scholarly journals Immunohistochemical Localization of the Na-K-Cl Co-transporter (NKCC1) in the Gerbil Inner Ear

1997 ◽  
Vol 45 (6) ◽  
pp. 773-778 ◽  
Author(s):  
James J. Crouch ◽  
Nobuki Sakaguchi ◽  
Christian Lytle ◽  
Bradley A. Schulte
Keyword(s):  
Inner Ear ◽  
Stria Vascularis ◽  
Ion Homeostasis ◽  
Human Colon ◽  
Pcr Primers ◽  
Immunohistochemical Localization ◽  
Physiological Data ◽  
Spiral Ligament ◽  
Dark Cells ◽  
Specific Pcr

We mapped the cellular and subcellular distribution of the Na-K-Cl co-transporter (NKCC) in the adult gerbil inner ear by immunostaining with a monoclonal antibody (MAb T4) generated against human colon NKCC. Heavy immunolabeling was seen in the basolateral plasma membrane of marginal cells in the stria vascularis and dark cells in the vestibular system. Subpopulations of fibrocytes in the cochlear spiral ligament and limbus and underlying the vestibular neurosensory epithelium also stained with moderate to strong intensity, apparently along their entire plasmalemma. Because MAb T4 recognizes both the basolateral secretory (NKCC1) and the apical absorptive (NKCC2) isoforms of the co-transporter, we employed reverse transcription and the polymerase chain reaction (RT-PCR) to explore isoform diversity in inner ear tissues. Using NKCC1 and NKCC2 isoform-specific PCR primers based on mouse and human sequences, only transcripts for NKCC1 were detected in the gerbil inner ear. The presence of abundant NKCC1 in the basolateral plasmalemma of strial marginal and vestibular dark cells confirms conclusions drawn from pharmacological and physiological data. The co-expression of NKCC1 and Na,K-ATPase in highly specialized subpopulations of cochlear and vestibular fibrocytes provides further evidence for their role in recycling K+ leaked or effluxed through hair cells into perilymph back to endolymph, as postulated in current models of inner ear ion homeostasis.

Specialized Epithelia in the Avian Labyrinth (Its Distribution and Ultrastructure)

1971 ◽  
Vol 29 ◽  
pp. 404-405
Author(s):  
E. Ishiyama ◽  
J. Weibel ◽  
E. N. Myers
Keyword(s):  
Electron Microscopy ◽  
Inner Ear ◽  
Fluid Transport ◽  
Stria Vascularis ◽  
Dark Cells ◽  
Ultrastructural Characteristics ◽  
Tegmentum Vasculosum ◽  
Light Cells

In recently years the distribution of the non-sensory specialized epithella in the inner ear has been interested among otologists. Increased understanding of the problem dealing with the fluid transport within the endolymph has been obtained these studied. To our knowledge, however, the distribution of the avian specialized cells in the inner ear has not been described adequately. In addition, ultrastructural characteristics of the osmiophilic(dark) cells in the avian labyrinth were not clearly demonstrated. Therefore, the specialized epithlia in the avian labyrinth should be further investigated and the nature of their function defined.To demonstrat this, pigeois were investigated using phase and electron-microscopy. The specialized cells(dark and light cells) were distributed in the crlstae ampullaris, the tegmentum vasculosum of the lagena which is analogous to the stria vascularis in mammals, the saccule, the otolith lagena, the utricle and the crista negletica.

scholarly journals Noncoding microdeletion in mouse Hgf disrupts neural crest migration into the stria vascularis, reduces the endocochlear potential and suggests the neuropathology for human nonsyndromic deafness DFNB39

2019 ◽  
Author(s):  
Robert J. Morell ◽  
Rafal Olszewski ◽  
Risa Tona ◽  
Samuel Leitess ◽  
Julie M. Schultz ◽  
...  
Keyword(s):  
Growth Factor ◽  
Neural Crest ◽  
Inner Ear ◽  
Stria Vascularis ◽  
Ion Homeostasis ◽  
Neural Crest Cells ◽  
Endocochlear Potential ◽  
Wild Type ◽  
Nonsyndromic Deafness ◽  
Hepatocyte Growth

