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PubMed2003

Trophic support of mouse inner ear by neural stem cell transplantation.

In the auditory system, efforts to reduce degeneration of spiral ganglion neurons have the immediate objective of improving clinical benefits of cochlear implants, which are small devices designed to stimulate spiral ganglion neurons electronically. Recent studies have indicated several neurotrophins can enhance survival of spiral ganglion neurons. However, the strategy for application of neurotrophins in inner ear is still a matter of debate. In this study, we examined the potential of cell therapy as a strategy for application of neurotrophins in the inner ear. Neural stem cells obtained from green fluorescent protein-transgenic mice were used as donor cells. Medium containing neural stem cells was injected into mouse inner ear. Histological analysis 4 weeks later revealed that transplant-derived cells survived in inner ear and that most transplant-derived cells in the cochlea had differentiated into glial cells. Moreover, expression of glial cell line-derived neurotrophic factor and brain-derived neurotrophic factor was observed in transplant-derived cells. These findings indicate that transplantation of neural stem cells can be a useful strategy for application of neurotrophins in inner ear.

Animals↗
PubMed1992

Immune-mediated inner ear disease.

Recent clinical studies, experimental research, and various testing techniques in otoimmunology have resulted in presentation of the synonymous terms autoimmune sensorineural hearing loss, autoimmune inner ear disease, immune-mediated sensorineural hearing loss, and immune-mediated inner ear disease or disorder. The development of this terminology and the clinical presentation of this disease as well as whether it is really a distinct clinical entity are discussed. Laboratory tests for immune-mediated inner ear disease are presented along with a discussion of progress being made in the growing field of otoimmunology.

Antigen-Antibody Complex↗
PubMed1985

Distribution of gentamicin by immunofluorescence in the guinea pig inner ear.

We studied the distribution of gentamicin in the inner ear, brain and kidney of the guinea pig following intraperitoneal administration or perfusion of gentamicin through the perilymphatic space. The resulting histopathological changes were examined by immunofluorescence using antigentamicin antiserum. After perfusion of gentamicin through the perilymphatic space, specific fluorescence was found in the cochlea, and was especially prominent in the outer hair cells, basilar membrane and basilar crest. Although no fluorescence was observed in the cochlea following intraperitoneal administration of high doses of gentamicin, type I hair cells in the vestibule were seen to be selectively stained with the antibody. Furthermore, some of the vestibular ganglion cells, Purkinje cells and unidentified nuclei in the brain stem were also stained. In particular, fine granules showing relatively intense fluorescence were recognized in the cytoplasm of the stained cells. In the cortex of kidney, only proximal tubular cells were stained with intense fluorescence. Our results suggest that the aminoglycoside antibiotics have two sites of action: one is the cell membrane of the sensory hair cells and the other is the cytoplasm.

Animals↗
PubMed2002

Revisiting cell fate specification in the inner ear.

Generating the diversity of cell types in the inner ear may require an interplay between regional compartmentalization and local cellular interactions. Recent evidence has come from gene targeting, lineage analysis, fate mapping and gene expression studies. Notch signaling and neurogenic gene regulation are involved in patterning or specification of sensory organs, ganglion cells and hair cell mechanoreceptors.

Animals↗
PubMed1999

The deltaA gene of zebrafish mediates lateral inhibition of hair cells in the inner ear and is regulated by pax2.1.

