Aplasia and hypoplasia of the vestibulocochlear nerve: Diagnosis with MR imaging

PURPOSE: To introduce aplasia or hypoplasia of the vestibulocochlear nerve (VCN) as a possible cause of hearing loss and to identify the magnetic resonance (MR) imaging characteristics of this entity. MATERIALS AND METHODS: In seven patients with congenital deafness or unexplained sensorineural hearing loss, MR imaging enabled diagnosis of aplasia or hypoplasia of the VCN. Axial (0.7-mm) three-dimensional Fourier transformation-constructive interference in steady state (3DFT-CISS) images and parasagittal reconstruction images perpendicular on the course of the VCN were obtained. Twenty normal inner ears were also studied; their findings were compared with those of the patients. RESULTS: The facial nerve and inferior and superior vestibular and cochlear branches of the VCN were identified on the MR images in the 20 normal inner ears. Aplasia of the VCN was detected in two patients with normal labyrinths but with a severe stenosis of the internal auditory canal. A common VCN with absence of the cochlear branch was found bilaterally in two patients with a congenital malformation of the labyrinth. A common VCN with absence or hypoplasia of the cochlear branch was found in three patients with normal internal auditory canals and labyrinths. CONCLUSION: Submillimetric gradient-echo images (eg, 3DFT-CLSS) should always be used to exclude aplasia or hypoplasia of the cochlear branch of the VCN in all cochlear implant candidates and patients with congenital deafness. This entity, which can occur with or without associated labyrinthine malformation, should be confirmed in two planes.