Fig. 2

CIB2 deficiency leads to profound sensorineural hearing loss and degeneration of the organ of Corti. a, b ABR thresholds to broadband clicks (a) and tone-pips with frequencies of 8 kHz, 16 kHz, and 32 kHz (b) in wild-type (open bar), Cib2 ^tm1a/+, Cib2 ^F91S/+ (black), Cib2 ^tm1a/tm1a (red), and Cib2 ^F91S/F91S (green) mice at P16. Number of animals in each group is shown in parentheses. The same animals were tested with clicks and tone pips. c DPOAEs of control (black; n = 13), Cib2 ^tm1a/tm1a (red; n = 10), and Cib2 ^F91S/F91S (green; n = 5) mice at P16-P18. Noise floor is shown in grey. All data in a–c are shown as Mean ± SEM (***p < 0.001). d SEM images of the organ of Corti at the apical, medial and basal turns of the cochlea in the control, and Cib2 ^tm1b/tm1b mice at P14, P21 and P110. Scale bar: 10 μm