Bass abrupt form audiogram and stria vascularis deafness.
نویسندگان
چکیده
منابع مشابه
Stria vascularis as source of endocochlear potential.
IT IS WELL KNOWN (1, 2, 12, 6) that, when a recording microelectrode is pushed into the endolympatic space of the cochlea of a guinea pig or a cat, the potential at the tip of the electrode rises 70-90 mV. above the potential level in the perilymphatic space. Although the ionic composition of the endolymph is unique among various body fluids because of its high potassium content (lo), the DC po...
متن کاملClaudins in stria vascularis marginal cells
Background: In epithelia, tight junction proteins form paracellular permeability barriers between the extracellular compartments. Claudins (20-28 kDa proteins) are essential components of the junctional strands. Knowledge of claudins in the inner ear cochlea is fragmentary: Hair cells express claudin-14, and its absence causes deafness. Marginal cells of the stria vascularis separate K-rich end...
متن کاملEpithelial Cell Stretching and Luminal Acidification Lead to a Retarded Development of Stria Vascularis and Deafness in Mice Lacking Pendrin
Loss-of-function mutations of SLC26A4/pendrin are among the most prevalent causes of deafness. Deafness and vestibular dysfunction in the corresponding mouse model, Slc26a4(-/-), are associated with an enlargement and acidification of the membranous labyrinth. Here we relate the onset of expression of the HCO(3) (-) transporter pendrin to the luminal pH and to enlargement-associated epithelial ...
متن کاملDeafness in Claudin 11-null mice reveals the critical contribution of basal cell tight junctions to stria vascularis function.
Generation of a strong electrical potential in the cochlea is uniquely mammalian and may reflect recent evolutionary advances in cellular voltage-dependent amplifiers. This endocochlear potential is hypothesized to dramatically improve hearing sensitivity, a concept that is difficult to explore experimentally, because manipulating cochlear function frequently causes rapid degenerative changes e...
متن کاملFree radical stress-mediated loss of Kcnj10 protein expression in stria vascularis contributes to deafness in Pendred syndrome mouse model.
Pendred syndrome is due to loss-of-function mutations of Slc26a4, which codes for the HCO(3)(-) transporter pendrin. Loss of pendrin causes deafness via a loss of the K(+) channel Kcnj10 in stria vascularis and consequent loss of the endocochlear potential. Pendrin and Kcnj10 are expressed in different cell types. Here, we report that free radical stress provides a link between the loss of Kcnj...
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ژورنال
عنوان ژورنال: AUDIOLOGY JAPAN
سال: 1997
ISSN: 1883-7301,0303-8106
DOI: 10.4295/audiology.40.519