Recent studies of inner ear development suggest that hair cells and support cells arise within a common equivalence group by cell-cell interactions mediated by Delta and Notch proteins. We have extended these studies by analyzing the effects of a mutant allele of the zebrafish deltaA gene, deltaA(dx2), which encodes a dominant-negative protein. deltaA(dx2/dx2 )homozygous mutants develop with a 5- to 6-fold excess of hair cells and a severe deficiency of support cells. In addition, deltaA(dx2/dx2) mutants show an increased number of cells expressing pax2.1 in regions where hair cells are normally produced. Immunohistological analysis of wild-type and deltaA(dx2/dx2) mutant embryos confirmed that pax2.1 is expressed during the initial stages of hair cell differentiation and is later maintained at high levels in mature hair cells. In contrast, pax2.1 is not expressed in support cells. To address the function of pax2.1, we analyzed hair cell differentiation in no isthmus mutant embryos, which are deficient for pax2.1 function. no isthmus mutant embryos develop with approximately twice the normal number of hair cells. This neurogenic defect correlates with reduced levels of expression of deltaA and deltaD in the hair cells in no isthmus mutants. Analysis of deltaA(dx2/dx2); no isthmus double mutants showed that no isthmus suppresses the deltaA(dx2) phenotype, probably by reducing levels of the dominant-negative mutant protein. This interpretation was supported by analysis of T(msxB)(b220), a deletion that removes the deltaA locus. Reducing the dose of deltaA(dx2) by generating deltaA(dx2)/T(msxB)(b220 )trans-heterozygotes weakens the neurogenic effects of deltaA(dx2), whereas T(msxB)(b220) enhances the neurogenic defects of no isthmus. mind bomb, another strong neurogenic mutation that may disrupt reception of Delta signals, causes a 10-fold increase in hair cell production and is epistatic to both no isthmus and deltaA(dx2). These data indicate that deltaA expressed by hair cells normally prevents adjacent cells from adopting the same cell fate, and that pax2.1 is required for normal levels of Delta-mediated lateral inhibition.

Animals↗
PubMed1995

[Thermal stress of the inner ear during laser stapedotomy. I: Continuous-wave laser].

As a consequence of perforating the footplate during laser stapedotomy, direct radiation to the inner ear will warm perilymph and adjacent structures. To determine the possible thermal dangers to cochlear structures from different laser parameters, heat transport mechanisms, temperature increases and temperature fields were investigated in a model system approximating caloric and physiologic changes in the inner ear. The temperature-time course of local cochlear warming showed a rapid convection-dependent increase that reached a peak at about the end of the laser impulse. An increase in power density caused an elevation of the temperature in all laser systems used. Maximum temperatures varied widely for CO2 lasers at the same wave-length, but a different beam-time behavior was found at a distance of 2 mm behind the perforation by using low-power densities. Heat values were lowest at a pulse duration of 50 ms in the superpulse (< 5 degrees C) and continuous wave (cw) modes (< 9 degrees C), while the highest value was found in the pulser mode (to 21 degrees C). After argon laser irradiation at high-power densities, temperatures were nearly independent of location (5.5-13 degrees C). When considering risks of possible inner ear damage from thermal stress during laser stapedotomy, application of the CO2 superpulse and cw laser appears to be safe over a large power-density range. Low energies using a small-beam diameter and short pulse durations (50-100 ms) are recommended. In contrast, use of the CO2 laser in a pulser mode may result in inner-ear damage because of the high temperatures produced. Structures located at a greater distance can be endangered by direct irradiation with the argon laser.

Body Temperature Regulation↗
PubMed2001

The expression of fibroblast growth factors and their receptors in the embryonic and neonatal mouse inner ear.

Four different fibroblast growth factor receptors (FGFR) are known, three of which have splice variants (known as the b and c variants) in the FGF-binding domain, to give different patterns of sensitivity to the different FGFs. The expression of the b and c variants of the FGF receptors, together with the expression of the ligands FGF1, FGF2, FGF3, FGF7, FGF8b and FGF8c, was determined by quantitative reverse transcription-polymerase chain reaction in developing whole mouse inner ears, and in dissected components of the postnatal mouse inner ear. At embryonic age (E)10.5 days, when the otocyst is a simple closed sac, the receptor most heavily expressed was FGFR2b, relative to the postnatal day 0 level. Over the period E10.5-E12.5, during which the structures of the inner ear start to form, the expression of the different FGF receptors increased 10(2)-10(4) fold per unit of tissue, and there was a gradual switch towards expression of the 'c' splice variants of FGFR2 and FGFR3 rather than the 'b' variants. At E10.5, the ligands most heavily expressed, relative to the postnatal day 0 level, were FGF3, FGF8b and FGF8c. In the postnatal inner ear, the patterns of expression of receptors and ligands tended to be correlated, such that receptor variants were expressed in the same regions as the ligands that are known to activate them effectively. The neural/sensory region expressed high levels of FGFR3c, and high levels of the ligand FGF8b. The same area also expressed high levels of FGFR1b and FGFR2b, and high levels of FGF3. The lateral wall of the cochlea (including the stria vascularis and the spiral ligament) expressed high levels of FGFR1c and FGF2. It is suggested that the different FGF receptors and ligands are expressed in a spatially coordinated pattern, to selectively program cochlear development.

