title: Usefulness of measuring electrically evoked auditory brainstem responses in children with inner ear malformations during cochlear implantation.
abstract: CONCLUSIONS
  EABR is a reliable and effective way of objectively confirming device function and implant-responsiveness of the peripheral auditory neurons up to the level of the brainstem in cases of inner ear malformation.
  
  
  OBJECTIVE
  To investigate the usefulness of measuring the intra-operative electrically evoked compound action potential (ECAP) and electrically evoked auditory brainstem response (EABR) in patients with and without congenital inner ear anomalies during cochlear implantation.
  
  
  METHODS
  Thirty-eight consecutive children (40 ears) aged 5 or younger with congenital profound hearing loss. Twenty-four (25 ears) lacked congenital inner ear anomalies. The 14 patients (15 ears) with a malformation had common cavities (four ears), incomplete partition type I (three ears), cochlea hypoplasia type III (three ears), enlarged vestibular aqueduct (four ears), and cochlear nerve canal stenosis (one ear). Main outcome measures are ECAP and EABR responses.
  
  
  RESULTS
  Of the 25 ears lacking any malformation, 21, three, and one showed 'Good', 'Variable', and 'No' ECAP responses, respectively, and 24 and one showed 'Good' and 'Variable' intra-cochlear responses, respectively. Of the 15 ears with a malformation, two showed 'Good' ECAP responses, nine had 'Variable' ECAP responses, and four showed 'No' ECAP responses. Moreover, five showed 'Good' EABR responses and 10 showed 'Variable' EABR responses.
authors.affiliation: National Institute of Sensory Organs, National Tokyo Medical Center , Tokyo , Japan.
authors.forename: Shujiro B
authors.initials: SB
authors.lastname: Minami
authors.forename: Hideki
authors.initials: H
authors.lastname: Takegoshi
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Shinjo
authors.forename: Chieko
authors.initials: C
authors.lastname: Enomoto
authors.forename: Kimitaka
authors.initials: K
authors.lastname: Kaga
mesh_terms: D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D019929:Cochlear Implantation / Q000379:methods*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000151:congenital / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D016343:Monitoring, Intraoperative / Q000379:methods*; D015203:Reproducibility of Results; D012189:Retrospective Studies
keywords: EABR; ECAP; cochlear nerve canal stenosis; common cavity; enlarged vestibular aqueduct; incomplete partition