Peripheral Vestibular Dysfunction in Patients With Primary Ciliary Dyskinesia

Article


Rimmer, Joanne, Patel, M., Agarwal, Kiran, Hogg, Claire, Arshad, Qadeer and Harcourt, Jonny 2015. Peripheral Vestibular Dysfunction in Patients With Primary Ciliary Dyskinesia. Otology and Neurotology. 36 (4), pp. 662-669.
AuthorsRimmer, Joanne, Patel, M., Agarwal, Kiran, Hogg, Claire, Arshad, Qadeer and Harcourt, Jonny
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.

JournalOtology and Neurotology
Journal citation36 (4), pp. 662-669
ISSN1531-7129
Year2015
PublisherLippincott, Williams & Wilkins
Accepted author manuscript
License
CC BY
Web address (URL)http://dx.doi.org/10.1097/MAO.0000000000000592
Publication dates
PrintApr 2015
Publication process dates
Deposited25 Nov 2015
Copyright information© 2015 Otology & Neurotology
Permalink -

https://repository.uel.ac.uk/item/856q5

  • 6
    total views
  • 31
    total downloads
  • 1
    views this month
  • 4
    downloads this month

Related outputs

Psychological Assessment and Documentation of Torture in Detention
Patel, M. 2017. Psychological Assessment and Documentation of Torture in Detention. in: Payne-James, Jason, Beynon, Jonathan and Vieira, Duarte (ed.) Monitoring Detention, Custody, Torture and Ill-treatment: A Practical Approach to Prevention and Documentation CRC Press: Taylor & Francis Group.
Developing policy-relevant skills in clinical psychology training
Peacock-Brennan, S., Harper, D. and Patel, M. 2018. Developing policy-relevant skills in clinical psychology training. Clinical Psychology Forum.
Oculomotor Deficits after Chemotherapy in Childhood
Rota, Rossella, Einarsson, Einar-Jón, Patel, M., Petersen, Hannes, Wiebe, Thomas, Magnusson, Måns, Moëll, Christian and Fransson, Per-Anders 2016. Oculomotor Deficits after Chemotherapy in Childhood. PLOS ONE. 11 (1), p. e0147703.
Vestibular and oculomotor influences on visual dependency
Roberts, R. Edward, Da Silva Melo, Mariane, Siddiqui, Aazim A, Arshad, Qadeer and Patel, M. 2016. Vestibular and oculomotor influences on visual dependency. Journal of Neurophysiology. 116 (3), pp. 1480-1487.
Acute alcohol intoxication impairs segmental body alignment in upright standing
Hafstrom, A, Patel, M., Modig, F, Magnusson, M and Fransson, Per-Anders 2014. Acute alcohol intoxication impairs segmental body alignment in upright standing. Journal of Vestibular Research. 24 (4), pp. 297-304.
Applications of neuromodulation to explore vestibular cortical processing; new insights into the effects of direct current cortical modulation upon pursuit, VOR and VOR suppression
Ahmad, H, Arshad, Qadeer, Siddiqui, S, Nigmatullina, Y., Patel, M., Bronstein, A. M. and Roberts, R. E. 2014. Applications of neuromodulation to explore vestibular cortical processing; new insights into the effects of direct current cortical modulation upon pursuit, VOR and VOR suppression. Journal of Vestibular Research. 24 (5-6), pp. 453-458.
Decreased postural control in adolescents born with extremely low birth weight
Petersen, Hannes, Tulinius, Arnar-Thor, Georgsdóttir, Ingibjörg, Einarsson, Einar-Jon, Patel, M., Haraldsson, Ásgeir and Fransson, Per-Anders 2015. Decreased postural control in adolescents born with extremely low birth weight. Experimental Brain Research. 233 (5), pp. 1651-1662.
Using Transcranial Magnetic Stimulation (TMS) to Probe Effects of Visual Motion Adaptation on Primary Visual Cortex (V1) Excitability in Bilateral Vestibular Failure (BVF) Patients
