title: Outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss.
abstract: OBJECTIVE
  Auditory neuropathy (AN) is a heterogeneous clinical entity for which the optimal method of auditory rehabilitation has been a matter of some debate. Such patients often do not receive sufficient benefit from hearing aids. Previous studies have shown that select AN patients may benefit from cochlear implantation (CI), but reported outcomes are variable and likely are a reflection of the heterogeneous patient population included under the umbrella diagnosis of AN. This study compares CI outcomes in a subset of the pediatric AN population who do not have a confounding cognitive disorder with their cochlear hearing loss peers. Additionally, it examines the impact of a confounding cognitive or developmental disorder on CI outcomes within the AN population.
  
  
  METHODS
  Retrospective chart review.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  Twenty-six pediatric patients with AN who received a CI were the subjects of this study. Seventeen of these children had a diagnosis of AN alone, and their CI outcomes were compared with those of a similar group of children with cochlear hearing loss. The remaining 9 children had a diagnosis of AN in association with a confounding cognitive or developmental disorder, and their CI outcomes were compared with those of children with isolated AN.
  
  
  METHODS
  Cochlear implantation.
  
  
  METHODS
  All subjects were evaluated preoperatively and postoperatively with age-appropriate speech perception testing.
  
  
  RESULTS
  Children with a diagnosis of AN alone performed comparably to their peers with cochlear hearing loss. The presence of a confounding cognitive or developmental disorder within the AN population was correlated with significantly poorer CI outcomes as compared with those of children with isolated AN.
  
  
  CONCLUSIONS
  Children with a diagnosis of AN without associated cognitive or developmental disorders perform at a level comparable to other children requiring a CI. Children with a diagnosis of AN associated with other developmental anomalies derive some benefit from CI but are significantly more likely to continue to rely on nonoral/aural methods of communication after implantation.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of Michigan, Ann Arbor, Michigan 48109, USA. cbudenz@med.umich.edu
authors.forename: Cameron L
authors.initials: CL
authors.lastname: Budenz
authors.forename: Steven A
authors.initials: SA
authors.lastname: Telian
authors.forename: Caroline
authors.initials: C
authors.lastname: Arnedt
authors.forename: Kelly
authors.initials: K
authors.lastname: Starr
authors.forename: Henry Alexander
authors.initials: HA
authors.lastname: Arts
authors.forename: Hussam K
authors.initials: HK
authors.lastname: El-Kashlan
authors.forename: Terry A
authors.initials: TA
authors.lastname: Zwolan
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D005260:Female; D006313:Hearing Loss, Central / Q000503:physiopathology / Q000601:surgery*; D006319:Hearing Loss, Sensorineural / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007804:Language Development; D008297:Male; D012189:Retrospective Studies; D013067:Speech Perception / Q000502:physiology*; D016896:Treatment Outcome