Animals↗
PubMed2007

Myosin VI and VIIa distribution among inner ear epithelia in diverse fishes.

Unconventional myosins are critical motor proteins in the vertebrate inner ear. Mutations in any one of at least six different myosins can lead to human hereditary deafness, but the precise functions of these proteins in the ear are unknown. This study uses a comparative approach to better understand the role of myosins VI and VIIa in vertebrate ears by examining protein distribution for these two myosins in the ears of evolutionarily diverse fishes and the aquatic clawed toad Xenopus laevis. Both myosins are expressed in the inner ears of all species examined in this study. Myo7a localizes to hair cells, particularly the actin-rich hair bundle, in all species studied. Myo6 also localizes to hair cells, but its distribution differs between species and end organs. Myo6 is found in hair bundles of most fish and frog epithelia examined here but not in anterior and posterior utricular hair bundles of American shad. These results show that myo7a distribution is highly conserved in diverse vertebrates and suggest functional conservation as well. The finding of myo6 in fish and Xenopus hair bundles, however, suggests a novel role for this protein in anamniotic hair cells. The lack of myo6 in specific American shad utricular hair bundles indicates a unique quality of these cells among fishes, perhaps relating to ultrasound detection capability that is found in this species.

Actins↗
PubMedDate not supplied

Congenital malformations of middle and inner ears of parabiotic twins.

We describe herein the congenital malformations of the middle and inner ears in temporal bones of parabiotic, monozygotic twins. Temporal bones were removed from twin B, who had no fetal cardiac activity and was born dead at 23-4/7 weeks, and twin A, the donor or "pump" twin in intrauterine life, who died shortly after birth at 20-6/7 weeks. The temporal bones were processed routinely in celoidin, stained with hematoxylin and eosin, and examined by light microscopy. We found that twin B had Mondini's dysplasia with associated deformities of the middle ear and in general showed more developmental anomalies than twin A, and we conclude that Mondini's dysplasia with anomalies of the middle ear may occur in the parabiotic twin syndrome, and the abnormalities may be explainable as the result of vascular disturbance, which also causes other lesions in these unusual cases.

Ear, Inner↗
PubMed1998

Effects of columella removal on inner ear morphology in the chick.

We are currently removing the single middle ear bone (columella) in the domestic chick to introduce chemical agents directly into the inner ear. Since we are interested in the effect of these agents on neural structures within the avian basilar papilla (BP), we are concerned about any subtle changes that might result from the surgical procedure of columella removal alone. The purpose of this study was to use light and transmission electron microscopy to analyze morphological changes in the inner ear after columella removal. Fifteen-day-old chicks underwent a unilateral, bilateral or a sham removal of the columella. After columella removal, the oval window was either plugged with Gelfoam or Kimwipe (standard accepted procedure to prevent possible perilymph leak) or left uncovered. After a 5-day survival period, morphological changes were observed in the tegmentum vasculosum (TV) of all ears receiving a columella removal as compared to unoperated ears. Further, ears with Gelfoam plugging the oval window also had damage to the hair cells and support cells of the basilar papilla. In contrast, there were no observable differences in either auditory afferent or efferent nerve terminals on hair cells in the BP from any ears that had the columella removed compared to those from unoperated ears. These results suggest that columella removal alone may produce morphological changes to the TV within 5 days of surgery but not to structures within the BP. On the other hand, columella removal with a Gelfoam plug results in damage not only to the TV but also to cells within the basilar papilla during this same survival time. Despite damage to other structures within the inner ear, cochlear efferent and afferent terminals on surviving hair cells were unaffected by columella removal with or without plugging.

Animals↗
PubMed2000

Sensory organ generation in the chicken inner ear: contributions of bone morphogenetic protein 4, serrate1, and lunatic fringe.

