Fig. 6.

Auditory evoked brainstem analysis in Ush1c mice treated with multiple doses 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 twice at P1 and 3 (P1–3, pink line, n = 9, 7, and 7 at 1, 3 and 6 months of age) or four times at P1, 3, 5, and 7 (P1–7, green line, n = 16, 13, and 12 at 1, 3, and 6 months of age); 216AA mice treated with ASO-C or untreated (red line, n = 27, 23, and 11 at 1, 3, and 6 months of age, respectively); 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 multiple doses 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 for additional comparisons in this dataset shown in Supplemental Table Comparisons 3 and 4. Total number of treatments (doses) is indicated. dB, decibels; SPL, sound pressure level; kHz, kilohertz; BBN, broad-band noise; GG/GA, Ush1c c.216GG/GA littermate controls, AA, Ush1c c.216AA