Can stapedius reflex testing objectively measure muscle function in Pompe patients?
نویسندگان
چکیده
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.
منابع مشابه
Stapedius reflex testing shows altered small muscle function in untreated Pompe patients and improvement after enzyme replacement therapy
Introduction Pompe disease primarily affects skeletal and cardiac muscles. It is difficult to assess therapeutic efficacy of enzyme replacement therapy (ERT) using primary end points based on changes in weakness of large limb-girdle muscles, gait-endurance, and respiratory function, which all depend on variable day-to-day patient performance. In contrast, muscle improvement might be shown by as...
متن کاملA preliminary study of endoscopic acoustic stapedial reflex in chronic otitis media.
OBJECTIVE To introduce the endoscopic acoustic stapedial reflex (EASR) as a technique for assessing stapedial mobility in ears with chronic otitis media (COM). METHODS This prospective study was performed from February 2008 to February 2009, patients with COM presented to the Otology Clinic, King Abdul-Aziz University Hospital, Riyadh, Kingdom of Saudi Arabia were examined with a rigid ear en...
متن کاملThe Stapedius Reflex: Beyond its Visually Observable Binary On/Off Behavior
Visually, the stapedius reflex is an on/off phenomenon that either appears or not. However, this paper presents some additional data that suggests a rather continuous stapedius reflex that above a certain threshold, leads to progressively increasing forces of the stapedius muscle. With this effect, the stapedius reflex can be used not only for the initial calibration of the cochlear implant’s s...
متن کاملAutomatic Detection of the Electrical Elicited Stapedius Reflex by Evaluation of Its Electromyographic Signals
This paper provides a first preliminary description of the dependencies between the stapedius muscle’s behavior and its neuronal activation with the adjustment of cochlear implants in mind. For that, stapedial electromyography (EMG) data are compared with EMG data which were derived from the quadriceps femoris muscle. The rationale behind is, that the stapedius muscle is classified as a striate...
متن کاملImprovement of Stapedial Muscle Reflex Threshold by Acoustic Sensitivity of the Saccule
Acoustic sensitivity of the human vestibular system has long been established and can be demonstrated by means of the cervical vestibular evoked myogenic potential (cVEMPs). The objective of this cross-sectional study is to investigate the possibility that the acoustic sensitivity of the saccule improves stapedial muscle reflex threshold. Twenty healthy persons and twenty-five patients as havin...
متن کامل