title: Hearing restoration with auditory brainstem implant in three children with cochlear nerve aplasia.
abstract: OBJECTIVE
  To verify the possibility of auditory habilitation in children with aplasia and hypoplasia of the cochlear nerve by direct electrical stimulation of the cochlear nuclei with an auditory brainstem implant.
  
  
  METHODS
  Retrospective case review.
  
  
  METHODS
  Study conducted at the Ear, Nose, and Throat Department of the University of Verona, Italy.
  
  
  METHODS
  Three children, aged 4, 3, and 2 years, respectively, with severe bilateral cochlear malformations and cochlear nerve aplasia have received an auditory brainstem implant at this institution in the past 2 years.
  
  
  METHODS
  The classic retrosigmoid approach was used. Correct positioning of the electrodes was evaluated using electric auditory brainstem responses and neural response telemetry. Before the patients were discharged, high-resolution computed tomography with a bone algorithm reconstruction technique was performed to evaluate electrode placement. The auditory brainstem implant was activated 30 to 60 days after implantation.
  
  
  RESULTS
  No postoperative complications were observed. To date, 21, 18, and 8 electrodes, respectively, have been activated in the three children. The first patient, 12 months after activation, had achieved good environmental sound awareness, good speech detection, and some speech recognition. The second child, 8 months after activation, had achieved good environmental sound awareness and moderate speech detection. The third patient, 1 month after activation, had obtained good environmental sound awareness.
  
  
  CONCLUSIONS
  This study indicates that auditory brainstem implantation is technically feasible in children with cochlear nerve aplasia. The early results suggest the possibility of achieving auditory habilitation with auditory brainstem implantation in this population.
authors.affiliation: Ear, Nose, and Throat Department, University of Verona, Italy. entdept@libero.it
authors.forename: Vittorio
authors.initials: V
authors.lastname: Colletti
authors.forename: Marco
authors.initials: M
authors.lastname: Carner
authors.forename: Francesco
authors.initials: F
authors.lastname: Fiorino
authors.forename: Luca
authors.initials: L
authors.lastname: Sacchetto
authors.forename: Veronica
authors.initials: V
authors.lastname: Miorelli
authors.forename: Alessandro
authors.initials: A
authors.lastname: Orsi
authors.forename: Francesco
authors.initials: F
authors.lastname: Cilurzo
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pacini
mesh_terms: D000465:Algorithms; D038262:Auditory Brain Stem Implants; D001861:Bone Regeneration / Q000502:physiology; D002675:Child, Preschool; D003056:Cochlear Nerve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D003638:Deafness / Q000601:surgery*; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013506:Otologic Surgical Procedures / Q000379:methods; D019736:Prostheses and Implants; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed