Deterioration of the auditory brainstem response in children with type 3 Gaucher disease

被引：25

作者：

Campbell, PE ^{[1
]}

Harris, CM

Vellodi, A

机构：

[1] Univ Plymouth, Ctr Theoret & Computat Neurosci, Sensorimotor Lab, Plymouth PL4 8AA, Devon, England

[2] Derriford Hosp, Dept Audiol, Plymouth PL6 8DH, Devon, England

[3] Great Ormond St Hosp Sick Children, Metab Med Unit, London, England

来源：

NEUROLOGY | 2004年 / 63卷 / 02期

关键词：

D O I：

10.1212/01.WNL.0000130191.31669.48

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Enzyme replacement therapy (ERT) has improved the quality of life in patients with Gaucher disease (GD). An emerging concern is whether ERT can also halt the neurologic progression in type 3 GD. The authors examined the auditory brainstem response (ABR) in eight children with type 3 GD undergoing high-dose ERT and found a consistent deterioration in ABR response. They conclude that ERT does not halt brainstem degeneration and that alternative therapies must be sought.

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页码：385 / 387

页数：3

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