The chicken inner ear is a remarkably complex structure consisting of eight morphologically distinct sensory organs. Unraveling how these sensory organs are specified during development is key to understanding how such a complex structure is generated. Previously, we have shown that each sensory organ in the chicken inner ear arises independently in the rudimentary otocyst based on Bone morphogenetic protein 4 (Bmp4) expression. Here, we compare the expression of Bmp4 with two other putative sensory organ markers, Lunatic Fringe (L-fng) and chicken Serrate1 (Ser1), both of which are components of the Notch signaling pathway. L-fng and Ser1 expression domains were asymmetrically distributed in the otic cup. At this early stage, expression of L-fng is similar to Delta1 (Dl1), in an anteroventral domain apparently corresponding to the neurogenic region, while Ser1 is expressed at both the anterior and posterior poles. By the otocyst stage, the expression of both L-fng and Ser1 largely coincided in the medial region. All presumptive sensory organs, as identified by Bmp4 expression, arose within the broad L-fng- and Ser1-positive domain, indicating the existence of a sensory-competent region in the rudimentary otocyst. In addition, there is a qualitative difference in the levels of expression between L-fng and Ser1 such that L-fng expression was stronger in the ventral anterior, whereas Ser1 was stronger in the dorsal posterior region of this broad domain. This early difference in expression may presage the differences among sensory organs as they arise from this sensory competent zone.

Age Factors↗
PubMed1990

Methods for cellular and subcellular visualization of intermediate filament proteins in the human inner ear.

The preservation of antigenicity for monoclonal antibodies (mAbs) directed against the five classes of intermediate filament proteins and their subgroups was analysed in inner ear specimens from both early embryonic (6-8 gestation-week-old) human labyrinths and inner ears from newborn CBA/CBA mice. After initial fixation in 2% paraformaldehyde, the specimens were embedded in either polyvinyl alcohol (PVA) or the low viscosity acrylic resin LR White. Both embedding media allowed sectioning at room temperature with a specimen thickness of 0.5-1 microns, which gives a resolution at the subcellular level in the light microscope. Immunoreactivity occurred in the PVA-embedded material, but not in specimens embedded in LR White. However, considerably fewer mAbs showed immunostaining in the PVA-embedded material than in both cryofixed-cryosectioned or paraformaldehyde-fixed-cryosectioned human inner ears. Immunoelectron microscopy using colloidal gold (particles 10 nm in diameter) was successful in the PVA-embedded (but not the LR White-embedded) material.

Acrylic Resins↗
PubMed1999

Targeted mutagenesis of the POU-domain gene Brn4/Pou3f4 causes developmental defects in the inner ear.

Targeted mutagenesis in mice demonstrates that the POU-domain gene Brn4/Pou3f4 plays a crucial role in the patterning of the mesenchymal compartment of the inner ear. Brn4 is expressed extensively throughout the condensing mesenchyme of the developing inner ear. Mutant animals displayed behavioral anomalies that resulted from functional deficits in both the auditory and vestibular systems, including vertical head bobbing, changes in gait, and hearing loss. Anatomical analyses of the temporal bone, which is derived in part from the otic mesenchyme, demonstrated several dysplastic features in the mutant animals, including enlargement of the internal auditory meatus. Many phenotypic features of the mutant animals resulted from the reduction or thinning of the bony compartment of the inner ear. Histological analyses demonstrated a hypoplasia of those regions of the cochlea derived from otic mesenchyme, including the spiral limbus, the scala tympani, and strial fibrocytes. Interestingly, we observed a reduction in the coiling of the cochlea, which suggests that Brn-4 plays a role in the epithelial-mesenchymal communication necessary for the cochlear anlage to develop correctly. Finally, the stapes demonstrated several malformations, including changes in the size and morphology of its footplate. Because the stapes anlage does not express the Brn4 gene, stapes malformations suggest that the Brn4 gene also plays a role in mesenchymal-mesenchymal signaling. On the basis of these data, we suggest that Brn-4 enhances the survival of mesodermal cells during the mesenchymal remodeling that forms the mature bony labyrinth and regulates inductive signaling mechanisms in the otic mesenchyme.

Acoustic Stimulation↗
PubMed1985

Oxygenation through the round window membrane and the inner ear function.

The effect of oxygenation through the round window membrane was estimated using polarographic technique and the combined action potential of the inner ear. The calculated amount of oxygen permeable through the round window membrane (14% in room air and 80% in pure oxygen) revealed to have very limited effect in maintaining the inner ear potential. Carbogen inflation in maintaining the N1 potential during asphyxia was significantly effective only at 8 kHz. Oxygen supply by this route, however, may enable the inner ear tissue alive even in misery perfusion and recover the high tone potential as a therapy of otitis media with effusion.

Animals↗
PubMed2004

Localization of the mitochondrial uncoupling protein family in the rat inner ear.

