Clinical Trials Logo

Clinical Trial Summary

One in six people in the United Kingdom and over 400 million people worldwide have disabling hearing loss. This figure will double by 2050 as predicted by the World Health Organisation. There is an urgent need to improve our knowledge regarding hearing loss, its underlying mechanisms, optimal diagnostic modalities, reliable and accurate functional and imaging biomarkers. A less-well studied condition associated with progressive hearing loss is infratentorial superficial siderosis (iSS). It results from iron deposition along the surfaces of brain structures which control hearing and balance. It is currently considered uncommon, but may well be under-recognised and therefore under-reported. Despite its severity, our current understanding of its impact on the hearing (auditory) and balance (vestibular) functions is limited, and this has an adverse impact on the treatment offered to these patients. Additionally, iSS patients have been reported to have cognitive impairment yet literature reports of cognitive assessment in iSS are few. The cognitive dysfunction may be specific to iSS or due to progressive hearing impairment or a combination of both, and further studies are required to establish this. Olfaction is also known to be affected in patients with iSS yet is rarely reported in the literature. Due to the significant morbidity and progressive nature, there is a clear need to improve our understanding of the audiovestibular dysfunction resulting from iSS. The aim of this study is to comprehensively assess audiovestibular function in iSS compared to age-related hearing loss and the controls/normative data and as a means to quantify deficits for monitoring disease progression and response to treatment, to assess the impact on the quality of life, to analyse clinically-obtained data (including imaging, cognitive and laboratory data), and correlate these with functional findings in iSS.


Clinical Trial Description

n/a


Study Design


Related Conditions & MeSH terms


NCT number NCT04200664
Study type Observational
Source University College, London
Contact
Status Completed
Phase
Start date February 7, 2020
Completion date October 19, 2021

See also
  Status Clinical Trial Phase
Recruiting NCT05794217 - A Multi-Site Leukopak Repository Providing Annotated Biospecimens for Approved Investigator-Directed Biomedical Research Initiatives
Completed NCT03680365 - Your Voice; Impact of Duchenne Muscular Dystrophy (DMD) on the Lives of Families
Completed NCT03290469 - NICUSeq: A Trial to Evaluate the Clinical Utility of Human Whole Genome Sequencing (WGS) Compared to Standard of Care in Acute Care Neonates and Infants N/A
Not yet recruiting NCT05955794 - Vocal Pattern Assessment as a New Key to Identifying Rare Syndromes N/A
Recruiting NCT04429750 - Intact Cord Resuscitation in CDH N/A
Not yet recruiting NCT04152876 - Functional Genomics of Rare Genetic Diseases: Realization of Innovative Tools With High Diagnostic Power
Recruiting NCT03683966 - MigALastat Therapy Adherence Among FABRY Patients: A Prospective Multicentral Observational Study
Not yet recruiting NCT04319796 - European Registry on Rare Neurological Diseases
Completed NCT02736565 - Pbi-shRNAâ„¢ EWS/FLI1 Type 1 LPX in Subjects With Advanced Ewing's Sarcoma Phase 1
Completed NCT05070988 - Oral Health Related Quality of Life of Patients With Rare Diseases: a Qualitative Approach
Completed NCT03563677 - Dual Guidance Structure for Evaluation of Patients With Unclear Diagnosis in Centers for Rare Diseases N/A
Recruiting NCT05499091 - Functional Study to Indentify Genetic Etiology of Rare Diseases - ORIGIN N/A
Completed NCT03954652 - Whole Genome Trio Sequencing as a Standard Routine Test in Patients With Rare Diseases - "GENOME FIRST APPROACH" N/A
Recruiting NCT05703893 - Investigation of the Neurovegetative Pattern in Patients With Thoracic Aortic Aneurysms (TAA)
Enrolling by invitation NCT04703179 - Rare and Undiagnosed Disease Research Biorepository
Recruiting NCT04024774 - Diagnostic Research in Patients With Rare Diseases -Solving the Unsolved Rare Diseases
Recruiting NCT06343558 - Gait and Balance Impairment in Rare and Very Rare Neurological Diseases
Not yet recruiting NCT06412718 - Validation of Human Drugs Target of Repurposed Drugs and Novel Therapies
Enrolling by invitation NCT04654000 - Rheopheresis as Adjuvant Treatment of Calciphylaxis N/A
Recruiting NCT04651439 - Severe Bullous Drug Eruption and Filgrastim Phase 2/Phase 3