Imaging in 28 children with cochlear nerve aplasia.
CONCLUSIONS
Preoperative CT and MRI assessment of children with severe or profound sensorineural hearing loss (SNHL) is critical for determining implant candidacy.


OBJECTIVE
There are a significant number of children who do not show any auditory development with a cochlear implant (CI), possibly due to cochlear nerve (CN) aplasia/hypoplasia. Regardless of the suspected etiology, if a CI is not providing auditory development the clinician should carefully evaluate the possibility of a CN malformation and re-evaluate the child with detailed neuroimaging studies. If the imaging evaluation shows severe cochlear malformation or CN aplasia there is some developmental urgency to consider auditory brainstem implant (ABI) surgery.


METHODS
Twenty-eight children affected by congenital SNHL were examined by CT and MRI. Evaluation of the cerebellopontine angle (CPA), internal auditory canal (IAC), cranial nerves, and membranous labyrinth was performed. Six children had been previously fitted elsewhere with a CI with no sound detection.


RESULTS
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.
Carner
Marco
M
ENT Department, University of Verona, Verona, Italy. carner.marco@yahoo.it
Colletti
Liliana
L
Shannon
Robert
R
Cerini
Roberto
R
Barillari
Marco
M
Mucelli
Roberto Pozzi
RP
Colletti
Vittorio
V
D000293:Adolescent; D038263:Auditory Brain Stem Implantation; D038262:Auditory Brain Stem Implants; D002648:Child; D002675:Child, Preschool; D003056:Cochlear Nerve / Q000002:abnormalities* / Q000000981:diagnostic imaging; D004594:Electrophysiology; D016057:Evoked Potentials, Auditory, Brain Stem; D006319:Hearing Loss, Sensorineural / Q000000981:diagnostic imaging* / Q000601:surgery; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome