Clinical Trial: Otolith Function in Patients With Primary Ciliary Dyskinesia

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Otolith Function in Patients With Primary Ciliary Dyskinesia: a Pilot Study

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 Summary:

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.


Sponsor: Imperial College Healthcare NHS Trust

Current Primary Outcome: Vestibular Evoked Myogenic Potentials (VEMPs) [ Time Frame: one day ]

These are the balance tests that specifically assess the otolith organ.


Original Primary Outcome: The results of the utricular centrifugation test, VEMPs & ocular counter-rolling test [ Time Frame: One session only ]

These are the balance tests that specifically assess the otolith organ. They will then be compared to normative data.


Current Secondary Outcome:

Original Secondary Outcome:

Information By: Imperial College Healthcare NHS Trust

Dates:
Date Received: November 22, 2010
Date Started: September 2010
Date Completion:
Last Updated: March 8, 2016
Last Verified: August 2014