Male mice (WT, control diet, n = 18; R6/2, control diet, n = 15; and R6/2 mice, 2% creatine [Cr], n = 11) were fed the indicated diet from the age of 4 weeks. The ABR thresholds upon application of click stimuli (A) or tone-burst stimuli (B) to the indicated mice at the age of 10.5 weeks are shown. (C) Total lysates were harvested from cochlea at the age of 10.5 weeks. Expression of the CKB protein was determined using Western blot analyses and normalized to that of actin as an internal control. Quantitative data are shown in the right panel. Immunostaining of the organ of Corti (D) and spiral ganglion (E) of WT and R6/2 mice (10.5 weeks of age) was conducted to determine the expression of SDH-A (green) and NIIs (red). Scale bars (D and E): 10 μm. Quantitative analyses are shown in the right panels. Data are presented as the mean ± SEM. **P < 0.01 and ***P < 0.001.