AbstractHepatocyte growth factor (HGF) is a multifunctional protein that signals through the MET receptor. HGF stimulates cell proliferation, cell dispersion, neuronal survival and wound healing. In the inner ear, levels of HGF must be fine-tuned for normal hearing. In mouse, a deficiency of HGF expression limited to the auditory system, or over-expression of HGF, cause neurosensory deafness. In human, noncoding variants in HGF are associated with nonsyndromic deafness DFNB39. However, the mechanism by which these noncoding variants causes deafness was unknown. Here, we reveal the cause of this deafness using a mouse model engineered with a noncoding intronic 10bp deletion (del10) in Hgf, which is located in the 3’UTR of a conserved short isoform (Hgf/NK0.5). Mice homozygous for del10 exhibit moderate-to-profound hearing loss at four weeks of age as measured by pure-tone auditory brainstem responses (ABRs). The wild type +80 millivolt endocochlear potential (EP) was significantly reduced in homozygous del10 mice compared to wild type littermates. In normal cochlea, EPs are dependent on ion homeostasis mediated by the stria vascularis (SV). Previous studies showed that developmental incorporation of neural crest cells into the SV depends on signaling from HGF/MET. We show by immunohistochemistry that in del10 homozygotes, neural crest cells fail to infiltrate the developing SV intermediate layer. Phenotyping and RNAseq analyses reveal no other significant abnormalities in other tissues. We conclude that, in the inner ear, the noncoding del10 mutation in Hgf leads to dysfunctional ion homeostasis in the SV and a loss of EP, recapitulating human DFNB39 deafness.Significance StatementHereditary deafness is a common, clinically and genetically heterogeneous neurosensory disorder. Previously we reported that human deafness DFNB39 is associated with noncoding variants in the 3’UTR of a short isoform of HGF encoding hepatocyte growth factor. For normal hearing, HGF levels must be fined-tuned as an excess or deficiency of HGF cause deafness in mouse. Using a Hgf mutant mouse with a small 10 base pair deletion recapitulating a human DFNB39 noncoding variant, we demonstrate that neural crest cells fail to migrate into the stria vascularis intermediate layer, resulting in a significantly reduced endocochlear potential, the driving force for sound transduction by inner ear hair cells. HGF-associated deafness is a neurocristopathy but, unlike many other neurocristopathies, it is not syndromic.

scholarly journals Creatine kinase in epithelium of the inner ear.

1992 ◽  
Vol 40 (2) ◽  
pp. 185-192 ◽  
Author(s):  
S S Spicer ◽  
B A Schulte
Keyword(s):  
Creatine Kinase ◽  
Inner Ear ◽  
Vestibular System ◽  
Hair Cells ◽  
Stria Vascularis ◽  
Photoreceptor Cells ◽  
Cell Types ◽  
Dark Cells ◽  
Transitional Cells ◽  
Marginal Cells

Epithelium of the inner ear in the gerbil and mouse was examined immunocytochemically for presence of creatine kinase (CK). Marginal cells of the cochlear stria vascularis and dark cells and transitional cells of the vestibular system were found to contain an abundance of the MM isozyme (MM-CK). CK in these cells concurs with that which is coupled to Na,K-ATPase in other cells and is considered to supply ATP for the Na,K-ATPase that mediates the high KCl of endolymph. Inner hair cells revealed content of the BB isozyme and in this respect resembled the energy-transducing photoreceptor cells in retina. In addition, outer phalangeal (Deiters') cells stained for both MM- and BB-CK whereas inner phalangeal cells evidenced content of only the BB isozyme. Immunolocalization of CK appeared similar in mouse and gerbil inner ear. Specificity of the staining was affirmed by observations in agreement with those reported for CK in various cell types and by staining with antisera from more than one source.

