Phenotypes for Pkhd1l1 Tg(Atoh1-cre)1Bfri MGI:6379251 MGI Mouse

abnormal outer hair cell stereociliary bundle morphology ( J:280240 )

• some outer hair cell bundles show slightly disarrayed or even missing stereocilia at P120 but this disorganization is not pronounced or systematic

• mice show reduced stereociliary surface coat at the tips of outer hair cell stereocilia

• however, hair cells show normal stereocilia bundle orientation in cochlea with no planar cell polarity defects and no major deficiency of the hair-cell mechanotrasnduction complex is seen

decreased outer hair cell stereocilia number ( J:280240 )

• some outer hair cell bundles show missing stereocilia at P120

abnormal tectorial membrane morphology ( J:280240 )

• the tectorial membrane surface appears not as smooth

increased or absent threshold for auditory brainstem response ( J:280240 )

• mice show elevated auditory brainstem response (ABR) thresholds, with reduced ABR peak 1 and increased latency of ABR peak 1 and width of ABR wave 1

abnormal distortion product otoacoustic emission ( J:280240 )

• mice show elevated thresholds in response to high-frequency tones (22.6 kHz, 32 kHz, and 45 kHz) as early as 3 weeks of age, indicating high-frequency hearing loss

• the high-frequency hearing loss persists and shows increased progression in older mice, resulting in threshold elevation of 16-32kHz at 6 and 12 weeks of age

• by 6 months, mice show elevated thresholds across all frequencies tested

impaired hearing ( J:280240 )

• mice show progressive hearing loss