scholarly journals The endocochlear potential depends on two K+ diffusion potentials and an electrical barrier in the stria vascularis of the inner ear

2008 ◽  
Vol 105 (5) ◽  
pp. 1751-1756 ◽  
Author(s):  
F. Nin ◽  
H. Hibino ◽  
K. Doi ◽  
T. Suzuki ◽  
Y. Hisa ◽  
...  
Keyword(s):  
Inner Ear ◽  
Stria Vascularis ◽  
Endocochlear Potential ◽  
Diffusion Potentials

scholarly journals KAB-2 (Kir4.1) which is essential for endocochlear potential of inner ear, is not expressed In stria vascularis of deaf mutant WV/WV mice

1997 ◽  
Vol 73 ◽  
pp. 82
Author(s):  
Hiroshi Hibino ◽  
Yoshiyuki Horio ◽  
Mitsuhiko Yamada ◽  
Atsushi Inanobe ◽  
Katsumi Doi ◽  
...  
Keyword(s):  
Inner Ear ◽  
Stria Vascularis ◽  
Endocochlear Potential

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 Preferential Cochleotoxicity of Cisplatin

2021 ◽  
Vol 15 ◽  
Author(s):  
Pattarawadee Prayuenyong ◽  
David M. Baguley ◽  
Corné J. Kros ◽  
Peter S. Steyger
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Driving Force ◽  
Stria Vascularis ◽  
Endocochlear Potential ◽  
Current Evidence ◽  
Cochlear Hair Cells ◽  
Anatomy And Physiology ◽  
Mechanoelectrical Transduction ◽  
Direct Exposure

Cisplatin-induced ototoxicity in humans is more predominant in the cochlea than in the vestibule. Neither definite nor substantial vestibular dysfunction after cisplatin treatment has been consistently reported in the current literature. Inner ear hair cells seem to have intrinsic characteristics that make them susceptible to direct exposure to cisplatin. The existing literature suggests, however, that cisplatin might have different patterns of drug trafficking across the blood-labyrinth-barrier, or different degrees of cisplatin uptake to the hair cells in the cochlear and vestibular compartments. This review proposes an explanation for the preferential cochleotoxicity of cisplatin based on current evidence as well as the anatomy and physiology of the inner ear. The endocochlear potential, generated by the stria vascularis, acting as the driving force for hair cell mechanoelectrical transduction might also augment cisplatin entry into cochlear hair cells. Better understanding of the stria vascularis might shed new light on cochleotoxic mechanisms and inform the development of otoprotective interventions to moderate cisplatin associated ototoxicity.

The fine structure of the stria vascularis of the cat inner ear

1966 ◽  
Vol 118 (2) ◽  
pp. 631-663 ◽  
Author(s):  
R. Hinojosa ◽  
E. L. Rodriguez-Echandia
Keyword(s):  
Fine Structure ◽  
Inner Ear ◽  
Stria Vascularis

Prenatal maturation of endocochlear potential and electrolyte composition of inner ear fluids in guinea pigs

1983 ◽  
Vol 237 (2) ◽  
pp. 147-152 ◽  
Author(s):  
Yehoash Raphael ◽  
Masaki Ohmura ◽  
Naoyuki Kanoh ◽  
Nobuya Yagi ◽  
Kazuo Makimoto
Keyword(s):  
Inner Ear ◽  
Guinea Pigs ◽  
Endocochlear Potential ◽  
Electrolyte Composition ◽  
Inner Ear Fluids

KCNJ10 (Kir4.1) potassium channel knockout abolishes endocochlear potential

2002 ◽  
Vol 282 (2) ◽  
pp. C403-C407 ◽  
Author(s):  
Daniel C. Marcus ◽  
Tao Wu ◽  
Philine Wangemann ◽  
Paulo Kofuji
Keyword(s):  
Driving Force ◽  
Stria Vascularis ◽  
Endocochlear Potential ◽  
Sensory Transduction ◽  
Basal Cells ◽  
Transport Pathways ◽  
Main Charge ◽  
Marginal Cells ◽  
Intermediate Cells ◽  
Two Barriers

Stria vascularis of the cochlea generates the endocochlear potential and secretes K+. K+ is the main charge carrier and the endocochlear potential the main driving force for the sensory transduction that leads to hearing. Stria vascularis consists of two barriers, marginal cells that secrete potassium and basal cells that are coupled via gap junctions to intermediate cells. Mice lacking the KCNJ10 (Kir4.1) K+ channel in strial intermediate cells did not generate an endocochlear potential. Endolymph volume and K+ concentration ([K+]) were reduced. These studies establish that the KCNJ10 K+ channel provides the molecular mechanism for generation of the endocochlear potential in concert with other transport pathways that establish the [K+] difference across the channel. KCNJ10 is also a limiting pathway for K+ secretion.

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