Uncoupling proteins (UCPs) are a proton transporter family located in the mitochondrial inner membrane. The molecular expression and activity of UCPs in brown adipose tissue and skeletal muscle are regulated by factors as diverse as chronic overeating and cold exposure, suggesting roles in energy expenditure and heat production. Although UCP2, UCP4 and brain mitochondrial carrier protein-1 (BMCP-1, i.e. UCP5) mRNAs are expressed in the central nervous system, their central function is unknown. This study presents the first evidence on localization and quantitative expression of UCPs in the rat inner ear by real-time PCR and immunohistochemistry. Real-time PCR studies revealed that UCP2 mRNA was expressed in the vestibular and spiral ganglia more abundantly than any other UCP. Neocortex, by contrast, contained UCP2 and UCP4 equally. Notably, UCP3 and UCP4 mRNAs were expressed in inner ear ganglia, but brain UCP3 mRNA expression level was undetectable by simple PCR. Immunohistochemical studies confirmed that both UCP2- and UCP3-like immunoreactivities were detected in vestibular and spiral ganglion cells and co-localized with a mitochondrial marker, MitoFluorGreen. According to previous reports, UCP2 and UCP3 are thermogenic in yeast and brain UCP2 has been suggested to modulate pre- and post-synaptic events by axonal thermogenesis. It has also been reported recently that UCP2 and UCP3 responses to superoxide application may be an antioxidant protective mechanism. Therefore, it is suggested that mitochondrial UCPs (UCP2, UCP3, UCP4) may play both a protective role against oxidative damage and a thermal signaling role in the eighth nerve.

Animals↗
PubMed2004

Identification and characterization of an L-type Cav1.2 channel in spiral ligament fibrocytes of gerbil inner ear.

Intracellular free Ca2+ levels are critical to the activity of BK channels in inner ear type I spiral ligament fibrocytes. However, the mechanisms for regulating intracellular Ca2+ levels in these cells are currently poorly understood. Using patch-clamp technique, we have identified a voltage-dependent L-type Ca2+ channel in type I spiral ligament fibrocytes cultured from gerbil inner ear. With 10 mM Ba2+ as the conductive cation, an inwardly rectifying current was elicited with little inactivation by membrane depolarization. The voltage activation threshold and the half-maximal voltage activation were -40 and -6 mV, respectively. This inward whole-cell current reached its peak at around 10 mV of membrane potential. The amplitude of the peak current varied among cells ranging from 50 to 274 pA with an average of 132.4 +/- 76.2 pA (n = 19); 10(-6) M nifedipine significantly inhibited the inward currents by 90.3 +/- 1.2% (n = 11). RT-PCR analysis revealed that cultured type I spiral ligament fibrocytes express the alpha1C isoform of the L-type Ca2+ channels encoded by the Cav1.2 gene. The expression of this channel in gerbil inner ear was confirmed by RT-PCR analysis using freshly isolated spiral ligament tissues. The Cav1.2 channel may function in conjunction with a previously identified intracellular Ca-ATPase (SERCA) to regulate intracellular free Ca2+ levels in type I spiral ligament fibrocytes, and thus modulate BK channel activity in these cells.

Animals↗
PubMedDate not supplied

An experimental study of inner ear pathology due to NaCl on the round window.

An animal experiment was performed to evaluate structural and ultrastructural changes to the inner ear as a result of placing 3-4 crystals of reagent grade sodium chloride (NaCl) on the round window membrane. Chinchillas were sacrificed at 8 and 24 hours after treatment and the cochlear and vestibular tissues were examined by light microscopy and scanning electron microscopy. Inner ear pathology consisted of destruction of both sensory and supporting cells in the basal turn of the organ of Corti, atrophy of the stria vascularis and alterations to the otoconia and the maculae and ampullae of the vestibular system. This study demonstrates that NaCl in the middle ear does not provide a model for Meniere's disease as previously suggested by Arslan. It may, however, be utilized in the destructive treatment of selected inner ear disorders.

Animals↗
PubMed1987

Experimental immune system of the inner ear.

The observation that certain clinical cases of sensorineural hearing loss could favorably respond to immunosuppressive therapy in addition to experimental studies that support the presence of an inner ear immune system have led to the suggestion that a number of inner ear disorders previously considered to be idiopathic may in fact have an immunological basis. The experimental evidence that establishes the operation of a distinct inner ear immune system is presented.

Antibody Formation↗