective tissue and dura. The principal GAG synthesized by the endolymphatic sac appears to be hyaluronan.

Amylases↗