Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder)
Multi-site diagnosis and management of 260 patients with Auditory Neuropathy/Dys-synchrony (Auditory Neuropathy Spectrum Disorder)
复制标题
DOI：
10.3109/14992020903160892
复制
发表时间：
2010-01-01
影响因子：
2.7
通讯作者：
Frisch, Stefan A.
中科院分区：
文献类型：
--
作者：
Berlin, Charles I.;Hood, Linda J.;Frisch, Stefan A.
总结了260例诊断为听神经病谱系障碍（ANSD）的患者的测试结果和管理数据。其中85名患者尝试了助听器，49名患者尝试了人工耳蜗植入。大约15%的患者报告助听器对语言学习有一定的益处，而85%的植入患者报告了言语理解和语言习得的改善。总人口中约有5%（13/260）在没有干预的情况下发展了正常的言语和语言。患者在我们的实验室诊断（n = 66）或从其他网站（n=194）转介，并都表现出缺席/严重异常的听觉脑干反应（ABR），经常“振铃”耳蜗微音，和耳声发射的存在或历史。病因和共存疾病包括遗传（n=41）、周围神经病变（n=20）、围产期黄疸和/或缺氧和/或早产（n=74）。这些患者占听力受损患者的10%或更多：他们的语言习得轨迹通常无法从听力图中预测。
Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n = 66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often 'ringing' cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients: their language acquisition trajectories are generally unpredictable from their audiograms.