Cochlear implantation in auditory neuropathy

Laryngoscope. 1999 Feb;109(2 Pt 1):181-5. doi: 10.1097/00005537-199902000-00002.


Objective: Auditory neuropathy is a recently described clinical entity characterized by sensorineural hearing loss in which the auditory evoked potential (ABR) is absent but otoacoustic emissions are present. This suggests a central locus for the associated hearing loss. In this study the results observed in a child with auditory neuropathy who received a cochlear implant are presented and compared with those of a matched group of children who were recipients of implants.

Methods: A single-subject, repeated-measures design, evaluating closed-set and open-set word recognition abilities was used to assess the subject and a control group of matched children with implants who had also experienced a progressive sensorineural hearing loss.

Results: The subject demonstrated improvements in vowel recognition (82% correct) by 1 year after implantation, which were only slightly lower than the control group. Consonant recognition and open-set word recognition scores were significantly lower.

Conclusion: Caution should be exercised when considering cochlear implantation in children with auditory neuropathy. As with conventional hearing aids, less than optimal results may be seen.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Audiometry, Pure-Tone / methods
  • Child, Preschool
  • Cochlear Diseases / complications*
  • Cochlear Diseases / physiopathology*
  • Cochlear Implantation*
  • Disease Progression
  • Evoked Potentials, Auditory, Brain Stem / physiology
  • Follow-Up Studies
  • Friedreich Ataxia / complications
  • Friedreich Ataxia / diagnosis
  • Hearing Loss, Sensorineural* / etiology
  • Hearing Loss, Sensorineural* / physiopathology
  • Hearing Loss, Sensorineural* / surgery
  • Humans
  • Male
  • Phonetics
  • Severity of Illness Index
  • Speech Perception / physiology