Raveh Eyal, Buller Nora, Badrana Ola, Attias Joseph
Pediatric Otolaryngology Unit, Schneider Children's Medical Center of Israel, Petah Tiqwa, Israel.
Am J Otolaryngol. 2007 Sep-Oct;28(5):302-8. doi: 10.1016/j.amjoto.2006.09.006.
Auditory neuropathy is characterized by congenital sensorineural hearing loss associated with absent or impaired auditory brainstem evoked responses and preservation of outer hair cell activity. This study describes the recent experience of our tertiary pediatric center with auditory neuropathy (AN).
The files of all children diagnosed with AN at our center from 2000 to 2005 were reviewed for background data, associated factors, laboratory and audiometry findings, management, and outcome.
Mean age at diagnosis was 13 months. Factors known to be associated with AN were found in 18 children, namely, prematurity, hyperbilirubinemia, parental consanguinity, or positive family history. Conception by in vitro fertilization was an additional factor not previously reported. The hearing loss was mostly moderate to severe, and bilateral in all patients but one. Otoacoustic emissions and/or cochlear microphonics were demonstrated in all cases. Hearing improved spontaneously in 4 patients. Management with a hearing aid was successful in 1 of 19 patients. Twelve patients received cochlear implants with good outcome.
Because neonates with AN have normal otoacoustic emissions and/or cochlear microphonics, screening tests for high-risk neonates should be complemented by auditory brainstem evoked responses to avoid false-negative findings. Because AN is considered a retrocochlear lesion, with normal outer hair cell function, rehabilitation with hearing aids is problematic. Although the level of pathology is apparently at the cochlear nerve, cochlear implantation is often a good solution for failures of conventional rehabilitation. However, our finding of spontaneous improvement in a small subgroup raises questions regarding implantation before age 1 year.
听神经病的特征为先天性感音神经性听力损失，伴有听觉脑干诱发电位缺失或受损，而外毛细胞活动保留。本研究描述了我们三级儿科中心近期在听神经病（AN）方面的经验。
回顾了2000年至2005年在我们中心诊断为AN的所有儿童的病历，以获取背景数据、相关因素、实验室和听力测定结果、治疗及预后情况。
诊断时的平均年龄为13个月。在18名儿童中发现了已知与AN相关的因素，即早产、高胆红素血症、父母近亲结婚或家族史阳性。体外受精受孕是此前未报告的另一个因素。听力损失大多为中度至重度，除1例患者外均为双侧。所有病例均检测到耳声发射和/或耳蜗微音电位。4例患者听力自发改善。19例患者中1例使用助听器治疗成功。12例患者接受了人工耳蜗植入，效果良好。
由于患有AN的新生儿耳声发射和/或耳蜗微音电位正常，高危新生儿的筛查测试应以听觉脑干诱发电位作为补充，以避免假阴性结果。由于AN被认为是一种蜗后病变，外毛细胞功能正常，使用助听器进行康复存在问题。尽管病理水平显然位于蜗神经，但人工耳蜗植入对于传统康复失败的患者通常是一个很好的解决方案。然而，我们在一小部分亚组中发现的自发改善现象引发了关于1岁前植入的疑问。
