Although conventional structural MRI provides vital information in the evaluation of congenital sensorineural hearing loss (SNHL), it is relatively insensitive to white matter microstructure. Our objective was to evaluate possible changes in microstructure of the auditory pathway in children with congenital sensorineural hearing loss (SNHL), and the possible distinction between good and poor outcome of cochlear implantation (CI) patients by using diffusion tensor imaging (DTI). Twenty-four patients with congenital SNHL and 20 healthy controls underwent conventional MRI and DTI examination using a 1.5T MR scanner. The DTI metrics of fractional anisotropy (FA) and mean diffusivity (MD) of six regions of interest (ROIs) positioned along the auditory pathway—the trapezoid body, superior olivary nucleus, inferior colliculus, medial geniculate body, auditory radiation and white matter of Heschl's gyrus—was measured in all subjects. Among the 24 patients, 8 patients with a categorie of auditory performance (CAP) score over 6 were classified into the good outcome group, and 16 patients with a CAP score below 6 were classified into the poor outcome group. A significant decrease was observed in FA values while MD values remained unchanged at the six ROIs of SNHL patients compared with healthy controls. Compared to good outcome subjects, poor outcome subjects displayed decreased FA values at all of the ROIs. No changes were observed in MD values. Correlation analyses only revealed strong correlations between FA values and CAP scores, and strong correlations between CAP scores and age at implant were also found. No correlations of FA values with age at implant were observed. Our results show that preoperative DTI can be used to evaluate microstructural alterations in the auditory pathway that are not detectable by conventional MR imaging, and may play an important role in evaluating the outcome of CI. Early cochlear implantation might be more effectively to restore hearing in SNHL patients.

BACKGROUND AND PURPOSE:Cochlear nerve deficiency (CND), including aplasia and hypoplasia, is a rare cause of congenital hearing loss. The purpose of this study was to evaluate the neuronal integrity of the subcortical auditory pathway in subjects with CND by diffusion tensor MR imaging (DTI).

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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.

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