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Linkage analysis of 15 Utah kindreds demonstrated that a gene responsible for von Recklinghausen neurofibromatosis (NF) is located near the centromere on chromosome 17. The families also gave no evidence for heterogeneity, indicating that a significant proportion of NF cases are due to mutations at a single locus. Further genetic analysis can now refine(More)
OBJECTIVES To determine whether cervical vestibular evoked myogenic potential (cVEMP) thresholds or ocular VEMP (oVEMP) amplitudes are more sensitive and specific in the diagnosis of superior semicircular canal dehiscence syndrome (SCDS). STUDY DESIGN Prospective case-control study. SETTING Tertiary referral center. SUBJECTS AND METHODS Twenty-nine(More)
OBJECTIVE To determine the test-retest reliability and age-related trends of the cervical and ocular vestibular evoked myogenic potential (cVEMP and oVEMP, respectively) responses to air-conducted sound and bone-conducted vibration stimulation. STUDY DESIGN Prospective study. SETTING Tertiary referral center. PATIENTS Fifty-three healthy adults with(More)
OBJECTIVE First, to define the best single-step suprathreshold screening test for superior canal dehiscence syndrome (SCDS); second, to obtain further insight into the relative sensitivity of vestibular afferents to sound vibration in the presence of a superior canal dehiscence. STUDY DESIGN Prospective study. SETTING Tertiary referral center. (More)
Opioid peptides and their receptors have been localized to the inner ear of the rat and guinea pig mammalian models. The expression of mu opioid receptor (MOR) in the human and mouse cochlea is not yet known. We present MOR protein localization by immunohistochemistry and mRNA expression by in situ hybridization in the human and mouse spiral ganglia (SG)(More)
Experimental animal models have suggested that the modulation of the amplitude and direction of vestibular reflexes are important functions of the vestibulocerebellum and contribute to the control of gaze and balance. These critical vestibular functions have been infrequently quantified in human cerebellar disease. In 13 subjects with ataxia telangiectasia(More)
The aim of this study was to correlate long-term vertigo control with reduction in vestibular function after intratympanic (IT) gentamicin therapy for unilateral Ménière's disease. IT gentamicin injections were given as needed to control vertigo attacks. Vertigo frequency and changes in angular vestibulo-ocular reflex (AVOR) gain (measured using magnetic(More)
47 Experimental animal models suggest that modulation of the amplitude and 48 direction of vestibular reflexes are important functions of the vestibulo-cerebellum and 49 contribute to the control of gaze and balance. These critical vestibular functions have 50 been infrequently quantified in human cerebellar disease. In 13 subjects with ataxia 51(More)
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