Phenotypes for Whrn MGI:3810337 MGI Mouse

• when placed on their backs, adult mice take several seconds to right themselves

head bobbing ( J:235390 )

circling ( J:235390 )

abnormal outer hair cell stereociliary bundle morphology ( J:235390 )

• at P21, OHC stereocilia bundles appear rounded (U- rather than W-shaped) and show irregular spacing between stereocilia rows relative to controls

short cochlear hair cell stereocilia ( J:132633 )

• hair cell stereocilia are shorter than in wild-type mice

short inner hair cell stereocilia ( J:235390 )

• at P21, IHC stereocilia are abnormally short

short vestibular hair cell stereocilia ( J:235390 )

• at P21, vestibular stereocilia are consistently shorter than in controls across all hair cells

• the length of tallest stereocilia in both the extrastriolar and striolar regions is reduced by ~80% relative to controls

absent cochlear microphonics ( J:235390 )

• at P13-P20, cochlear microphonics responses are below detection limits, within the noise floor

increased or absent threshold for auditory brainstem response ( J:235390 )

• at P20 and P98, mice fail to show any auditory brainstem response (ABR) to the highest sound stimulus used

abnormal auditory summating potential ( J:235390 )

• at P13-P20, summating potentials are detected in 27 of 28 mice studied, albeit at high stimulus intensities, suggesting that some hair cells can depolarize in response to sound

absent cochlear nerve compound action potential ( J:235390 )

• at P20, mice exhibit no compound action potentials (CAPs) even at the highest stimulus intensities used

deafness ( J:235390 )

• adult mice are profoundly deaf