Fig. 5.

Auditory evoked brainstem analysis in Ush1c mice treated with a single dose of ASOs. Average ABR thresholds (dB SPL) at 1 month (a), 3 months (b), and 6 months (c) of age to pure tones ranging from 8 to 32 kHz or BBN from 216AA mice treated with 300 mg/kg of ASO-29 one time at P1 (purple line, n = 6, 6, and 6 at 1, 3, and 6 months of age, respectively), P5 (blue line, n = 9, 9, and 9 at 1, 3, and 6 months of age, respectively) or P7 (orange line, n = 7, 7 and 6 at 1, 3, and 6 months of age, respectively); 216AA mice treated with ASO-C or untreated (red line, n = 27, 23 and 11 at 1, 3 and 6 months of age); and 216GG/GA mice treated by IP injection with ASO-29, ASO-C, and untreated (black line, n = 31, 28, and 24 at 1, 3, and 6 months of age, respectively). ABR thresholds are reduced at some frequencies at all ages in 216AA mice treated with a single dose of ASOs in the first week of life. 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 Comparisons 3 and 4. dB, decibels; SPL, sound pressure level; kHz, kilohertz; BBN, broad-band noise; GG/GA, Ush1c c.216GG/GA littermate controls, AA, Ush1c c.216AA