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Otolith Function in Patients With Primary Ciliary Dyskinesia

This study has been completed.
Information provided by (Responsible Party):
Imperial College Healthcare NHS Trust Identifier:
First received: November 22, 2010
Last updated: March 8, 2016
Last verified: August 2014
The purpose of this study is to determine whether patients with primary ciliary dyskinesia (PCD) have reduced or absent otolith function.The otolith system is a specific part of the inner ear vestibular (balance) system that detects linear movement.

Condition Intervention
Primary Ciliary Dyskinesia
Other: Vestibular evoked myogenic potentials (VEMPs)
Other: Utricular centrifugation test

Study Type: Observational
Study Design: Observational Model: Case-Only
Time Perspective: Cross-Sectional
Official Title: Otolith Function in Patients With Primary Ciliary Dyskinesia: a Pilot Study

Resource links provided by NLM:

Further study details as provided by Imperial College Healthcare NHS Trust:

Primary Outcome Measures:
  • Vestibular Evoked Myogenic Potentials (VEMPs) [ Time Frame: one day ]
    These are the balance tests that specifically assess the otolith organ.

Other Outcome Measures:
  • Utricular Centrifugation Test (UCF) [ Time Frame: one day ]
    balance assessment

Enrollment: 5
Study Start Date: September 2010
Study Completion Date: December 2013
Primary Completion Date: December 2013 (Final data collection date for primary outcome measure)
Groups/Cohorts Assigned Interventions
Test group
Standard tests of balance function
Other: Vestibular evoked myogenic potentials (VEMPs)
Standard test of balance function
Other: Utricular centrifugation test
Standard test of balance function

Detailed Description:

Primary ciliary dyskinesia (PCD) is a genetically inherited condition. It is due to structural abnormalities of cilia, which are microscopic hairs found in organs and cells throughout the body. Patients with this condition typically develop upper respiratory tract symptoms such as sinusitis and glue ear, lower respiratory tract problems such as recurrent chest infections, and fertility problems. There is currently no evidence that patients with PCD have a higher incidence of balance problems. However, recent animal studies have shown that cilia may also be important in the development of part of the inner ear balance (vestibular) system, specifically the part that detects linear movement known as the otolith system.

The investigators hope to determine whether patients with PCD have absent or reduced otolith function compared to the normal population. Balance problems are not currently screened for in PCD patients, and could be unrecognized and therefore untreated. It is also possible that PCD patients have compensated for absent otolith function and so are unaffected under normal circumstances; any additional insult to their balance system would cause more pronounced difficulties than expected and treatment might need to reflect this.


Ages Eligible for Study:   16 Years to 30 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Ten patients from the PCD clinic at the Royal Brompton Hospital, London.

Inclusion Criteria:

  • aged 16-30 years
  • Confirmed diagnosis of PCD under the care of the PCD team at the Royal Brompton Hospital

Exclusion Criteria:

  • Aged under 16 years
  • Unconfirmed or "suspected" PCD
  • Previous history of balance disorders or diagnoses
  • Previous history of sensorineural hearing loss
  • Previous middle or inner ear surgery (other than grommet insertion)
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Please refer to this study by its identifier: NCT01246258

United Kingdom
Charing Cross Hospital
London, United Kingdom, W6 8RF
Sponsors and Collaborators
Imperial College Healthcare NHS Trust
Principal Investigator: Joanne Rimmer, MA FRCS Imperial College Healthcare NHS Trust
Study Director: Jonny Harcourt, MA FRCS Imperial College Healthcare NHS Trust
  More Information

Responsible Party: Imperial College Healthcare NHS Trust Identifier: NCT01246258     History of Changes
Other Study ID Numbers: JROHH0046
Study First Received: November 22, 2010
Results First Received: October 5, 2015
Last Updated: March 8, 2016

Keywords provided by Imperial College Healthcare NHS Trust:
primary ciliary dyskinesia
vestibular tests

Additional relevant MeSH terms:
Ciliary Motility Disorders
Kartagener Syndrome
Movement Disorders
Central Nervous System Diseases
Nervous System Diseases
Neurologic Manifestations
Signs and Symptoms
Respiratory Tract Diseases
Otorhinolaryngologic Diseases
Bronchial Diseases
Respiratory System Abnormalities
Heart Defects, Congenital
Cardiovascular Abnormalities
Cardiovascular Diseases
Heart Diseases
Congenital Abnormalities
Situs Inversus
Genetic Diseases, Inborn processed this record on May 25, 2017