title: Systematic Review of Nontumor Pediatric Auditory Brainstem Implant Outcomes.
abstract: OBJECTIVE
  The auditory brainstem implant (ABI) was initially developed for patients with deafness as a result of neurofibromatosis type 2. ABI indications have recently extended to children with congenital deafness who are not cochlear implant candidates. Few multi-institutional outcome data exist. Herein, we aim to provide a systematic review of outcomes following implantation of the ABI in pediatric patients with nontumor diagnosis, with a focus on audiometric outcomes.
  
  
  METHODS
  PubMed, Embase, and Cochrane.
  
  
  METHODS
  A systematic review of literature was performed using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analyses) recommendations. Variables assessed included age at implantation, diagnosis, medical history, cochlear implant history, radiographic findings, ABI device implanted, surgical approach, complications, side effects, and auditory outcomes.
  
  
  RESULTS
  The initial search identified 304 articles; 21 met inclusion criteria for a total of 162 children. The majority of these patients had cochlear nerve aplasia (63.6%, 103 of 162). Cerebrospinal fluid leak occurred in up to 8.5% of cases. Audiometric outcomes improved over time. After 5 years, almost 50% of patients reached Categories of Auditory Performance scores >4; however, patients with nonauditory disabilities did not demonstrate a similar increase in scores.
  
  
  CONCLUSIONS
  ABI surgery is a reasonable option for the habilitation of deaf children who are not cochlear implant candidates. Although improvement in Categories of Auditory Performance scores was seen across studies, pediatric ABI users with nonauditory disabilities have inferior audiometric outcomes.
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Kimberley S
authors.initials: KS
authors.lastname: Noij
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Elliott D
authors.initials: ED
authors.lastname: Kozin
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Rosh
authors.initials: R
authors.lastname: Sethi
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Parth V
authors.initials: PV
authors.lastname: Shah
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Alyson B
authors.initials: AB
authors.lastname: Kaplan
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Audiology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Barbara
authors.initials: B
authors.lastname: Herrmann
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA.
authors.forename: Aaron
authors.initials: A
authors.lastname: Remenschneider
authors.affiliation: Department of Otology and Laryngology, Harvard Medical School, Boston, Massachusetts, USA Department of Otolaryngology-Head and Neck Surgery, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, USA Daniel_Lee@meei.harvard.edu.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Lee
mesh_terms: D038262:Auditory Brain Stem Implants; D002648:Child; D003638:Deafness / Q000209:etiology / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D016518:Neurofibromatosis 2 / Q000150:complications*; D013067:Speech Perception / Q000502:physiology*; D016896:Treatment Outcome
keywords: auditory brainstem implant; children; deafness; hearing loss; nontumor; pediatric; review; systematic review