title: Experiences from Auditory Brainstem Implantation (ABIs) in four paediatric patients.
abstract: BACKGROUND
  Indications for auditory brainstem implants (ABIs) have been widened from patients with neurofibromatosis type 2 (NF2) to paediatric patients with congenital cochlear malformations, cochlear nerve hypoplasia/aplasia, or cochlear ossification after meningitis. We present four ABI surgeries performed in children at Uppsala University Hospital in Sweden since 2009.
  
  
  METHODS
  Three children were implanted with implants from Cochlear Ltd. (Lane Cove, Australia) and one child with an implant from MedEl GMBH (Innsbruck, Austria). A boy with Goldenhar syndrome was implanted with a Cochlear Nucleus ABI24M at age 2 years (patient 1). Another boy with CHARGE syndrome was implanted with a Cochlear Nucleus ABI541 at age 2.5 years (patient 2). Another boy with post-ossification meningitis was implanted with a Cochlear Nucleus ABI24M at age 4 years (patient 3). A girl with cochlear aplasia was implanted with a MedEl Synchrony ABI at age 3 years (patient 4). In patients 1, 2, and 3, the trans-labyrinthine approach was used, and in patient 4 the retro-sigmoid approach was used.
  
  
  RESULTS
  Three of the four children benefited from their ABIs and use it full time. Two of the full time users had categories of auditory performance (CAP) score of 4 at their last follow up visit (6 and 2.5 years postoperative) which means they can discriminate consistently any combination of two of Ling's sounds. One child has not been fully evaluated yet, but is a full time user and had CAP 2 (responds to speech sounds) after 3 months of ABI use. No severe side or unpleasant stimulation effects have been observed so far. There was one case of immediate electrode migration and one case of implant device failure after 6.5 years.
  
  
  CONCLUSIONS
  ABI should be considered as an option in the rehabilitation of children with similar diagnoses.
authors: a Department of Surgical Sciences, Section of Otorhinolaryngology, and Head & Neck Surgery , Uppsala University , Uppsala , Sweden.
authors: Karin
authors: K
authors: Lundin
authors: a Department of Surgical Sciences, Section of Otorhinolaryngology, and Head & Neck Surgery , Uppsala University , Uppsala , Sweden.
authors: Fredrik
authors: F
authors: Stillesjö
authors: a Department of Surgical Sciences, Section of Otorhinolaryngology, and Head & Neck Surgery , Uppsala University , Uppsala , Sweden.
authors: Gunnar
authors: G
authors: Nyberg
authors: a Department of Surgical Sciences, Section of Otorhinolaryngology, and Head & Neck Surgery , Uppsala University , Uppsala , Sweden.
authors: Helge
authors: H
authors: Rask-Andersen
mesh_terms: D038263:Auditory Brain Stem Implantation / Q000379:methods*; D058747:CHARGE Syndrome / Q000601:surgery*; D002675:Child, Preschool; D015834:Cochlear Diseases / Q000601:surgery*; D005260:Female; D006053:Goldenhar Syndrome / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D008581:Meningitis / Q000150:complications; D010040:Otosclerosis / Q000209:etiology / Q000601:surgery*; D013548:Sweden; D016896:Treatment Outcome
keywords: ABI; Auditory Brainstem Implants; Non-tumour patients; Peadiatric patients; eABR