Cochlear Implantation in Children with Auditory Neuropathy Spectrum Disorder

Abstract

This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.

Evaluation of Hearing Handicap in Adults with Auditory Neuropathy Spectrum Disorder

“…Thus, at present the management options for late-onset ANSD are limited, especially in a developing country like India that cannot afford costly CI and frequency modulation devices [14] . There are other studies that contradict the use of CIand show that it might be of limited usefulness [40,41] . In addition, there are very limited studies that have attempted to determine the usefulness of CI in adults with acquired ANSD.…”

Satisfaction of Children with Auditory Neuropathy and Cochlear Implant

“…Thus, some of these children's speech perception will be very poor, resulting in disappointing language development skills. On the other hand, most of these children will achieve satisfactory speech perception and development following CI probably because the electric stimulation through the CI overcomes the existing auditory dyssynchrony [38] .…”

Performance of Cochlear Implants in Pediatric Patients with Auditory Neuropathy Spectrum Disorder

“…Poor prognosis is related to central nervous diseases and multiple deficiencies. Here electrically-evoked intracochlear compound action potential testing may be helpful to select patients with AN as potential good candidates to receive a CI [34] .…”

“…Poor prognosis is related to central nervous diseases and multiple deficiencies. Here electrically-evoked intracochlear compound action potential testing may be helpful to select patients with AN as potential good candidates to receive a CI [34] .It should be noted that the measure of pure tone thresholds, the recognition rate of speech, and the speech perception test are objective measures of great importance in indicating and monitoring patients with CI, particularly with oralized patients (post-lingual). But these tests have limitations, especially in not oralized patients.…”