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

Abstract

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 .