Fig. 4.

Distortion product otoacoustic emissions analysis in Ush1c mice treated with ASOs. Average DPOAE thresholds (dB SPL) at 1 month (a), 3 months (b), and 6 months (c) of age to tone pairs ranging from 6363 to 29,378.5 kHz from 216AA mice treated with 300 mg/kg of ASO-29 one time at P1 (purple line, n = 8, 6, and 6 at 1, 3, and 6 months of age, respectively) or P5 (blue line, n = 5) or multiple times at P1–3 (pink line, n = 9, 8, and 7 at 1, 3, and 6 months, respectively) or P1–7 (green line, n = 17, 13, 12 at 1, 3 and 6 months of age, respectively); 216AA mice treated with ASO-C or untreated (red line, n = 15, 12, 7 at 1, 3 and 6 months of age, respectively); and 216 untreated GA mice (black line, n = 11, 11, 8 at 1, 3, and 6 months of age, respectively). DPOAE thresholds are reduced with some ASO treatments in 216AA mice. Total number of treatments (doses) is indicated. Error bars indicate SEM. For each frequency, asterisks indicate significant difference (*P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001, ANOVA with Tukey-Kramer post-test) from control mutant thresholds (red). P values, specific test, test value, and degrees of freedom for all comparisons in this dataset are shown in Supplemental Table Comparison 2. dB, decibels; SPL, sound pressure level; kHz, kilohertz; BBN, broad-band noise; GA, Ush1c c.216GA littermate controls, AA, Ush1c c.216AA