Ahmad, Hena, Roberts, Richard, Arshad, Qadeer Arshad, Patel, M. and Bronstein, Adolfo 2015. Using Transcranial Magnetic Stimulation (TMS) to Probe Effects of Visual Motion Adaptation on Primary Visual Cortex (V1) Excitability in Bilateral Vestibular Failure (BVF) Patients. Journal of Neurology, Neurosurgery & Psychiatry. 86 (11), pp. e4.70-e4.
Acquired Pendular Nystagmus in Stargardt's Syndrome Suppressed by Alcohol
Ahmad, Hena, Arshad, Qadeer, Patel, M., Roberts, Richard and Bronstein, Adolfo 2015. Acquired Pendular Nystagmus in Stargardt's Syndrome Suppressed by Alcohol. Journal of Neurology, Neurosurgery & Psychiatry. 86 (11), pp. e4.120-e4.
Effects of Prochlorperazine on Normal Vestibular Ocular and Perceptual Responses: A Randomised, Double-Blind, Crossover, Placebo-Controlled Study
Patel, M., Nigmatullina, Y., Seemungal, B.M., Golding, J.F. and Bronstein, A.M. 2013. Effects of Prochlorperazine on Normal Vestibular Ocular and Perceptual Responses: A Randomised, Double-Blind, Crossover, Placebo-Controlled Study. Audiology and Neurotology. 19 (2), pp. 91-96.
Attention modulates adaptive motor learning in the ‘broken escalator’ paradigm
Patel, M., Kaski, Diego and Bronstein, Adolfo M. 2014. Attention modulates adaptive motor learning in the ‘broken escalator’ paradigm. Experimental Brain Research. 232 (7), pp. 2349-2357.
Does outstretching the arms improve postural stability?
Patel, M., Buckwell, David, Hawken, Malcolm and Bronstein, Adolfo M. 2014. Does outstretching the arms improve postural stability? Neuroscience Letters. 579, pp. 97-100.
Long-Term Effects from Bacterial Meningitis in Childhood and Adolescence on Postural Control
Lidzba, Karen, Petersen, Hannes, Patel, M., Ingason, Einar F., Einarsson, Einar J., Haraldsson, Ásgeir and Fransson, Per-Anders 2014. Long-Term Effects from Bacterial Meningitis in Childhood and Adolescence on Postural Control. PLOS ONE. 9 (11), p. e112016.
A brief review of the clinical anatomy of the vestibular-ocular connections—how much do we know?
Bronstein, A M, Patel, M. and Arshad, Q 2015. A brief review of the clinical anatomy of the vestibular-ocular connections—how much do we know? Eye. 29 (2), pp. 163-170.
Role of handedness-related vestibular cortical dominance upon the vestibular–ocular reflex
Arshad, Q., Patel, M., Goga, U., Nigmatullina, Y. and Bronstein, A. M. 2015. Role of handedness-related vestibular cortical dominance upon the vestibular–ocular reflex. Journal of Neurology. 262 (4), pp. 1069-1071.
Galvanic Vestibular Stimulation Induces a Spatial Bias in Whole-body Position Estimates
Patel, M., Roberts, R. Edward, Arshad, Qadeer, Ahmed, Maroof, Riyaz, Mohammed U. and Bronstein, Adolfo M. 2015. Galvanic Vestibular Stimulation Induces a Spatial Bias in Whole-body Position Estimates. Brain Stimulation. 8 (5), pp. 981-983.
Locomotor adaptation is modulated by observing the actions of others
Patel, M., Roberts, R. Edward, Riyaz, Mohammed U., Ahmed, Maroof, Buckwell, David, Bunday, Karen, Ahmad, Hena, Kaski, Diego, Arshad, Qadeer and Bronstein, Adolfo M. 2015. Locomotor adaptation is modulated by observing the actions of others. Journal of Neurophysiology. 114 (3), pp. 1538-1544.
Chronic symptoms after vestibular neuritis and the high velocity vestibulo-ocular reflex
Patel, M., Arshad, Qadeer, Roberts, R. Edward, Ahmad, Hena and Bronstein, Adolfo M. 2016. Chronic symptoms after vestibular neuritis and the high velocity vestibulo-ocular reflex. Otology and Neurotology. 37 (2), pp. 179-184.