title: Imaging in 28 children with cochlear nerve aplasia.
abstract: 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.
authors.affiliation: ENT Department, University of Verona, Verona, Italy. carner.marco@yahoo.it
authors.forename: Marco
authors.initials: M
authors.lastname: Carner
authors.forename: Liliana
authors.initials: L
authors.lastname: Colletti
authors.forename: Robert
authors.initials: R
authors.lastname: Shannon
authors.forename: Roberto
authors.initials: R
authors.lastname: Cerini
authors.forename: Marco
authors.initials: M
authors.lastname: Barillari
authors.forename: Roberto Pozzi
authors.initials: RP
authors.lastname: Mucelli
authors.forename: Vittorio
authors.initials: V
authors.lastname: Colletti
mesh_terms: 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