Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Mansoura, Egypt.
Gastroenterology and Hepatology Unit, Mansoura Children Hospital, Mansoura Faculty of Medicine, Mansoura, Egypt.
J Child Neurol. 2022 Feb;37(2):119-126. doi: 10.1177/08830738211025865. Epub 2021 Dec 27.
To evaluate the role of diffusion tensor imaging of the auditory pathway in patients with Crigler Najjar syndrome type I and its relation to auditory brainstem response.
Prospective study was done including 12 patients with Crigler Najjar syndrome type I and 10 age- and sex-matched controls that underwent diffusion tensor imaging of brain. Mean diffusivity and fractional anisotropy at 4 regions of the brain and brainstem on each side were measured and correlated with the results of auditory brainstem response for patients.
There was significantly higher mean diffusivity of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls on both sides for all regions ( = .001). The fractional anisotropy of cochlear nucleus, superior olivary nucleus, inferior colliculus, and auditory cortex of patients versus controls was significantly lower, with values of, respectively, .001, .001, .003, and .001 on the right side and .001, .001, .003, and .001 on left side, respectively. Also, a negative correlation was found between the maximum bilirubin level and fractional anisotropy of the left superior olivary nucleus and inferior colliculus of both sides. A positive correlation was found between the mean diffusivity and auditory brainstem response wave latency of the right inferior colliculus and left cochlear nucleus. The fractional anisotropy and auditory brainstem response wave latency of the right superior olivary nucleus, left cochlear nucleus, and inferior colliculus of both sides were negatively correlated.
Diffusion tensor imaging can detect microstructural changes in the auditory pathway in Crigler Najjar syndrome type I that can be correlated with auditory brainstem response.
评估听觉通路弥散张量成像在Ⅰ型克里格勒-纳贾尔综合征患者中的作用及其与听脑干反应的关系。
对 12 例Ⅰ型克里格勒-纳贾尔综合征患者和 10 例年龄和性别匹配的对照组进行前瞻性研究，所有患者均接受脑弥散张量成像检查。测量双侧脑和脑干 4 个区域的平均弥散度和各向异性分数，并与患者听脑干反应结果相关。
与对照组相比，双侧各区域蜗神经核、上橄榄核、下丘和听皮层的平均弥散度均显著升高（均 P＜0.001）。患者蜗神经核、上橄榄核、下丘和听皮层的各向异性分数均显著降低，右侧分别为 0.001、0.001、0.003 和 0.001，左侧分别为 0.001、0.001、0.003 和 0.001。此外，还发现双侧左、右上橄榄核的最大胆红素水平与各向异性分数呈负相关，右、左侧下丘和左蜗神经核的平均弥散度与听脑干反应波潜伏期呈正相关，右、左侧上橄榄核、左蜗神经核和下丘的各向异性分数与听脑干反应波潜伏期呈负相关。
弥散张量成像可以检测Ⅰ型克里格勒-纳贾尔综合征患者听觉通路的微观结构变化，并与听脑干反应相关。
