{"type":"trials","record":{"id":28371,"source_id":"clinicaltrials-gov","external_id":"NCT04200664","title":"Audiovestibular Function in Infratentorial Superficial Siderosis","brief_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.","overall_status":"Completed","phases":[],"study_type":"OBSERVATIONAL","sponsor":"University College, London","enrollment":11,"countries":["United Kingdom"],"start_date":"2020-02-07","completion_date":"2021-10-19","last_update_date":"2025-06-10","source_url":"https://clinicaltrials.gov/study/NCT04200664","editorial_summary":"A registered study indexed because it matched monitored longevity research terms. Registry status: Completed. Registration does not establish safety or effectiveness.","first_seen_at":"2026-09-25T00:17:13.50034+00:00","last_seen_at":"2026-09-25T06:25:04.647+00:00","metadata":{"sex":"ALL","acronym":"AViSS","age_range":"18 Years","comparator":null,"organization":"University College, London","interventions":["Hearing assessment","Vestibular/balance assessment","Quality of life assessment","Olfactory (smell) function testing","DNA bio-banking"],"registry_source":"ClinicalTrials.gov","outcome_measures":["Hearing Evaluation - Pure Tone Audiometry — Baseline visit","Vestibular/Balance Evaluation — Baseline visit","Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes — Baseline visit","Hearing Evaluation - Tympanogram — Baseline","Hearing Evaluation - Otoacoustic Emissions — Baseline","Hearing Evaluation - Listening in Spatialised Sentences Noise Test — Baseline","Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment R…","Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the…","Smell Identification Test — Baseline visit"],"design_description":null,"source_has_results":true},"controlled_terms":["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"],"relevance_confidence":100,"source_quality_score":100,"freshness_score":65,"publication_state":"published","match_explanation":"Abstract contains controlled term: age-related hearing loss.","quality_checked_at":"2026-09-25T06:25:07.732232+00:00","duplicate_cluster_key":"id:nct04200664","duplicate_of_id":null,"evidence_snapshot":{"status":"structured","version":1,"duration":"2020-02-07 to 2021-10-19","comparator":null,"confidence":"structured-source","population":"ALL · 18 Years","provenance":{"duration":"start_date and completion_date","comparator":"registry arm fields","population":"registry eligibility fields","intervention":"registry intervention fields","participants":"enrollment","study_design":"study_type and registry design fields","evidence_stage":"phases","reported_outcome":"not available","outcomes_measured":"registry outcome-measure fields"},"generated_at":"2026-09-25T06:25:04.659Z","intervention":["Hearing assessment","Vestibular/balance assessment","Quality of life assessment","Olfactory (smell) function testing","DNA bio-banking"],"participants":11,"study_design":"OBSERVATIONAL","subject_scope":"Human clinical study registration","evidence_stage":"Phase not reported","safety_context":"Eligibility, adverse-event details, and clinical decisions must be checked in the official registry and with qualified clinicians.","source_support":"Structured registry protocol metadata; no finding-level conclusion is generated.","main_limitation":"This is a study registration. No reusable structured result is available here, so it cannot show whether the intervention worked or was safe.","reported_outcome":null,"outcomes_measured":["Hearing Evaluation - Pure Tone Audiometry — Baseline visit","Vestibular/Balance Evaluation — Baseline visit","Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes — Baseline visit","Hearing Evaluation - Tympanogram — Baseline","Hearing Evaluation - Otoacoustic Emissions — Baseline","Hearing Evaluation - Listening in Spatialised Sentences Noise Test — Baseline","Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment R…","Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the…","Smell Identification Test — Baseline visit"],"regulatory_context":"Trial registration is not regulatory approval and does not establish that an intervention is available."},"clinical_trial_topics":[{"topic_slug":"hearing-aging","is_published":true,"match_reasons":["Abstract contains controlled term: age-related hearing loss.","Source terminology contains: presbycusis.","Abstract supplies longevity context: aging, age-related.","Study type is explicitly identified as OBSERVATIONAL."],"matched_fields":["abstract","controlled terminology","abstract context","study type"],"relevance_score":100,"intelligence_topics":{"name":"Hearing ageing","slug":"hearing-aging"}}],"content_sources":{"name":"ClinicalTrials.gov","homepage_url":"https://clinicaltrials.gov/"}},"canonical_url":"https://www.immortal.life/trials/28371","automation_disclosure":"Generated automatically from cited source metadata. No scientist, clinician, researcher, editor, or human reviewer evaluates this publication before release."}