Cochlear hearing loss in patients with Laron syndrome
Cochlear hearing loss in patients with Laron syndrome
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DOI：
10.1007/s00405-011-1668-x
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发表时间：
2012-02-01
影响因子：
2.6
通讯作者：
Laron, Zvi
中科院分区：
文献类型：
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作者：
Attias, Joseph;Zarchi, Omer;Laron, Zvi
这项前瞻性临床研究的目的是测试未经治疗或接受胰岛素样生长因子I(IGF-I)治疗的Laron综合征患者的听觉功能。研究组包括11例Laron综合征患者：5例未经治疗的成人，5例2岁开始接受IGF-I替代治疗的儿童和年轻人，1例从4.6岁开始替代治疗的青少年。听力评估包括纯音和言语测听、鼓室导纳和声反射、耳声发射、响度动力学、听性脑干反应和听力减退问卷。所有未经治疗的患者和开始治疗较晚的患者均有不同程度的感音神经性听力损失和听觉超敏反应，其中大部分患者中耳听觉反射缺失。所有接受治疗的儿童听力正常，没有听觉过敏症；大多数有可记录的中耳声反射。总而言之，听觉缺陷似乎与Laron综合征有关，可以通过在发育早期开始使用IGF-I治疗来预防。
The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age < 2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.