Age Related Hearing Loss, Neurological Disorder, Presbycusis, Rare Diseases, Siderosis
Conditions
Keywords
Superficial siderosis, infratentorial, Nervous system diseases, Hearing loss, Hearing tests, Auditory pathways, Vestibulocochlear nerve, Ataxia, Vestibular function tests, Biomarkers, Central nervous system
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.
Detailed description
This study focuses on a rare condition called infratentorial superficial siderosis (iSS)-a disease where iron gradually accumulates in the superficial layers of the brain and spinal cord. Over time, this buildup can damage the nerves responsible for hearing and balance amongst others, leading to symptoms like progressive hearing loss, dizziness, and problems with coordination. iSS has not been well studied in the past. The hearing and balance function in persons with iSS has been described as variable - meaning, there has not been a specific pattern of hearing loss or imbalance or dizziness that can be associated with iSS. The studies to date have been with small groups of patients but mainly case reports. 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.
Interventions
DIAGNOSTIC_TESTHearing assessment
All study participants will undergo hearing tests, including hearing-specific questionnaires
DIAGNOSTIC_TESTVestibular/balance assessment
Siderosis group participants will undergo vestibular/balance tests, including gait assessment and balance-specific questionnaires
OTHERQuality of life assessment
All study participants will be asked to complete a set of quality of life questionnaires
DIAGNOSTIC_TESTOlfactory (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
GENETICDNA bio-banking
Siderosis group participants will be asked to provide a saliva sample for DNA bio-banking
Sponsors
NIHR UCLH Biomedical Research Centre (BRC)
Bernice Bibby Research Trust
University College, London
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
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;
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Hearing Evaluation - Listening in Spatialised Sentences Noise Test | Baseline | 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. |
| Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes | Baseline visit | 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. |
| Hearing Evaluation - Tympanogram | Baseline | 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. |
| Hearing Evaluation - Otoacoustic Emissions | Baseline | 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. |
| Hearing Evaluation - Pure Tone Audiometry | Baseline visit | 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. |
| Vestibular/Balance Evaluation | Baseline visit | Clinical vestibular and balance tests were performed to assess vestibular/balance function. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the Hearing and Balance Assessments | Baseline visit | 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. |
| Smell Identification Test | Baseline visit | 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. |
| Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Baseline visit | 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). |
Countries
United Kingdom
Participant flow
Recruitment details
Of the 90 anticipated participants, only 11 participants were recruited to the study - into the Siderosis group. Participants were not recruited into the non siderosis groups (ie Age-Related Hearing Loss group and Control group) due COVID19 restrictions at the time of the study, which impacted significantly on recruitment opportunities and dramatically reduced the participant number in the study.
Pre-assignment details
There were limited opportunities to recruit participants into study, (we anticipated 30 participants in each group). The apparent discrepancy between the anticipated recruitment number and the actual number of participants across all groups was due to COVID-19 pandemic and social restrictions placed. Subsequent easing off of the restrictions allowed to recruit 11 participants (from November 2019 to July 2021) but study-related time constraints did not allow to proceed with further recruitment.
Participants by arm
| Arm | Count |
|---|---|
| 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 | 10 |
| Total | 10 |
Withdrawals & dropouts
| Period | Reason | FG000 | FG001 | FG002 |
|---|---|---|---|---|
| Overall Study | Withdrawal by Subject | 1 | 0 | 0 |
Baseline characteristics
| Characteristic | Siderosis (iSS) Group | — |
|---|---|---|
| Age, Customized | 52.5 years STANDARD_DEVIATION 14.5 | — |
| Race and Ethnicity Not Collected | — | — Participants |
| Sex: Female, Male Female | 2 Participants | — |
| Sex: Female, Male Male | 8 Participants | — |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 0 / 11 |
| other Total, other adverse events | 0 / 11 |
| serious Total, serious adverse events | 0 / 11 |
Outcome results
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.
Time frame: Baseline visit
Population: 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
| Arm | Measure | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Siderosis (iSS) Group | Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes | Abnormal | 5 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes | Inconclusive | 2 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Auditory Brainstem Responses/Acoustic Reflexes | Not performed | 4 Participants |
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.
Time frame: Baseline
| Arm | Measure | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Siderosis (iSS) Group | Hearing Evaluation - Listening in Spatialised Sentences Noise Test | Abnormal | 4 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Listening in Spatialised Sentences Noise Test | Normal | 1 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Listening in Spatialised Sentences Noise Test | Not tested | 6 Participants |
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.
