title: Relationship between electrically evoked auditory brainstem response and auditory performance after cochlear implant in patients with auditory neuropathy spectrum disorder.
abstract: OBJECTIVE
  To analyze the pattern of electrically evoked auditory brainstem response (EABR) in auditory neuropathy spectrum disorder (ANSD) patients and to compare their performances with controls.
  
  
  METHODS
  Retrospective analysis.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  Eleven patients with ANSD and 9 control subjects with sensorineural hearing loss who did not have neural pathology.
  
  
  METHODS
  Diagnostic.
  
  
  METHODS
  Patients and control subjects each received a cochlear implant (CI) and underwent EABR. EABR threshold, wave V latency, and amplitude were measured as EABR parameters. The results of EABR were categorized as good response, variable response, or no response. Speech perception ability was assessed by the categories of auditory performance (CAP) score.
  
  
  RESULTS
  All controls responded to EABR, whereas 6 of the 11 ANSD patients did not respond to EABR. The EABR threshold of the ANSD patients was measured almost within the value of disease controls. However, the Wave V latency displayed variable lengths, and the amplitude showed a wider distribution compared with the value of the disease control. The EABR response group among the ANSD patients showed relatively good performance after CI. In contrast, the nonresponse group demonstrated variable outcomes, although all of them still benefited from CI.
  
  
  CONCLUSIONS
  The data suggested that all ANSD patients require CI and that EABR results can help establish realistic expectations about future performance. Even if electrical stimulation fails to generate sufficiently synchronized signal for eliciting EABR, CI provides at least partial, measurable auditory benefit in ANSD.
authors.affiliation: Department of Otorhinolaryngology, Inje University College of Medicine, Ilsan Paik Hospital, Goyang-si, Republic of Korea.
authors.forename: Ju Hyun
authors.initials: JH
authors.lastname: Jeon
authors.forename: Mi Ran
authors.initials: MR
authors.lastname: Bae
authors.forename: Mee Hyun
authors.initials: MH
authors.lastname: Song
authors.forename: Seung Hwan
authors.initials: SH
authors.lastname: Noh
authors.forename: Ki Hoon
authors.initials: KH
authors.lastname: Choi
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Choi
mesh_terms: D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003054:Cochlear Implants; D003055:Cochlear Microphonic Potentials / Q000502:physiology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006309:Hearing / Q000502:physiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology* / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D000160:Vestibulocochlear Nerve Diseases / Q000503:physiopathology* / Q000601:surgery*