Auditory neuropathy characteristics in children with cochlear nerve
Auditory neuropathy characteristics in children with cochlear nerve
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DOI：
10.1097/01.aud.0000224100.30525.ab
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发表时间：
2006-08-01
期刊：
影响因子：
3.7
通讯作者：
Grose, John H.
中科院分区：
文献类型：
--
作者：
Buchman, Craig A.;Roush, Patricia A.;Grose, John H.
目的：描述一组表现出听神经病（AN）特征性电生理反应的儿童，这些儿童随后被鉴定为耳蜗神经缺失或较小（即，设计：回顾性分析临床记录、听力学测试结果和磁共振成像（MRI）研究。在听觉脑干反应（ABR）测试中，65名具有AN特征的儿童中有51名有MRI可供审查。在这51名具有AN的ABR特征的儿童中，有9名（18%）在MRI上被确定为耳蜗神经小（N = 2; 4%）或缺失（N = 7; 14%）。九分之八的新生儿听力筛查失败后，而一个在3岁。在诊断性ABR测试中，所有9名儿童（13只受影响的耳朵中有9只; 69%）都有耳蜗颤噪（CM）的证据，至少有一只耳朵没有神经反应。在单侧病例中，所有受累耳均检测到AN特征。在双侧病例中，每个孩子至少有一只耳朵表现出AN表型，而对侧耳没有发现CM。只有一只耳蜗神经缺损的耳朵有目前的耳声发射测量失真产品耳声发射。在具有适当可用行为测试结果的儿童中，所有没有耳蜗神经的耳朵都被确定为具有深度听力损失。耳蜗神经缺损13耳中仅4耳（31%）MRI表现为内听道狭窄。结论：耳蜗神经缺损患儿可出现AN的电生理表现。这些儿童经常参考使用ABR为基础的测试方法的新生儿筛查检查。与AN的其他原因类似，诊断性ABR测试将显示CM缺乏神经反应。在我们的项目中，51名具有AN的MRI和电生理特征的儿童中有9名（18%）被确定为耳蜗神经缺陷，因此这是一种相对常见的诊断。这些结果表明，MRI适用于所有诊断为AN的儿童。此外，电生理证据单侧AN与深度听力损失应使临床医生高度怀疑这个问题。虽然有小神经的耳蜗神经缺陷儿童可能受益于人工耳蜗植入或放大，但这些干预措施显然禁忌于完全没有耳蜗神经的儿童。
Objective: To describe a group of children exhibiting electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency).Design: A retrospective review of the clinical records, audiological testing results, and magnetic resonance imaging (MRI) studies. Fifty-one of 65 children with AN characteristics on auditory brain stem response (ABR) testing had MRI available for review. Nine (18%) of these 51 children with ABR characteristic of AN have been identified as having small (N = 2; 4%) or absent (N = 7; 14%) cochlear nerves on MRI.Results: Of the nine children with cochlear nerve deficiency, five (56%) were affected unilaterally and four (44%) bilaterally. Eight of nine presented after failing a newborn infant hearing screening, whereas one presented at 3 yr of age. On diagnostic ABR testing, all 9 children (9 of 13 affected ears; 69%) had evidence of a cochlear microphonic (CM) and absent neural responses in at least one ear. In the unilateral cases, AN characteristics were detected in all affected ears. In bilateral cases, at least one of the ears in each child demonstrated the AN phenotype, whereas the contralateral ear had no CM identified. Only one ear with cochlear nerve deficiency had present otoacoustic emissions as measured by distortion-product otoacoustic emissions. In children with appropriate available behavioral testing results, all ears without cochlear nerves were identified as having a profound hearing loss. Only 4 (31%) of the 13 ears with cochlear nerve deficiency had a small internal auditory canal on MRI.Conclusions: Children with cochlear nerve deficiency can present with electrophysiologic evidence of AN. These children frequently refer on newborn screening examinations that use ABR-based testing methods. Similar to other causes of AN, diagnostic ABR testing will show a CM with absent neural responses. Given that 9 (18%) of 51 children with available MRI and electrophysiologic characteristics of AN in our program have been identified as having cochlear nerve deficiency makes this a relatively common diagnosis. These findings suggest that MRI is indicated for all children diagnosed with AN. Moreover, electrophysiologic evidence of unilateral AN in association with a profound hearing loss should make the clinician highly suspicious for this problem. Although children with cochlear nerve deficiency who have a small nerve may benefit from cochlear implantation or amplification, these interventions are obviously contraindicated in children with completely absent cochlear nerves.