Evaluation of Pediatric Sensorineural Hearing Loss With Magnetic Resonance Imaging

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.…”

Cochlear Implantation in Patient with Cochlear Nerve Deficiency: A Case Report

“…But cochlear nerve agenesis or hypoplasia is challenging situations for the patients and their physicians. McClay et al [3] reported that cochleovestibular nerve anomalies were seen in 18% of children with sensorineural hearing loss. To be aware that situation, neuroimaging of the all patients should examined carefully and all kind of audiological tests including otoacoustic emissions, brainstem response should performed to the patients with sensorineural hearing loss.…”

“…Magnetic resonance imaging (MRI) is able to demonstrate the vestibulocochlear nerve and facial nerve in the internal acoustic canal [3] . Also computed tomography can be helpful to determination of the deficiency of the cochlear nerve [4,5] .…”

Pediatric Sensorineural Hearing Loss, Part 1: Practical Aspects for Neuroradiologists

“…18,19 CND CND refers to the absence or reduction in caliber of the cochlear nerve and is observed in 12%-18% of ears affected with SNHL. 10,20 In pediatric patients, CND is usually congenital, but it can occasionally develop subsequent to birth due to atrophy of the nerve in patients who previously demonstrated normal hearing in the affected ear. 21 Because cochlear implants are generally contraindicated in CND, 22,23 it is important to identify this condition in children being considered for implantation.…”

“…Most children with congenital SNHL show normal inner ear morphology on CT and MR imaging, with congenital inner ear anomalies reported in only 20%-30%. 10,20,30 This is because hearing loss is frequently due to isolated abnormalities on a cellular or microscopic level, which are not resolvable by current imaging techniques and which do not overtly affect the appearance of the bony otic capsule or membranous inner ear.…”