title: Speech perception and cortical event related potentials in children with auditory neuropathy.
abstract: OBJECTIVE
  1) To investigate the unaided and aided speech perception abilities of children with auditory neuropathy (AN) and to compare their performance to children with sensorineural hearing loss. 2) To establish whether cortical event related potentials (ERPs) could be recorded in children with AN, and to determine the relationship between the presence of these responses and speech perception.
  
  
  METHODS
  Unaided and aided speech perception assessments (PBK words), and cortical-ERP testing was carried out in a group of 18 children with AN. Data also were obtained from a cohort of age and hearing level matched children with sensorineural hearing loss.
  
  
  RESULTS
  The speech perception performance of the 15 children with AN able to complete a PBK-word assessment, fell into two distinct categories. The children either showed no open-set speech perception ability (7/15 cases), or performance levels similar to their sensorineural counterparts (8/15 cases). Approximately 50% of children with AN showed ERPs of normal latency, amplitude and morphology. In all cases, response presence (at normal latencies) was consistent with reasonable speech perception ability, and response absence was consistent with negligible speech perception.
  
  
  CONCLUSIONS
  In approximately 50% of children with auditory neuropathy, the provision of amplification results in significant open-set speech perception improvements. The results confirm the previously published reports that speech perception ability cannot be reliably estimated from the behavioral audiogram in children with AN. Obligatory ERP test results may offer a means of predicting perceptual skills in newly diagnosed youngsters as the presence of ERPs (with age-appropriate latency and morphology) was correlated with significant open set speech perception abilities and amplification benefit. The absence of the ERP in contrast, indicated profound hearing disability evidenced by profound hearing loss and/or extremely poor speech perception.
authors.affiliation: Department of Otolaryngology, School of Audiology, University of Melbourne, 172 Victoria Parade, East Melbourne, 3002 Victoria, Australia.
authors.forename: Gary
authors.initials: G
authors.lastname: Rance
authors.forename: Barbara
authors.initials: B
authors.lastname: Cone-Wesson
authors.forename: Julia
authors.initials: J
authors.lastname: Wunderlich
authors.forename: Richard
authors.initials: R
authors.lastname: Dowell
mesh_terms: D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002540:Cerebral Cortex / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D015331:Cohort Studies; D004569:Electroencephalography; D005071:Evoked Potentials / Q000502:physiology*; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000503:physiopathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D013067:Speech Perception; D013069:Speech Reception Threshold Test