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. 2022 Mar 7;14(4):e14817. doi: 10.15252/emmm.202114817

Figure EV2. Impaired auditory system in USH1C pigs.

Figure EV2

  • A
    ABR measurements were conducted on two WT pigs to determine the threshold for sufficient detection of response peak V to click stimuli.
  • B
    Scanning electron microscopy of the cochlear sensory epithelium in 3‐week old WT and USH1C pigs reveals alterations in the hair bundle arrangements of outer (OHC) and inner cochlear hair cells (IHC) (arrows). USH1C OHC lack stereocilia rows in the hair cell bundles, indicated by colored lines at higher magnification. Scale bars, upper panel 10 µm; lower panel: 5 µm.
  • C, D
    (C) and (D) show exemplary response curves for determining hearing thresholds (dB SPL) by click ABR in an 8‐week‐old USH1C animal (C) as well as 2‐year‐old USH1C and WT pigs (D).
  • E, F
    Frequency‐specific ABR of animals at an age of 3 months (E) and 6 months (F) indicate an increased threshold for heterozygous USH1C+/‐ pigs, compared to WT littermate controls. Data points are presented as mean value (MV) ± SD. Statistical examination was carried out by t‐test for each frequency, a 2‐side ANOVA test was performed over all frequencies. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001.