Imaging in 28 children with cochlear nerve aplasia

Abstract

Suspected congenital anomalies were confirmed by CT and MRI in all 28 children: 16 with associated labyrinthine malformations; 1 with outer, middle, and inner ear malformations; and 2 with associated monolateral facial nerve aplasia.

The vestibulocochlear nerve: aplasia and hypoplasia in combination with inner ear malformations

“…Contrary to our findings, Carner et al [15] reported on five cases of cochlear nerve aplasia in combination with an IPT2. While this might be due to different classification, this does not seem very plausible as an IPT 2 is a malformation welldefined by Mondini [16] and Sennaroglu et al [17,18].…”

Brain Stem and Inner Ear Abnormalities in Children with Auditory Neuropathy Spectrum Disorder and Cochlear Nerve Deficiency

“…The reported prevalence of CND in children with ANSD is between 18% and 28% [10][11][12] and is higher than that in CND described in children with SNHL not distinguished as ANSD (between 6% and 16.1%). [13][14][15][16] Several studies have suggested that inner ear and brain abnormalities are common among patients with CND, 13,14,[16][17][18][19][20] but only 1 of these studies has looked specifically at patients with ANSD. In that study, substantially more patients with CND had concomitant inner ear anomalies than patients with normalappearing cochlear nerves.…”

Management of Children with Severe, Severe-profound, and Profound Sensorineural Hearing Loss

“…24 One shortcoming of HRCT as a single imaging modality is that it cannot identify CND in the setting of a normal caliber internal auditory canal. 6,7,[11][12][13][29][30][31] Both MRI and HRCT are important in assessing cochlear patency in children suffering from hearing loss secondary to meningitis (Fig. 4).…”