Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age

Abstract

Objective To determine the influence of the presence of auditory neuropathy spectrum disorder (ANSD) on speech, language, and psycho-social development of children at three years of age. Design A population-based, longitudinal study was performed on outcomes of children with hearing impairment (LOCHI) in Australia. The demographic characteristics of the children were described, and their developmental outcomes were evaluated at three years of age. Performance of children with ANSD was compared with that of children without ANSD in the LOCHI study. Study sample There were 47 children with ANSD in the study sample. Results Sixty-four percent of children with ANSD have hearing sensitivity loss ranging from mild to severe degree, and the remaining have profound hearing loss. At three years, 27 children used hearing aids, 19 used cochlear implants, and one child did not use any hearing device. Thirty percent of children have disabilities in addition to hearing loss. On average, there were no significant differences in performance level between children with and without ANSD. Also, the variability of scores was not significantly different between the two groups. Conclusions There was no significant difference in performance levels or variability between children with and without ANSD, both for children who use hearing aids, and children who use cochlear implants.

Clinical role of electrocochleography in children with auditory neuropathy spectrum disorder

“…Conversely, the association between premature birth and achievement of open-set speech perception was positive. Additional disabilities may affect speech perception outcomes of pediatric CI recipients regardless of etiology of hearing loss [8, 12, 18, 19, 46–49]. The present study indicates that prelingual deafness and CNS abnormalities had a negative association with the outcome.…”

Profound childhood hearing loss in a South Africa cohort: Risk profile, diagnosis and age of intervention

“…This increased incidence of additional disabilities is typically reported for children with ANSD [46].…”

“…Two separate Nigerian studies indicated a prevalence of 4.9% in a sample of children diagnosed with SNHL [45] and 13.5% in a cohort of children with severe-profound hearing loss [44]. Hyperbilirubinemia has been associated as the most commonly reported risk factor for ANSD, varying between 30 to 70% [38,[46][47][48]. The current study had 50% of ANSD cases presenting with hyperbilirubinemia as a risk factor similar to a recent report from South Africa in which half of the ANSD cases (n=5/10) also presented with hyperbilirubinemia [24].…”

Auditory neuropathy — neural and synaptic mechanisms

“…At first glance, solely amplifying sound is not expected to improve auditory signalling, whereas the strong electrical stimulus of a cochlear implant can synchronously activate SGNs even in cases of impaired auditory nerve function 133 . However, although the outcome of auditory neuropathy in children with cochlear implants can indeed be similar to those with hearing impairment owing to other aetiologies, it does not seem to be substantially better than that of auditory neuro pathy fitted with hearing aids 134,135 . Of course, it is quite possible that the patients with auditory neuro pathy who were fitted with cochlear implants were more severely affected and would have had a worse outcome with hearing aids.…”