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Peripheral vestibular dysfunction in patients with primary ciliary dyskinesia: abnormal otoconial development?
- Source :
-
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology [Otol Neurotol] 2015 Apr; Vol. 36 (4), pp. 662-9. - Publication Year :
- 2015
-
Abstract
- Hypothesis: Patients with primary ciliary dyskinesia (PCD) have absent or reduced otoconial function compared to the normal population.<br />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.<br />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.<br />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.<br />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.
- Subjects :
- Adolescent
Aged
Dizziness physiopathology
Electronystagmography
Female
Humans
Male
Middle Aged
Pilot Projects
Reflex, Vestibulo-Ocular physiology
Vestibular Diseases epidemiology
Vestibular Diseases physiopathology
Vestibular Evoked Myogenic Potentials physiology
Vestibular Function Tests
Kartagener Syndrome complications
Otolithic Membrane abnormalities
Vestibular Diseases etiology
Subjects
Details
- Language :
- English
- ISSN :
- 1537-4505
- Volume :
- 36
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology
- Publication Type :
- Academic Journal
- Accession number :
- 25226371
- Full Text :
- https://doi.org/10.1097/MAO.0000000000000592