All Phenotypes Clrn1<tm1.1Kuna> MGI Mouse

hearing/vestibular/ear phenotype ( J:150215 , J:186316 )

N	• between P21 and P30, vestibular hair cell morphology is normal (J:150215) • hair cell ribbon synapses are normal (J:186316)

absent cochlear inner hair cells ( J:150215 )

• by P30, outer and inner hair cells are missing unlike in wild-type mice

absent cochlear outer hair cells ( J:150215 )

• by P21, outer hair cells are lost from the cochlea unlike wild-type mice

• by P30 outer and inner hair cells are missing unlike in wild-type mice

abnormal cochlear hair cell stereociliary bundle morphology ( J:186316 )

• disrupted integrity that is more severe than in Clrn1^tm2.1Kuna homozygotes

• however, tip links are normal

abnormal inner hair cell stereociliary bundle morphology ( J:255408 )

• disrupted at age P2, P10, P21, P36 and P100

abnormal outer hair cell stereociliary bundle morphology ( J:150215 , J:255408 )

• outer hair cell stereociliary bundles are disorganized and circular clusters of abnormal stereocilia are observed (J:150215)

• there is little progression in the severity of stereocilia defects between P2 and P15 (J:150215)

• disrupted at age P2, P10, P21, P36 and P100 (J:255408)

abnormal orientation of outer hair cell stereociliary bundles ( J:150215 )

• at P2, P6, and P10

degeneration of organ of Corti supporting cells ( J:150215 )

• in the basal turn of the cochlea by P30

organ of Corti degeneration ( J:150215 )

• by P30, the organ of Corti collapses

abnormal cochlear hair cell physiology ( J:186316 )

• cochlear hair cells exhibit reduced FM1-43 loading compared with wild-type cells

• cochlear hair cells exhibit reduced amplitudes and sensitivity of transduction currents compared with wild-type cells

decreased cochlear microphonics ( J:186316 )

• at P18 at 2, 4 and 8 kHz

abnormal vestibular hair cell physiology ( J:186316 )

• vestibular hair cells exhibit reduced amplitudes and sensitivity of transduction currents compared with wild-type cells

abnormal auditory brainstem response waveform shape ( J:150215 )

• at P14 and P20, peak latencies are decreased compared to controls

• at P21, response peak latencies are prolonged compared to controls

increased or absent threshold for auditory brainstem response ( J:150215 )

• at P14 and P20, in brainstem auditory evoked potential thresholds are elevated compared to controls

• at P21, 30% mice exhibit an increase in brainstem auditory evoked potential threshold compared with wild-type mice

absent distortion product otoacoustic emissions ( J:150215 )

• at P21

impaired hearing ( J:150215 , J:255408 )

• mice exhibit progressive hearing loss (J:150215)

• at P30, hearing function is absent (J:150215)

• progressive hearing loss resulting in deafness by age P22 (J:255408)

deafness ( J:150215 )

• by P30

reduced linear vestibular evoked potential ( J:150215 )

• at P1, linear vestibular evoked potential response peak latencies are prolonged compared to in wild-type mice

• between P21 and P30, the threshold of vestibular evoked potential is higher in some mice than in wild-type mice

• between P21 and P30, mice exhibit abnormalities in linear vestibular evoked potential response peak latencies compared with wild-type mice

impaired balance ( J:150215 )

• between P21 and P90, mice are less stable in the water and tend to roll from one side to the other compared with wild-type mice

head bobbing ( J:150215 )

• between p21 to P40, mice exhibit mild and variable head bobbing unlike wild-type mice

vision/eye phenotype ( J:150215 )

N	• mice exhibit normal photoreceptor and neuron morphology and physiology