Can stapedius reflex testing objectively measure muscle function in Pompe patients?
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Updated: Mar 30, 2026

Assessment of Neuromuscular Function Using Percutaneous Electrical Nerve Stimulation
Published on: September 13, 2015
Max J Hilz1, Ulrich Hoppe2, Sebastian Moeller1
1Department of Neurology, University of Erlangen-Nuremberg Schwabachanlage 6, D-91054, Erlangen, Germany.
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.
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