Value of computed tomography in the evaluation of children with cochlear nerve deficiency

To assess the predictive value of high-resolution computed tomography (HRCT) in the evaluation of children with cochlear nerve deficiency (CND).

Retrospective review of medical records.

: Tertiary referral center, hospital setting.

Nineteen children (31 ears) with CND.

Magnetic resonance imaging (MRI), HRCT, and audiologic evaluation.

Comparisons of the morphology of the internal auditory canal (IAC), the bony cochlear nerve canal (BCNC) as seen on HRCT, and audiologic data.

Of 12 ears with MRI evidence of an absent cochlear nerve (CN) and a normal-size IAC, all had a patent BCNC as revealed by HRCT. Four of these ears failed auditory stimulation after cochlear implantation, confirming clinically significant CND. Of 15 ears with a narrow IAC and a single nerve visible on MRI, 2 (13.3%) had a normal-size BCNC, 4 (26.7%) were narrow, and 9 (60.0%) were absent. One ear with a narrow IAC, normal BCNC, and a single nerve as revealed by MRI has benefited from cochlear implantation.

Using BCNC patency, as revealed by HRCT, as a means of identifying CND would miss all cases of absent CNs in the setting of a normal-size IAC. Thus, MRI should be the primary modality for imaging children with severe to profound sensorineural hearing loss. When MRI demonstrates a single nerve within a narrow IAC, the addition of HRCT can further identify more than half of these cases as involving absent CNs because of an absent BCNC. In a subset of patients, CN status remains indeterminate.