Can stapedius reflex testing objectively measure muscle function in Pompe patients?

Clinical Case Reports

Max J. Hilz¹  Ulrich Hoppe²  Sebastian Moeller¹  Ruihao Wang¹ 

[1] Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany;Department of Audiology, University of Erlangen-Nuremberg, Erlangen, Germany

关键词: Enzyme replacement therapy;    muscle function;    Pompe disease;    stapedius reflex testing;   

DOI  :  10.1002/ccr3.400

来源: Wiley

【 摘 要 】

Key Clinical Message

We assessed the value of stapedius reflex testing as an objective measurement of striated muscle function in four patients with Pompe disease. Common tests of disease severity and efficacy of enzyme replacement therapy (ERT) yield highly variable results that depend on patient cooperation and day-to-day performance, whereas stapedius reflex thresholds improved in seven of eight ears after 2 years of ERT.

【 授权许可】

CC BY-NC-ND   
© 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.

Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.