Cochlear Implantation in Auditory Neuropathy

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.