Axial Mesodermal Dysplasia Complex with a Unique Abnormal Course of Vestibulocochlear Nerve

Abstract

Axial mesodermal dysplasia complex (AMDC) is a combination of multiple congenital malformations arising due to the mesodermal cell migration, neural tube fusion, and rhombencephalon segmentation. Here, we present the imaging findings of a 15-year-old boy with AMDC who has bilateral inner ear malformations associated with a vestibulocochlear nerve extending to Meckel cave, cystic lesion in prepontine cisterna, cervical vertebral segmentation anomalies, and maxillar bone anomalies.