Morant Ventura A, Orts Alborch M, García Callejo J, Pitarch Ribas M I, Marco Algarra J
Servicio de ORL, Hospital Clínico de Valencia, Universidad de Valencia.
Acta Otorrinolaringol Esp. 2000 Aug-Sep;51(6):530-4.
Auditory neuropathy is a sensorineural disorder characterized by absent or abnormal auditory evoked potentials, and normal cochlear outer hair cell function. It is believed that a variety of processes are involved in its pathophysiology and their influence on hearing can differ. We describe the diagnostic sequence and management of two new cases of auditory neuropathy in infants. The first case was a girl with no risk factors for hearing loss. Her absence of response to sounds was compared with her twin's reactions. Otoacoustic emissions were present but no auditory evoked potential response was detected. Her evolution was characteristic of deep sensorineural hearing loss without a hearing aid. At present she is awaiting a cochlear implant. The second case was a boy who underwent hearing loss screening for hyperbilirubinemia. Examinations were repeated three months later because the mother suspected hearing loss. Objective tests showed a pattern of auditory neuropathy. The boy's evolution was different: in spite of the presence of destructured auditory evoked potentials; it was evident that the boy conserved some hearing and had thresholds suggestive of moderate hearing loss.
听神经病是一种感觉神经性疾病，其特征为听觉诱发电位缺失或异常，而耳蜗外毛细胞功能正常。据信，其病理生理学涉及多种过程，且它们对听力的影响可能有所不同。我们描述了两例婴儿听神经病新病例的诊断过程和治疗情况。第一例是一名无听力损失危险因素的女孩。将她对声音无反应的情况与其双胞胎的反应进行了比较。耳声发射存在，但未检测到听觉诱发电位反应。她的病情发展具有重度感觉神经性听力损失且未佩戴助听器的特征。目前她正在等待人工耳蜗植入。第二例是一名因高胆红素血症接受听力损失筛查的男孩。由于母亲怀疑听力损失，三个月后重复进行了检查。客观测试显示出听神经病的模式。该男孩的病情发展有所不同：尽管听觉诱发电位结构紊乱，但很明显该男孩仍保留了一些听力，其阈值提示为中度听力损失。