The localization and specificity of guinea pig inner ear antigenic epitopes

1995 ◽  
Vol 109 (1) ◽  
pp. 19-23 ◽  
Author(s):  
Ming-Yu Cao ◽  
Michel Gersdorff ◽  
Naïma Deggouj ◽  
Jean-Paul Tomasi
Keyword(s):  
Guinea Pig ◽  
Inner Ear ◽  
Basilar Membrane ◽  
Stria Vascularis ◽  
Polyacrylamide Gel Electrophoresis ◽  
Spiral Ligament ◽  
Antigenic Epitope ◽  
Acoustic Nerve ◽  
The Brain ◽  
Antigenic Epitopes

AbstractIn this study, we investigated the relative localization of some antigenic epitopes in the inner ear. The inner ear protein antigens were extracted from various parts of the guinea pig inner ear. Brain, kidney, lung, heart and liver extracts were also obtained. We found by SDS-polyacrylamide gel electrophoresis that total inner ear extracts separated into three high concentration polypeptide bands with molecular weights of approximately 30, 42, 58 kd and three low density bands of 20,25 and 35 kd. The 30 kd band was found mainly in the extract of the spiral ganglion and the acoustic nerve in the modiolus. The 42 and 58 kd bands were detected in the extract of the spiral ligament and the stria vascularis. The Organ of Corti and the basilar membrane extract gave rise to three bands of 30,42 and 58 kd. Twenty-eight of the 75 sera from patients with inner ear disease reacted with the 30 and 58 kd bands of the inner ear protein extracts by immunoblotting. Sixteen of these 28 positive sera were then used to probe immunoblots of the brain, kidney, lung, heart and liver extracts. The 58 kd band was also found in protein extracts of the brain, the lung and the liver. This study suggests that the 30 kd antigenic epitope may be mainly related to the acoustic nerve and that the 58 kd antigenic epitope is not cochlear specific.

S239 – Labyrinthine Changes in Otitis Media

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P155-P155
Author(s):  
Shruti Siddharth Joglekar ◽  
Armin Farajzadeh Deroee ◽  
Norimasa Morita ◽  
Sebahattin Cureoglu ◽  
Schachern Patricia ◽  
...  
Keyword(s):  
Inner Ear ◽  
Otitis Media ◽  
Stria Vascularis ◽  
Chronic Otitis Media ◽  
Inflammatory Cells ◽  
Spiral Ligament ◽  
Histopathologic Study ◽  
Significant Difference ◽  
Temporal Bones ◽  
Inflammatory Changes

Objectives Otitis media causes labyrinthine changes and subsequent sensorineural hearing loss. The aim of this histopathologic study was to evaluate the extension of inflammation to the inner ear and its effects. Methods Out of 614 temporal bones with otitis media, 47 (30 cases) with chronic and 35 (21 cases) with purulent were selected for histopathologic study. Subjects with a history of acoustic trauma, head trauma, ototoxic drugs and other otologic and systemic diseases affecting the inner ear were excluded. The pattern of labyrinthine inflammation was classified as localized purulent, localized serous, generalized seropurulent and generalized serous. Inner ear findings were compared to age-matched controls. Results 19% of temporal bones with chronic and 9% of temporal bones with purulent otitis media showed labyrinthine inflammatory changes. In chronic otitis media, inflammatory changes were: 56% localized purulent; 22% localized serous; 11% generalized seropurulent; and 11% generalized serous. Inflammatory changes in temporal bones with purulent otitis media included: 67% localized purulent; and 33% generalized seropurulent. Pathological findings included: serofibrinous precipitates and inflammatory cells in the scala tympani of basal turn and cochlear aqueduct; significant decrease in area of stria vascularis (p = 0.033); and loss of hair cells in the organs of Corti. No significant difference was found in area of spiral ligament area or number of fibrocytes in diseased and control bones. Conclusions Middle ear/inner ear interaction in otitis media can result in labyrinthine inflammation and cochlear damage. Early diagnosis and treatment of otitis media is important in preventing inner ear damage.

scholarly journals Immunohistochemical localization of vimentin in the gerbil inner ear.

