title: Pediatric Auditory Brainstem Implant Surgery: A New Option for Auditory Habilitation in Congenital Deafness?
abstract: BACKGROUND
  The auditory brainstem implant (ABI) is a neuroprosthetic device that provides sound sensations to individuals with profound hearing loss who are not candidates for a cochlear implant (CI) because of anatomic constraints. Herein we describe the ABI for family physicians.
  
  
  METHODS
  PubMed was searched to identify articles relevant to the ABI, as well as articles that contain outcomes data for pediatric patients (age <18 years) who have undergone ABI surgery.
  
  
  RESULTS
  The ABI was originally developed for patients with neurofibromatosis type 2 (NF2) who become deaf from bilateral vestibular schwannomas. Over the past decade, indications for an ABI have expanded to adult patients without tumors (without NF2) who cannot receive a CI and children with no cochlea or cochlear nerve. Outcomes among NF2 ABI users are modest compared to cochlear implant patients, but recent studies from Europe suggest that some non-tumor adult and pediatric ABI users achieve speech perception.
  
  
  CONCLUSIONS
  The ABI is a reasonable surgical option for children with profound hearing loss due to severe cochlear or cochlear nerve deformities. Continued prospective data collection from several clinical trials in the U.S. will provide greater understanding on long term outcomes that focus on speech intelligibility.
authors.affiliation: From the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
authors.forename: Parth V
authors.initials: PV
authors.lastname: Shah
authors.affiliation: From the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA. elliott_kozin@meei.harvard.edu.
authors.forename: Elliott D
authors.initials: ED
authors.lastname: Kozin
authors.affiliation: From the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
authors.forename: Alyson B
authors.initials: AB
authors.lastname: Kaplan
authors.affiliation: From the Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D038262:Auditory Brain Stem Implants / Q000009:adverse effects / Q000706:statistics & numerical data*; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000002:abnormalities; D003056:Cochlear Nerve / Q000002:abnormalities; D015331:Cohort Studies; D000075202:Contraindications; D003638:Deafness / Q000151:congenital* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D016518:Neurofibromatosis 2 / Q000601:surgery*; D010821:Physicians, Family / Q000193:education; D013067:Speech Perception; D016896:Treatment Outcome
keywords: Auditory Brain Stem Implants; Cochlear Implants; Cochlear Nucleus; Deafness; Hearing Loss; Pediatrics