Open AccessCan stapedius reflex testing objectively measure muscle function in Pompe patients?
Author(s) -
Hilz Max J.,
Hoppe Ulrich,
Moeller Sebastian,
Wang Ruihao,
Koehn Julia
Publication year - 2015
Publication title -
clinical case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.21
H-Index - 9
ISSN - 2050-0904
DOI - 10.1002/ccr3.400
Subject(s) - medicine , enzyme replacement therapy , acoustic reflex , reflex , audiology , disease , anesthesia
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 .