Diffusion Tensor Imaging of Auditory Pathway in Patients With Crigler-Najjar Syndrome Type I: Correlation With
Ahmed Abdel Khalek Abdel Razek1, Mohamed Ezz El Regal2, Mortada El-Shabrawi3
1Department of Diagnostic Radiology, Mansoura Faculty of Medicine, Mansoura, Egypt.
Journal of Child Neurology
|December 28, 2021
Summary
Diffusion tensor imaging reveals microstructural changes in the auditory pathway of Crigler Najjar syndrome type I patients. These changes correlate with auditory brainstem response, offering insights into neurological impact.
Area of Science:
- Neuroimaging
- Auditory Neuroscience
- Genetic Disorders
Background:
- Crigler Najjar syndrome type I is a rare genetic disorder.
- Bilirubin encephalopathy can affect auditory pathways.
Purpose of the Study:
- To assess diffusion tensor imaging (DTI) of the auditory pathway in Crigler Najjar syndrome type I.
- To correlate DTI findings with auditory brainstem response (ABR).
Main Methods:
- Prospective study of 12 Crigler Najjar syndrome type I patients and 10 controls.
- DTI of the brain was performed.
- Mean diffusivity and fractional anisotropy were measured and correlated with ABR.
Main Results:
- Patients showed significantly higher mean diffusivity and lower fractional anisotropy in auditory pathway regions compared to controls.
- Elevated bilirubin levels correlated with reduced fractional anisotropy.
- DTI metrics correlated with ABR wave latencies.
Conclusions:
- DTI can detect microstructural alterations in the auditory pathway in Crigler Najjar syndrome type I.
- DTI findings correlate with ABR results, indicating functional impact.