Time frame: Baseline
| Arm | Measure | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Siderosis (iSS) Group | Hearing Evaluation - Otoacoustic Emissions | Present | 3 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Otoacoustic Emissions | Absent | 3 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Otoacoustic Emissions | Not performed | 5 Participants |
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.
Time frame: Baseline visit
Population: Participants with known diagnosis of infratentorial superficial siderosis.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Siderosis (iSS) Group | Hearing Evaluation - Pure Tone Audiometry | 57.3 decibel hearing level | Standard Deviation 39.9 |
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.
Time frame: Baseline
| Arm | Measure | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Siderosis (iSS) Group | Hearing Evaluation - Tympanogram | Abnormal | 0 Participants |
| Siderosis (iSS) Group | Hearing Evaluation - Tympanogram | Normal | 11 Participants |
Primary
Vestibular/Balance Evaluation
Clinical vestibular and balance tests were performed to assess vestibular/balance function.
Time frame: Baseline visit
Population: 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;
| Arm | Measure | Group | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|---|
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electronystagmography (oculomotor; rotatory chair) - peripheral vestibular assessment | Not tested | 0 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video head impulse test | Abnormal | 6 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video head impulse test | Normal | 1 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video head impulse test | Not tested | 3 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electro-nystagmography (oculomotor; rotatory chair) - central vestibular assessment | Abnormal | 9 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electro-nystagmography (oculomotor; rotatory chair) - central vestibular assessment | Normal | 1 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electro-nystagmography (oculomotor; rotatory chair) - central vestibular assessment | Not tested | 0 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electronystagmography (oculomotor; rotatory chair) - peripheral vestibular assessment | Abnormal | 7 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Video- electronystagmography (oculomotor; rotatory chair) - peripheral vestibular assessment | Normal | 3 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Bithermal calorics | Abnormal | 1 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Bithermal calorics | Normal | 2 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | Bithermal calorics | Not tested | 7 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | ocular Vestibular Evoked Myogenic Potentials | Abnormal | 0 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | ocular Vestibular Evoked Myogenic Potentials | Normal | 9 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | ocular Vestibular Evoked Myogenic Potentials | Not tested | 1 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | cervical Vestibular Evoked Myogenic Potentials | Abnormal | 3 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | cervical Vestibular Evoked Myogenic Potentials | Normal | 7 Participants |
| Siderosis (iSS) Group | Vestibular/Balance Evaluation | cervical Vestibular Evoked Myogenic Potentials | Not tested | 0 Participants |
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).
Time frame: Baseline visit
| Arm | Measure | Group | Category | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|---|
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Superior cerebellar vermis | Hypointense signal consistent with superficial siderosis observed | 10 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Superior cerebellar vermis | Hypointense signal consistent with superficial siderosis not observed | 0 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Cerebellar folia | Hypointense signal consistent with superficial siderosis observed | 10 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Cerebellar folia | Hypointense signal consistent with superficial siderosis not observed | 0 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Midbrain | Hypointense signal consistent with superficial siderosis observed | 10 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Midbrain | Hypointense signal consistent with superficial siderosis not observed | 0 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Pons | Hypointense signal consistent with superficial siderosis observed | 9 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Pons | Hypointense signal consistent with superficial siderosis not observed | 1 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Medulla | Hypointense signal consistent with superficial siderosis observed | 9 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Medulla | Hypointense signal consistent with superficial siderosis not observed | 1 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | 8th cranial nerve (unilateral or bilateral) | Hypointense signal consistent with superficial siderosis observed | 9 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | 8th cranial nerve (unilateral or bilateral) | Hypointense signal consistent with superficial siderosis not observed | 1 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Supratentorial extension | Hypointense signal consistent with superficial siderosis observed | 10 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Supratentorial extension | Hypointense signal consistent with superficial siderosis not observed | 0 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Sylvian fissures | Hypointense signal consistent with superficial siderosis observed | 9 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Imaging (Siderosis Group Participants) and the Hearing and Balance Assessment Results | Sylvian fissures | Hypointense signal consistent with superficial siderosis not observed | 1 Participants |
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.
Time frame: Baseline visit
| Arm | Measure | Group | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|---|
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the Hearing and Balance Assessments | Participants who underwent neurocognitive assessment | 7 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the Hearing and Balance Assessments | Executive function abnormal | 5 Participants |
| Siderosis (iSS) Group | Correlation Between Clinically Obtained Results of Cognitive Function Assessment (Siderosis Group Participants) and the Hearing and Balance Assessments | Visual memory abnormal | 5 Participants |
p-value: 0.176Kendall tau-b
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.
Time frame: Baseline visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Siderosis (iSS) Group | Smell Identification Test | 25.5 UPSIT score | Standard Deviation 7.8 |