| Primary | Hearing Evaluation - Pure Tone Audiometry | Clinical audiological (hearing) tests were performed to assess hearing function. The hearing tests included psychophysical, physiological and electrophysiological measures, (pure-tone audiometry, tympanometry, auditory brainstem responses and acoustic reflexes). The tests were perfomed in line with the procedure guidelines by the British Society of Audiology (www.thebsa.org.uk). The mean value of pure-tone audiometric thresholds for both ears measured in decibel hearing level (as described in the guidelines by the British Society of Audiology) was calculated and reported. Other parameters were reported as normal or abnormal (in at least one ear) or inconclusive. | Participants with known diagnosis of infratentorial superficial siderosis. | Posted | | Mean | Standard Deviation | decibel hearing level | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
| | | Title | Denominators | Categories |
|---|
| | |
| |
| Primary | Vestibular/Balance Evaluation | Clinical vestibular and balance tests were performed to assess vestibular/balance function. | Vestibular tests included video head impulse test, oculomotor and rotatory chair testing using video or electronystagmography; bithermal calorics, ocular and cervical vestibular evoked myogenic potentials. The results are presented as normal if bilateral or unilateral normal findings; | Posted | | Count of Participants | | Participants | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
| |
| Primary | Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes | Clinical audiological (hearing) tests were performed to assess hearing function. The hearing tests included psychophysical, physiological and electrophysiological measures, (pure-tone audiometry, tympanometry, auditory brainstem responses and acoustic reflexes). The tests were perfomed in line with the procedure guidelines by the British Society of Audiology (www.thebsa.org.uk). The mean value of pure-tone audiometric thresholds for both ears measured in decibel hearing level (as described in the guidelines by the British Society of Audiology) was calculated and reported. Other parameters were reported as normal or abnormal (in at least one ear) or inconclusive. | Results for psychophysical, physiological/electrophysiological measures (units: participants); normal if bilateral or unilateral normal findings; Auditory brainstem responses were recorded as normal, abnormal or inconclusive | Posted | | Count of Participants | | Participants | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
| |
| Primary | Hearing Evaluation - Tympanogram | Clinical audiological (hearing) tests were performed to assess hearing function. The hearing tests included psychophysical, physiological and electrophysiological measures, (pure-tone audiometry, tympanometry, auditory brainstem responses and acoustic reflexes). The tests were perfomed in line with the procedure guidelines by the British Society of Audiology (www.thebsa.org.uk). The mean value of pure-tone audiometric thresholds for both ears measured in decibel hearing level (as described in the guidelines by the British Society of Audiology) was calculated and reported. Other parameters were reported as normal or abnormal (in at least one ear) or inconclusive. | | Posted | | Count of Participants | | Participants | | Baseline | | | | ID | Title | Description |
|---|
| OG000 | 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 |
|
| Primary | Hearing Evaluation - Otoacoustic Emissions | Clinical audiological (hearing) tests were performed to assess hearing function. The hearing tests included psychophysical, physiological and electrophysiological measures, (pure-tone audiometry, tympanometry, auditory brainstem responses and acoustic reflexes). The tests were perfomed in line with the procedure guidelines by the British Society of Audiology (www.thebsa.org.uk). The mean value of pure-tone audiometric thresholds for both ears measured in decibel hearing level (as described in the guidelines by the British Society of Audiology) was calculated and reported. Other parameters were reported as normal or abnormal (in at least one ear) or inconclusive. | | Posted | | Count of Participants | | Participants | | Baseline | | | | ID | Title | Description |
|---|
| OG000 | 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 |
|
| Primary | Hearing Evaluation - Listening in Spatialised Sentences Noise Test | Clinical audiological (hearing) tests were performed to assess hearing function. The hearing tests included psychophysical, physiological and electrophysiological measures, (pure-tone audiometry, tympanometry, auditory brainstem responses and acoustic reflexes). The tests were perfomed in line with the procedure guidelines by the British Society of Audiology (www.thebsa.org.uk). The mean value of pure-tone audiometric thresholds for both ears measured in decibel hearing level (as described in the guidelines by the British Society of Audiology) was calculated and reported. Other parameters were reported as normal or abnormal (in at least one ear) or inconclusive. Cameron S, Dillon H. The listening in spatialized noise-sentences test (LISN-S): comparison to the prototype LISN and results from children with either a suspected (central) auditory processing disorder or a confirmed language disorder. J Am Acad Audiol. 2008 May;19(5):377-91. | | Posted | | Count of Participants | | Participants | | Baseline | | | | ID | Title | Description |
|---|
| OG000 | 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 |
|
| Secondary | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Siderosis group participants underwent formal imaging procedures as part of their clinical care pathway. The imaging results were reviewed with the results obtained from hearing and vestibular/balance assessments. All participants had MRI Brain, as part of their clinical care pathway; susceptibility-weighted images were reviewed to identify the appearance of superficial siderosis, in keeping with the radiological diagnostic criteria (Reference: Wilson et al, 2017). | | Posted | | Count of Participants | | Participants | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
| |
| Secondary | Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the Hearing and Balance Assessments | Siderosis group participants underwent a formal neuro-cognitive assessment as part of their clinical care pathway. The results of this clinical assessment were reviewed to determine if an association exists between the cognitive assessment results and the results obtained from hearing and vestibular/balance assessments performed during the study. A formal neuro-cognitive assessment was performed using the Wechsler Adult Intelligence Scale test. Cognitive impairment in a domain was defined as scoring at or below the fifth percentile on any one test in that domain. | | Posted | | Count of Participants | | Participants | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
|
| Secondary | Smell Identification Test | University of Pennsylvania Smell Identification Test (UPSIT, British version) was performed (siderosis group participants) to assess their olfactory function. UPSIT is a forced-choice 40-item self-administered test. The scores are calculated using manufacturer's answer key as the sum of correct answers (maximum best = 40, minimum score 6; anosmia <19(males, females); normosmia >33 (males) and >34 (females)), and compared to the age- and gender-matched norms. The scores are also grouped into categories (ranging from normosmia to anosmia), with higher scores indicating better olfactory function. | | Posted | | Mean | Standard Deviation | UPSIT score | | Baseline visit | | | | ID | Title | Description |
|---|
| OG000 | 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 |
| |