Phenotype Image Detail

Image

Caption

Cochlear histopathology of the Kcnq1^tm1Apf/Kcnq1^tm1Apf mouse. A: Normal histology of unaffected Kcnq1^tm1Apf/Kcnq1⁺ mouse for comparison. B and C: Base (B) and apex (C) of affected Kcnq1^tm1Apf/Kcnq1^tm1Apf mouse, showing complete loss of hair cells and supporting cells from the organ of Corti, which is replaced by fibrosis between the tectorial membrane and the basilar membrane (open arrow). The cell density in the spiral ganglion (SG) is decreased in the base but normal in the apex. There is marked degeneration of the stria vascularis (SV) throughout the cochlea, with more dramatic loss seen in the basal and middle half-turns. Reissner's membrane is adherent to the spiral ligament and the tectorial membrane in the basal regions of the cochlea, resulting in the obliteration of the scala media (filled arrow in B). The reduction of the scala media volume is more severe in the base than in the apex. IHC, inner hair cells; OHC, outer hair cells; RM, Reissner's membrane; TM, tectorial membrane, TC, tunnel of Corti.

Copyright

This image is from Lee MP, J Clin Invest 2000 Dec;106(12):1447-55 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:66428

Associated Alleles

Symbol	Name
Kcnq1^tm1Apf	potassium voltage-gated channel, subfamily Q, member 1; targeted mutation 1, Andrew P Feinberg

Associated Genotypes

Allelic Composition	Genetic Background
Kcnq1^tm1Apf/Kcnq1^tm1Apf	involves: 129P2/OlaHsd * C57BL/6