Auditory neuropathy: unexpectedly common in a screened newborn population

Auditory neuropathy: unexpectedly common in a screened newborn population
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DOI:
10.1111/j.1469-8749.2009.03298.x
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发表时间:
2009-08-01
影响因子:
3.8
通讯作者:
Gibbin, Kevin P.
Gibbin, Kevin P.
中科院分区:
医学2区
文献类型:
--
作者:
Dowley, Andrew C.;Whitehouse, William P.;Gibbin, Kevin P.

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听觉神经病,或不同步,是由异常或缺乏听觉脑干反应,但完整的耳声发射或耳蜗微音。它与行为纯音测听的听力受损、声反射缺失和言语感知差有关,特别是在嘈杂的环境中。这些结果表明内毛细胞和/或第八神经功能障碍。我们描述了一个病例说明调查的患者有和没有听神经病,使用的数据从当地的新生儿听力筛查计划收集的前瞻性从2002年至2007年。在此期间,对45050名婴儿进行了耳声发射筛查,30名患者被诊断为疑似重度至极重度听力损失(16名男性,14名女性),其中12名患有听神经病(6名男性,6名女性)。听神经病组的平均胎龄为33周1天,非听神经病组为35周。听神经病最显著的风险因素是高胆红素血症(p=0.018)、脓毒症(p=0.024)和庆大霉素暴露(p=0.024)。听神经病儿童包括一个听力障碍患者亚组,涉及最常见的与新生儿重症监护病房入院相关的风险因素相关的不同病理。随着成熟,至少在少数人中,改善是可能的。
Auditory neuropathy, or dyssynchrony, is defined by an abnormal or absent auditory brainstem response but intact otoacoustic emissions or cochlear microphonics. It is associated with impaired hearing on behavioural pure-tone audiometry, absent acoustic reflexes, and poor speech perception, particularly in noisy environments. These results suggest a disorder of inner hair-cell and or eighth-nerve function. We describe a case-note survey of patients with and without auditory neuropathy, using data from the local newborn hearing screening programme collected prospectively from 2002 to 2007. During this period, 45 050 infants were screened with otoacoustic emissions, 30 patients were diagnosed with suspected severe to profound hearing loss (16 males, 14 females), and 12 of those 30 had auditory neuropathy (six males, six females). Mean gestational age was 33 weeks 1 day in the auditory neuropathy group and 35 weeks in the non-auditory neuropathy group. The most significant risk factors for auditory neuropathy were hyperbilirubinaemia (p=0.018), sepsis (p=0.024), and gentamicin exposure (p=0.024). Children with auditory neuropathy comprise a subgroup of patients with hearing impairment involving different pathologies most commonly associated with the risk factors related to admission to neonatal intensive care units. Improvement is possible with maturity, at least in a minority.