Inner hair cell and neuron degeneration contribute to hearing loss in a DFNA2-like mouse model

Abstract

HIGHLIGHTSO_LIKCNQ4 knock-out mouse shows hair cells and spiral ganglion neuron degeneration.\nC_LIO_LIInner hair cells and spiral ganglion neuron loss begin 30 weeks later than outer hair cells in Kcnq4-/- mice.\nC_LIO_LIInner hair cell loss kinetic is faster than that of outer hair cells in cochlear basal turn in Kcnq4-/-.\nC_LIO_LIOuter hair cells from Kcnq4-/- mice degenerate slower in apical than in basal turn.\nC_LIO_LIKcnq4 knock-out allele expressed in C3H/HeJ strain reproduces the two phases of DFNA2 hearing loss.\nC_LI\n\nGRAPHICAL ABSTRACT\n\nO_FIG O_LINKSMALLFIG WIDTH=200 HEIGHT=78 SRC=\"FIGDIR/small/469676_ufig1.gif\" ALT=\"Figure 1\">\nView larger version (31K):\norg.highwire.dtl.DTLVardef@1bdff20org.highwire.dtl.DTLVardef@b8cc51org.highwire.dtl.DTLVardef@6deeb9org.highwire.dtl.DTLVardef@153f381_HPS_FORMAT_FIGEXP M_FIG C_FIG