Our aim was to assess microstructural alterations in the cerebrums of paediatric patients with congenital sensorineural hearing loss (SNHL) using diffusion kurtosis imaging (DKI). Seventy-two paediatric SNHL patients and 38 age-matched healthy volunteers were examined via DKI using a 3.0 T magnetic resonance (MR) imager. Fractional anisotropy (FA) and mean kurtosis (MK) values were computed for 12 cerebral regions in both the controls and the SNHL patients. Compared with patients below age 3, patients in the older age group were found to have more significant differences in MK than in FA, and these appeared in more major areas of the brain. In contrast, in 1- to 3-year-old children, a few major brain areas exhibited differences in FA, but none exhibited appreciable differences in MK. There were significant decreases in the FA or MK values (P < 0.05, all) in more areas of the brain in patients with lesions than in patients with normal-appearing brains. DKI offers comprehensive measurements for quantitative evaluation of age-related microstructural changes in both white and grey matter in SNHL patients. DKI scans of children with SNHL exhibiting significant decreases in MK might play an important role in evaluating the severity of developmental delay.