Otolith Function in Patients With Primary Ciliary Dyskinesia

Completed

• Conditions: Primary Ciliary Dyskinesia
• Interventions: Other: Vestibular evoked myogenic potentials (VEMPs); Other: Utricular centrifugation test

• Registration Number: NCT01246258
• Lead Sponsor: Imperial College Healthcare NHS Trust

Brief Summary

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.

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.

Study Timeline

• Study Start: 2010-09
• Study First Submitted: 2010-11-22
• Study First Submitted QC: 2010-11-22
• Study First Posted: 2010-11-23
• Primary Completion: 2013-12
• Study Completion: 2013-12
• Status Verified: 2014-08
• Results First Submitted: 2015-10-05
• Results First Submitted QC: 2016-03-08
• Last Update Submitted: 2016-03-08
• Results First Posted: 2016-04-06
• Last Update Posted: 2016-04-06

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 5

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)

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Test group	Vestibular evoked myogenic potentials (VEMPs)	Standard tests of balance function
Test group	Utricular centrifugation test	Standard tests of balance function

Primary Outcome Measures

Name	Time	Method
Vestibular Evoked Myogenic Potentials (VEMPs)	one day	These are the balance tests that specifically assess the otolith organ.

Trial Locations

Locations (1)

Charing Cross Hospital; 🇬🇧 London, United Kingdom