title: Cochlear implantation versus hearing amplification in patients with auditory neuropathy spectrum disorder.
abstract: OBJECTIVE
  Patients with auditory neuropathy spectrum disorder (ANSD) exhibit altered neural synchrony in response to auditory stimuli. Cochlear implantation (CI) is thought to improve neural synchrony in response to auditory stimuli and improve speech perception relative to conventional hearing amplification (HA).
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  Tertiary otologic practice.
  
  
  METHODS
  Subjects included patients with ANSD treated at Vanderbilt University from 1999 to 2011. Sixteen patients underwent CI, and 10 received binaural HAs. Pretreatment performance was assessed through speech reception thresholds and parent questionnaire (Infant-Toddler Meaningful Auditory Integration Scale [IT-MAIS]). Posttreatment outcomes were assessed using IT-MAIS and closed-/open-set speech perception scores.
  
  
  RESULTS
  Two HA users underwent neuromaturation and were excluded from further analysis. For the remaining patients, median duration of device use was 48 months. All CI patients had a prior binaural HA trial with failure of auditory skills development. Median available pretreatment IT-MAIS score was 13 and 30 for CI and HA groups, respectively (rank sum test, P = .32). Posttreatment, 6 of 16 CI patients and 4 of 8 HA patients achieved open-set speech perception scores ≥ 60%. No differences between groups were found in posttreatment IT-MAIS scores (rank sum test, P = .11) or the percentage of patients achieving the above levels of open-set speech perception (Fisher exact test, P = .67).
  
  
  CONCLUSIONS
  A wide range of speech perception outcomes are observed in ANSD patients. In our ANSD population, patients who exhibited failure of auditory skills development with HAs were able to achieve comparable overall speech perception outcomes after CI relative to those who continued to make appropriate auditory progress with HAs alone.
authors: Department of Otolaryngology, Vanderbilt University Medical Center, Nashville, Tennessee 37232, USA. stanley.pelosi@vanderbilt.edu
authors: Stanley
authors: S
authors: Pelosi
authors: George
authors: G
authors: Wanna
authors: Cathrine
authors: C
authors: Hayes
authors: Linsey
authors: L
authors: Sunderhaus
authors: David S
authors: DS
authors: Haynes
authors: Marc L
authors: ML
authors: Bennett
authors: Robert F
authors: RF
authors: Labadie
authors: Alejandro
authors: A
authors: Rivas
mesh_terms: D019929:Cochlear Implantation; D005260:Female; D006310:Hearing Aids; D006313:Hearing Loss, Central / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013067:Speech Perception