Audiovestibular Function in Infratentorial Superficial Siderosis

Study Purpose

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.

Recruitment Criteria

Accepts Healthy Volunteers

Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms

Yes
Study Type

An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes.


An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes.


Searching Both is inclusive of interventional and observational studies.

Observational
Eligible Ages 18 Years and Over
Gender All
More Inclusion & Exclusion Criteria

Inclusion Criteria:

  • - Siderosis group: adults (male and female) of 18+ years old with a known diagnosis of iSS (defined using standardised radiological criteria) confirmed by a consultant neurologist with expertise in this condition at University College London Hospitals National Health Service (NHS) Foundation Trust - Age-related hearing loss (ARHL) group: adults (male and female) of 18+ years old with ARHL - Control group: adults (male and female) of 18+ years old with no previous diagnosis of hearing loss or no known neurological disorder (including iSS) that affects hearing, with the aim to recruit such participants of 50 years of age and above; however, should difficulty with the recruitment of such participants arise, participants of 18 years of age and above will be invited to participate in the study.

Exclusion Criteria:

- All groups: individuals younger than 18 years old; individuals with a physical or mental impairment that prevents the potential participant from giving informed consent or undergoing the hearing and/or vestibular assessment; - Siderosis group: individuals with no prior diagnosis of iSS - Age-related hearing loss (ARHL) group: individuals with no previous diagnosis of ARHL or with a diagnosis of hearing loss of aetiology other than age-related; individuals with a history of exposure to high-intensity noise or ototoxic drugs or evidence of middle ear disease/dysfunction or family history of non age-related hearing loss; - Control group: individuals with a known history of hearing loss (of any cause) or with a known neurological disorder that affects their hearing; individuals with history of exposure to high-intensity noise or ototoxic drugs or evidence of middle ear disease or family history of non age-related hearing loss;

Trial Details

Trial ID:

This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.

NCT04200664
Phase

Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans.

Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data.

Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs.

Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.

Lead Sponsor

The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.

University College, London
Principal Investigator

The person who is responsible for the scientific and technical direction of the entire clinical study.

Doris-Eva Bamiou, ProfessorDavid J Werring, Professor
Principal Investigator Affiliation The Ear Institute, University College London; Department of Neuro-otology, University College London Hospitals NHS Foundation Trust, UKDepartment of Brain Repair and Rehabilitation, Stroke Research Centre, Institute of Neurology, University College London; Department of Neurology, University College London Hospitals NHS Foundation Trust, UK
Agency Class

Category of organization(s) involved as sponsor (and collaborator) supporting the trial.

OtherOtherOther
Overall Status Recruiting
Countries United Kingdom
Conditions

The disease, disorder, syndrome, illness, or injury that is being studied.

Siderosis, Neurological Disorder, Rare Diseases, Age Related Hearing Loss, Presbycusis
Study Website: View Trial Website
Arms & Interventions

Arms

: Siderosis (iSS) group

participants with a known diagnosis of infratentorial superficial siderosis (defined using standardised radiological criteria) confirmed by a consultant neurologist with expertise in this condition at University College London Hospitals National Health Service (NHS) Foundation Trust

: Age-related hearing loss (ARHL) group

participants with age-related hearing loss (as identified from participant's clinical history and examination, with hearing thresholds confirmed on a pure-tone audiogram)

: Control group

participants with no known or previously reported hearing loss (as identified from participant's clinical history and examination, with hearing thresholds confirmed on a pure-tone audiogram)

Interventions

Diagnostic Test: - Hearing assessment

All study participants will undergo hearing tests, including hearing-specific questionnaires

Diagnostic Test: - Vestibular/balance assessment

Siderosis group participants will undergo vestibular/balance tests, including gait assessment and balance-specific questionnaires

Other: - Quality of life assessment

All study participants will be asked to complete a set of quality of life questionnaires

Diagnostic Test: - Olfactory (smell) function testing

Siderosis group participants will undergo a formal smell identification testing by means of self-administered smell chart to identify the revealed scents

Genetic: - DNA bio-banking

Siderosis group participants will be asked to provide a saliva sample for DNA bio-banking

Contact a Trial Team

If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.

International Sites

UCL Ear Institute, London, United Kingdom

Status

Recruiting

Address

UCL Ear Institute

London, ,

Site Contact

Natallia Kharytaniuk, Dr

n.kharytaniuk@ucl.ac.uk

+447539465486

London, United Kingdom

Status

Recruiting

Address

University College London Hospitals NHS Foundation Trust

London, ,

Site Contact

Natallia Kharytaniuk, Dr

n.kharytaniuk@ucl.ac.uk

+447539465486

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