Figure 2.

Vestibular sensory evoked potential (VsEP) waveforms for individual representative treated and untreated Usher mice. Response tracings for each mouse are represented by two separate VsEP recordings that are superposed to show replicability of response components. There are four of such response pairs shown for example in group I representing four individual heterozygote mice. Response trace pairs are shown for representative animals for all groups I through VI. Treatments of ASO-29 and ASO-C were 300 mg/kg doses injected intraperitoneally on days indicated. The Ush1c heterozygotes (216GA, group I) had robust VsEPs. Response peaks p1 and n1 are marked with a vertical line in the first pair of response waveforms of treatment group I. Ush1c 216AA mice not receiving treatment (group II) and those receiving ASO-C (group III) lacked discernable normal vestibular responses. Responses were present in Ush1c homozygotes 216AA receiving ASO-29 at P4 or P5 (P4/5, group IV). Robust responses were present in 216AA mice treated ASO-29 at P1 (group V). Responses were poor or absent in 216AA mice treated with ASO-29 at P15 (group VI). Mean values for threshold, latency and amplitudes are given in Table 1 for each group. Stimulus level for all responses was +6 dB re:1g/ms. Scale bar indicates amplitude and time. The stimulus is initiated at time = 0 at the beginning (left) of response traces. Although not shown, vestibular responses in 216AA mice treated with multiple doses of ASO-29 were comparable to those shown for groups I and V.