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

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...

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Autores principales: Hilz, Max J, Hoppe, Ulrich, Moeller, Sebastian, Wang, Ruihao, Koehn, Julia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2015
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4641478/
https://www.ncbi.nlm.nih.gov/pubmed/26576276
http://dx.doi.org/10.1002/ccr3.400
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author Hilz, Max J
Hoppe, Ulrich
Moeller, Sebastian
Wang, Ruihao
Koehn, Julia
author_facet Hilz, Max J
Hoppe, Ulrich
Moeller, Sebastian
Wang, Ruihao
Koehn, Julia
author_sort Hilz, Max J
collection PubMed
description 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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spelling pubmed-46414782015-11-16 Can stapedius reflex testing objectively measure muscle function in Pompe patients? Hilz, Max J Hoppe, Ulrich Moeller, Sebastian Wang, Ruihao Koehn, Julia Clin Case Rep Case Reports 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. John Wiley & Sons, Ltd 2015-11 2015-09-28 /pmc/articles/PMC4641478/ /pubmed/26576276 http://dx.doi.org/10.1002/ccr3.400 Text en © 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the terms of the 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.
spellingShingle Case Reports
Hilz, Max J
Hoppe, Ulrich
Moeller, Sebastian
Wang, Ruihao
Koehn, Julia
Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title_full Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title_fullStr Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title_full_unstemmed Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title_short Can stapedius reflex testing objectively measure muscle function in Pompe patients?
title_sort can stapedius reflex testing objectively measure muscle function in pompe patients?
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4641478/
https://www.ncbi.nlm.nih.gov/pubmed/26576276
http://dx.doi.org/10.1002/ccr3.400
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