Audiovestibular Function in Infratentorial Superficial Siderosis
NCT ID: NCT04200664
Last Updated: 2025-06-10
Study Results
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View full resultsBasic Information
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COMPLETED
11 participants
OBSERVATIONAL
2020-02-07
2021-10-19
Brief Summary
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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.
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Detailed Description
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The aim of the AVISS study has been to address this gap in the knowledge. This study investigates how iSS affects hearing, balance, and even sense of smell by studying three groups of people:
1. People with iSS, who already have hearing and balance problems.
2. People with age-related hearing loss, to compare how hearing declines over time.
3. People with normal hearing, serving as a control group.
The study will take place over three years, using a range of tests to assess hearing and balance:
* Hearing tests like pure tone audiometry, speech-in-noise assessments, and auditory brainstem response.
* Balance tests such as vestibular evaluations, gait assessments, and head impulse testing.
* Olfactory (smell) tests, since iSS might also affect sensory perception. By collecting this data, the study aims to improve how iSS is diagnosed, identify better rehabilitation methods, and explore new treatment options for those affected.
The study is funded by NIHR UCLH Biomedical Research Centre and the Bernice Bibby Research Trust, ensuring experts have the resources needed to make meaningful discoveries. Ultimately, the goal is to help doctors recognize iSS earlier, support affected individuals more effectively and improve their overall quality of life.
Conditions
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Study Design
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CASE_CONTROL
PROSPECTIVE
Study Groups
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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
Hearing assessment
All study participants will undergo hearing tests, including hearing-specific questionnaires
Vestibular/balance assessment
Siderosis group participants will undergo vestibular/balance tests, including gait assessment and balance-specific questionnaires
Quality of life assessment
All study participants will be asked to complete a set of quality of life questionnaires
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
DNA bio-banking
Siderosis group participants will be asked to provide a saliva sample for DNA bio-banking
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)
Hearing assessment
All study participants will undergo hearing tests, including hearing-specific questionnaires
Quality of life assessment
All study participants will be asked to complete a set of quality of life questionnaires
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)
Hearing assessment
All study participants will undergo hearing tests, including hearing-specific questionnaires
Quality of life assessment
All study participants will be asked to complete a set of quality of life questionnaires
Interventions
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Hearing assessment
All study participants will undergo hearing tests, including hearing-specific questionnaires
Vestibular/balance assessment
Siderosis group participants will undergo vestibular/balance tests, including gait assessment and balance-specific questionnaires
Quality of life assessment
All study participants will be asked to complete a set of quality of life questionnaires
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
DNA bio-banking
Siderosis group participants will be asked to provide a saliva sample for DNA bio-banking
Eligibility Criteria
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Inclusion Criteria
* 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
* 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;
18 Years
ALL
Yes
Sponsors
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NIHR UCLH Biomedical Research Centre (BRC)
UNKNOWN
Bernice Bibby Research Trust
UNKNOWN
University College, London
OTHER
Responsible Party
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Principal Investigators
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Doris-Eva Bamiou, Professor
Role: PRINCIPAL_INVESTIGATOR
UCL Ear Institute; UCLH NHS Foundation Trust, UK
David J Werring, Professor
Role: PRINCIPAL_INVESTIGATOR
Stroke Research Centre UCL IoN; UCLH NHS Foundation Trust, UK
Locations
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UCL Ear Institute
London, , United Kingdom
University College London Hospitals NHS Foundation Trust
London, , United Kingdom
Countries
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References
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Wilson D, Chatterjee F, Farmer SF, Rudge P, McCarron MO, Cowley P, Werring DJ. Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. 2017 Mar;81(3):333-343. doi: 10.1002/ana.24850. Epub 2017 Jan 28.
Fearnley JM, Stevens JM, Rudge P. Superficial siderosis of the central nervous system. Brain. 1995 Aug;118 ( Pt 4):1051-66. doi: 10.1093/brain/118.4.1051.
Koeppen AH, Dickson AC, Chu RC, Thach RE. The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol. 1993 Nov;34(5):646-53. doi: 10.1002/ana.410340505.
Kumar N. Superficial siderosis: associations and therapeutic implications. Arch Neurol. 2007 Apr;64(4):491-6. doi: 10.1001/archneur.64.4.491.
IWANOWSKI L, OLSZEWSKI J. The effects of subarachnoid injections of iron-containing substances on the central nervous system. J Neuropathol Exp Neurol. 1960 Jul;19:433-48. doi: 10.1097/00005072-196007000-00010. No abstract available.
Posti JP, Juvela S, Parkkola R, Roine S. Three cases of superficial siderosis of the central nervous system and review of the literature. Acta Neurochir (Wien). 2011 Oct;153(10):2067-73. doi: 10.1007/s00701-011-1116-0. Epub 2011 Aug 7.
Sydlowski SA, Cevette MJ, Shallop J. Superficial siderosis of the central nervous system: phenotype and implications for audiology and otology. Otol Neurotol. 2011 Aug;32(6):900-8. doi: 10.1097/MAO.0b013e31822558a9.
Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G. Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology. 1996 May;38 Suppl 1:S51-6. doi: 10.1007/BF02278119.
Sydlowski SA, Levy M, Hanks WD, Clark MD, Ackley RS. Auditory profile in superficial siderosis of the central nervous system: a prospective study. Otol Neurotol. 2013 Jun;34(4):611-9. doi: 10.1097/MAO.0b013e3182908c5a.
Vibert D, Hausler R, Lovblad KO, Schroth G. Hearing loss and vertigo in superficial siderosis of the central nervous system. Am J Otolaryngol. 2004 Mar-Apr;25(2):142-9. doi: 10.1016/j.amjoto.2003.10.001.
Takeda T, Kawashima Y, Hirai C, Makabe A, Ito T, Fujikawa T, Yamamoto K, Maruyama A, Tsutsumi T. Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System. Otol Neurotol. 2018 Jul;39(6):e468-e474. doi: 10.1097/MAO.0000000000001844.
Pribitkin EA, Rondinella L, Rosenberg Si, Yousem DM. Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia. Am J Otol. 1994 May;15(3):415-8.
Kwartler JA, De La Cruz A, Lo WW. Superficial siderosis of the central nervous system. Ann Otol Rhinol Laryngol. 1991 Mar;100(3):249-50. doi: 10.1177/000348949110000315. No abstract available.
van Harskamp NJ, Rudge P, Cipolotti L. Cognitive and social impairments in patients with superficial siderosis. Brain. 2005 May;128(Pt 5):1082-92. doi: 10.1093/brain/awh487. Epub 2005 Mar 23.
Provided Documents
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Document Type: Study Protocol and Statistical Analysis Plan
Related Links
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WHO global estimates on prevalence of hearing loss
Other Identifiers
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121603
Identifier Type: -
Identifier Source: org_study_id
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