2010
DOI: 10.1179/146701010x12671177818588
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Longitudinal Outcomes of Early Implantation in Children with Auditory Neuropathy Spectrum Disorder (ANSD)

Abstract: Auditory Neuropathy Spectrum Disorder (ANSD) has been identified in 12 of the 75 children who received a cochlear implant at SCIC following identification through newborn infant screening. The longitudinal outcomes of 5 of these children who have thin or absent auditory nerves will be discussed in this paper with attention to the preoperative and postoperative management and factors impacting on their results. Implications for future management of children with ANSD will be addressed.

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Cited by 8 publications
(12 citation statements)
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“…Bilateral simultaneous CI usually make it impossible to check for any error in the early diagnosis of permanent hypoacusia, depriving the team involved in selecting candidates for CI of a helpful feedback. On the other hand, recording cortical auditory evoked potentials (CAEP) has recently proved a useful diagnostic tool when nerve and/or brainstem dysmaturity is suspected [28,29], so it may become possible to identify infants' hearing thresholds more reliably in the future.…”
Section: Discussionmentioning
confidence: 99%
“…Bilateral simultaneous CI usually make it impossible to check for any error in the early diagnosis of permanent hypoacusia, depriving the team involved in selecting candidates for CI of a helpful feedback. On the other hand, recording cortical auditory evoked potentials (CAEP) has recently proved a useful diagnostic tool when nerve and/or brainstem dysmaturity is suspected [28,29], so it may become possible to identify infants' hearing thresholds more reliably in the future.…”
Section: Discussionmentioning
confidence: 99%
“…28 A study was carried out in the Sydney Cochlear Implant Centre to evaluate the longitudinal outcomes of early implantation in auditory neuropathy spectrum disorder children with thin or hypoplastic cochlear nerves. 29 The study reported the speech perception and speech and language outcomes of five children who received cochlear implants. Two implantees had no evidence of auditory brainstem activity with the cochlear implant, two had activity only in a few electrodes, and only one had clear electrically evoked ABRs with the cochlear implant.…”
Section: Discussionmentioning
confidence: 99%
“…Integration gradually improved over the years in all five patients. 29 Jeon et al retrospectively analysed 11 auditory neuropathy spectrum disorder patients who underwent cochlear implantation and compared them with 9 control subjects with sensorineural hearing loss who did not have neural pathology. 30 The electrically evoked ABR thresholds of the auditory neuropathy spectrum disorder patients were almost within the values of the disease controls.…”
Section: Discussionmentioning
confidence: 99%
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“…Ancak, patolojinin doğasındaki heterojenlik, koklear implant adayının belirlenmesini zorlaştırdığından, detaylı bir değerlendirme gerektirmektedir. 4,[12][13][14] Koklear implant tedavisinin etkinliğini en çok etkileyen faktör komorbiditedir. Bazı araştırmacılar, işitsel nöropatiye eşlik eden başka bir bozukluk bulunmuyor ise bu durumu "izole işitsel nöropati" olarak adlandırılmaktadır.…”
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