Fig. 2

sAAV-Tmc1 restores ABRs and DPOAEs in Tmc1^∆/∆ mice. a Families of ABR waveforms recorded at P28-P30 from uninjected Tmc1^∆/∆ mouse (left) and from Tmc1^∆/∆ mice injected with sAAV-Tmc1 or AAV2/1-Tmc1 and one WT control, as indicated above. ABRs were recorded using 11.3-kHz tone bursts at sound pressure levels increasing by 5-dB until peak amplitudes reached 0.55 μV. Thresholds determined by the presence of Peak 1 and is indicated by colored traces. Scale bar applies to all families. b ABR thresholds plotted as a function of stimulus frequency for eleven Tmc1^∆/∆ mice injected with sAAV-Tmc1 tested at P28-P30 (gray traces). Best (green), and median (blue) recovery are indicated. Black lines show mean ± S.D. (n = 6 mice) for WT and Tmc1^∆/∆ uninjected mice as indicated. Red line shows mean ± S.D. (n = 6 mice) for Tmc1^∆/∆ injected with AAV2/1-Tmc1. c Peak 1 amplitudes measured from 11.3-kHz ABR waveforms, as shown in a, for eleven Tmc1^∆/∆ mice injected with sAAV-Tmc1. Colors correspond to conditions indicated in b. d Peak 1 latencies measured from 11.3-kHz ABR waveforms, as shown in a for eleven Tmc1^∆/∆ mice injected with sAAV-Tmc1. Colors correspond to conditions indicated in b. e DPOAE thresholds plotted as a function of stimulus frequency for eleven Tmc1^∆/∆ mice injected with sAAV-Tmc1 tested at P28-P30. Colors correspond to conditions indicated in b. f Mean ± S.D. (n = number of mice) ABR thresholds plotted as a function of stimulus frequency for Tmc1^∆/∆ mice injected with sAAV-Tmc1 at P1 tested at 4, 6, and 12 weeks. g Mean ± S.D. DPOAE thresholds plotted as a function of stimulus frequency for the same Tmc1^∆/∆ mice shown in panel G injected with sAAV-Tmc1