University College, London
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.
Siderosis
Neurological Disorder
Rare Diseases
Age Related Hearing Loss
Presbycusis
Hearing assessment
Vestibular/balance assessment
Quality of life assessment
Olfactory (smell) function testing
DNA bio-banking
Study Type : | Observational |
Estimated Enrollment : | 90 participants |
Official Title: | Clinical and Imaging Biomarkers of Audiovestibular Function in Infratentorial Superficial Siderosis |
Actual Study Start Date : | February 7, 2020 |
Estimated Primary Completion Date : | October 19, 2021 |
Estimated Study Completion Date : | October 19, 2021 |
Information not available for Arms and Intervention/treatment
Ages Eligible for Study: | 18 Years |
Sexes Eligible for Study: | All |
Accepts Healthy Volunteers: | Accepts Healthy Volunteers |
Want to participate in this study, select a site at your convenience, send yourself email to get contact details and prescreening steps.
Not yet recruiting
UCL Ear Institute
London, United Kingdom,
Not yet recruiting
University College London Hospitals NHS Foundation Trust
London, United Kingdom,