Clinical and audiological features in auditory neuropathy
Clinical and audiological features in auditory neuropathy
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DOI：
10.1001/archotol.128.9.1026
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发表时间：
2002-09-01
影响因子：
--
通讯作者：
Choo, DI
中科院分区：
文献类型：
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作者：
Madden, C;Rutter, M;Choo, DI
目的：从医学和听力学上描述被诊断为患有听神经病 (AN) 的儿童群体。研究设计：回顾性病历审查设置/对象：我们从三级儿科医院的儿科耳科诊所确定了 22 名患者。结果：通过我们对 3 个家庭的 2 名患病儿童和另外 2 名有听力损失家族史的儿童的鉴定，表明 AN 存在遗传因素。我们人群中常见的临床特征包括高胆红素血症史（n = 11 [50%]）、早产（n = 10 [45%]）、耳毒性药物暴露（n = 9 [41%]）、听力损失家族史（n = 8 [36%]）、新生儿呼吸机依赖（n = 8 [36%]）和脑瘫（n = 2 [9%]）。 22 名儿童中的 18 名获得了完整的临床和听力学数据，包括耳声发射、耳蜗麦克风的听觉脑干反应以及适合年龄的听力测定结果。值得注意的是，随着时间的推移，这 18 名患者中有 9 名的行为阈值有所改善，这表明一部分患有 AN 的儿童可能会恢复有用的听力水平。同样重要的是 4 名儿童的人工耳蜗植入成功。结论：儿童 AN 的治疗需要进行系列临床和听力评估，其中行为测试发挥着重要作用。早产、遗传和高胆红素血症似乎是 AN 发生的重要因素；高胆红素血症可能与听力阈值的自发改善有关。对于那些无法从扩音或调频系统中受益的儿童来说，人工耳蜗植入仍然是一种可能成功的康复方法。
Objective: To medically and audiologically characterize a population of children diagnosed as having auditory neuropathy (AN).Study Design: Retrospective medical chart reviewSetting/Subjects: We identified 22 patients from a pediatric otology clinic in a tertiary care pediatric hospital setting.Results: A genetic factor in AN is suggested by our identification of 3 families with 2 affected children and 2 other children with family histories that were positive for hearing loss. Clinical features common among our population included a history of hyperbilirubinemia (n = 11 [50%]), prematurity (n = 10 [45%]), ototoxic drug exposure (n = 9 [41%]), family history of hearing loss (n = 8 [36%]), neonatal ventilator dependence (n = 8 [36%]), and cerebral palsy (n = 2 [9%]). Full clinical and audiological data were available for 18 of the 22 children, including otoacoustic emissions, auditory brainstem responses with cochlear microphonics, and age-appropriate audiometric findings. Significantly, 9 of these 18 patients showed improvement in behavioral thresholds over time, indicating that a subset of children with AN may recover useful hearing levels. Also significant was the success of cochlear implantation in 4 children.Conclusions: Management of AN in children requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. Prematurity, genetics, and hyperbilirubinemia appear to be significant factors in the development of AN; hyperbilirubinemia can be associated with spontaneous improvement of hearing thresholds. For those children not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.