4 research outputs found

    Repeatability of sound evoked triceps myogenic potentials

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    Objective: To investigate the repeatability of sound-evoked vestibular evoked myogenic potentials recorded from the triceps (tVEMPs) with and without visual feedback. Design: tVEMP responses to 95 dB nHL 500-Hz tone bursts were recorded in a longitudinal, repeated measures study where P1 and N1 latencies and amplitudes were measured on three separate occasions from the same individuals. Analysis of variance, intra-class correlations, and limits of repeatability analyses were used to assess tVEMP repeatability and effects of visual feedback. Study sample: Fifteen participants (nine women) aged between 18 and 41 years took part. Results: Response rates of 63% and 68% were obtained for tVEMPs with eyes open and closed, respectively. When present, tVEMP latencies and amplitudes exhibited fair to good repeatability. Repeatability of tVEMP latencies and amplitudes measured using Bland-Altman methods was poorer with eyes closed. Conclusions: Sound-evoked tVEMP response rates are too low to support their clinical utility at the moment. tVEMP response rate may be improved by refining the balance task to include a force related target. Better tVEMP repeatability with eyes open supports the hypothesis that the response is modulated by visual feedback, and is consistent with studies reporting triceps responses to galvanic stimulation

    Superoxide production is inversely related to complex I activity in inherited complex I deficiency.

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    Contains fulltext : 53008.pdf (publisher's version ) (Closed access)Deficiency of NADH:ubiquinone oxidoreductase or complex I (CI) is the most common cause of disorders of the oxidative phosphorylation system in humans. Using life cell imaging and blue-native electrophoresis we quantitatively compared superoxide production and CI amount and activity in cultured skin fibroblasts of 7 healthy control subjects and 21 children with inherited isolated CI deficiency. Thirteen children had a disease causing mutation in one of the nuclear-encoded CI subunits, whereas in the remainder the genetic cause of the disease is not yet established. Superoxide production was significantly increased in all but two of the patient cell lines. An inverse relationship with the amount and residual activity of CI was observed. In agreement with this finding, rotenone, a potent inhibitor of CI activity, dose-dependently increased superoxide production in healthy control cells. Also in this case an inverse relationship with the residual activity of CI was observed. In sharp contrast, however, rotenone did not decrease the amount of CI. The data presented show that superoxide production is increased in inherited CI deficiency and that this increase is primarily a consequence of the reduction in cellular CI activity and not of a further leakage of electrons from mutationally malformed complexes
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