1989 ◽  
Vol 37 (12) ◽  
pp. 1787-1797 ◽  
Author(s):  
B A Schulte ◽  
J C Adams
Keyword(s):  
Epithelial Cells ◽  
Inner Ear ◽  
Hair Cells ◽  
Stria Vascularis ◽  
Cell Types ◽  
Immunohistochemical Localization ◽  
Outer Hair Cells ◽  
Type I ◽  
Basal Cells

Cells containing immunoreactive vimentin-type intermediate filaments (IF) were identified in paraffin sections and whole-mount preparations of the gerbil inner ear. Most connective tissue cells showed positive immunostaining, although one unusual class of stromal cell lacked vimentin. Several different types of epithelial cells contained high levels of vimentin. In the cochlea, Deiters' cells, inner phalangeal cells, Boettcher's cells, some outer sulcus cells, and the intermediate cells of the stria vascularis showed strong immunoreactivity. Strial basal cells exhibited weaker and less consistent staining. Neither inner nor outer hair cells were stained. In the vestibular system, hair cells with a morphology and location more characteristic of type I than of type II cells showed strong immunostaining for vimentin. Supporting cells in vestibular neurosensory epithelium stained with less intensity. These results were surprising because epithelial cells in vivo only rarely express vimentin-type IF. Although the functional significance of vimentin remains to be established, its presence in some but not other highly specialized cell types provides an excellent marker for investigating the lineage and morphogenesis of the complex inner ear tissues.

Morphologic Effects of Glycerol and Urea on Cochlear Tissues of the Chinchilla

1988 ◽  
Vol 97 (1) ◽  
pp. 67-73 ◽  
Author(s):  
Charles G. Wright ◽  
David H. Lee ◽  
William L. Meyerhoff ◽  
Peter S. Roland
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Endolymphatic Hydrops ◽  
Stria Vascularis ◽  
Metabolic Stress ◽  
Mechanisms Of Action ◽  
Outer Hair Cells ◽  
Ultrastructural Changes ◽  
Spiral Ligament ◽  
Subcutaneous Injections

Glycerol and urea are used as test agents in confirming the diagnosis of endolymphatic hydrops. Although both substances act as osmotic diuretics, recent evidence suggests that they may have differing physiologic effects on the inner ear. This study was designed to compare the morphologic effects of urea and glycerol on cochlear tissues, using the chinchilla as an experimental model. Animals were given subcutaneous injections of glycerol (2 g/kg) or urea (1.2 g/kg) over periods of 3 hours, 4 days, or 1 week. Both agents were found to produce ultrastructural changes, including spiral ligament vacuolization, intracellular alterations of the stria vascularis, and increased numbers of Hensen's bodies in outer hair cells. These alterations appeared indicative of metabolic stress, but not toxicity. The morphologic findings provided no evidence that glycerol and urea affect the inner ear by fundamentally different mechanisms of action.

Stria vascularis and vestibular dark cells: characterisation of main structures responsible for inner-ear homeostasis, and their pathophysiological relations

2008 ◽  
Vol 123 (2) ◽  
pp. 151-162 ◽  
Author(s):  
R R Ciuman
Keyword(s):  
Inner Ear ◽  
Volume Concentration ◽  
Current Knowledge ◽  
Stria Vascularis ◽  
Dark Cells ◽  
Adequate Response ◽  
Complex Process ◽  
And Function ◽  
Vestibular Dark Cells ◽  
Distinct Morphology

AbstractThe regulation of inner-ear fluid homeostasis, with its parameters volume, concentration, osmolarity and pressure, is the basis for adequate response to stimulation. Many structures are involved in the complex process of inner-ear homeostasis. The stria vascularis and vestibular dark cells are the two main structures responsible for endolymph secretion, and possess many similarities. The characteristics of these structures are the basis for regulation of inner-ear homeostasis, while impaired function is related to various diseases. Their distinct morphology and function are described, and related to current knowledge of associated inner-ear diseases. Further research on the distinct function and regulation of these structures is necessary in order to develop future clinical interventions.

Sign in / Sign up

Export Citation Format

Share Document