Facial canal bifurcation with inner ear anomalies in a case of Klippel-Feil syndrome: A case report

We present a case of Klippel-Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel-Feil syndrome.