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Peripheral vestibular dysfunction in patients With primary ciliary dyskinesia
Rimmer, Joanne Patel, Mitesh Agarwal, Kiran Hogg, Claire Arshad, Qadeer Harcourt, Jonny
Rimmer, Joanne
Patel, Mitesh
Agarwal, Kiran
Hogg, Claire
Arshad, Qadeer
Harcourt, Jonny
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2015-04
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Abstract
Hypothesis Patients with primary ciliary dyskinesia (PCD) have absent or reduced otoconial function compared to the normal population. Background Investigations in zebrafish show that ciliation is important for the development of the otolith organs, but this has never been evaluated in humans. PCD is a congenital defect of ciliary structure. We undertook a pilot study to determine whether patients with PCD have absent or reduced otoconial function compared to the normal population. Methods Vestibular function testing, including utricular centrifugation (UCF) testing, vestibular evoked myogenic potentials (VEMPs), and electronystagmography, was undertaken in five patients with known PCD. Patients also completed validated questionnaires regarding subjective balance function and symptoms. Results There were markedly reduced or unobtainable VEMPs bilaterally in three of the five subjects and unilaterally in the remaining two subjects. No subject had a pathological UCF asymmetry, but three subjects showed utricular abnormalities. The vestibulo-ocular reflex (VOR) at 0.25 Hz sinusoidal rotation was normal in all subjects. There were no subjective dizzy symptoms or balance issues. Conclusion We speculate that the reduced saccular and utricular function in PCD patients observed in this pilot study suggests a relationship between cilia structure and/or motility, and otoconia seeding and/or positioning. Further investigation is warranted.
Citation
Rimmer, J., Patel, M., Agarwal, K., Hogg, C., et al. (2015) Peripheral vestibular dysfunction in patients with primary ciliary dyskinesia, Otology & Neurotology, 36 (4) pp. 662-669 DOI: 10.1097/MAO.0000000000000592
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Journal article
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en
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This is an accepted manuscript of an article published by Lippincott, Williams and Wilkins in Otology and Neurotology in April 2015, available online: https://doi.org/10.1097/MAO.0000000000000592
The accepted version of the publication may differ from the final published version.
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1531-7129