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Health-Related Quality of Life in Adults With Classical Infratentorial Superficial Siderosis: A Cross-sectional Study

Kharytaniuk, Natallia; Mazaheri, Amir Ala; Pavlou, Menelaos; Werring, David; Bamiou, Doris-Eva; (2022) Health-Related Quality of Life in Adults With Classical Infratentorial Superficial Siderosis: A Cross-sectional Study. Neurology , 99 (19) e2201-e2211. 10.1212/WNL.0000000000201115. Green open access

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Abstract

BACKGROUND AND OBJECTIVES: Infratentorial superficial siderosis (iSS) is a rare but disabling neurological condition characterised by progressive hearing loss, balance and mobility problems. The functional decline in these neurological domains with iSS progression is likely to adversely impact health-related quality of life (HRQoL). We studied HRQoL of adults with iSS using two common generic HRQoL measures (Health Utilities Index Mark III (HUI3) and EuroQoL EQ5D (5 Level) to determine the most impacted domains and evaluate the association between HRQoL scores and disease duration. METHODS: This observational study was an anonymous online survey. Following institutional Research Ethics Committee approval, we contacted dedicated international organisations, charities and patient-groups identified through online searches, social media and collaborative networks, to distribute the study information and study link, inviting their members diagnosed with iSS to participate. Participation required access to a digital device connected to the internet, confirmation of eligibility (aged ≥18 years and previously diagnosed with iSS) and informed consent to participate in the survey, which included study-specific questions (demographics, iSS, hearing) and HRQoL questionnaires. Survey responses were captured by the Research Electronic Data Capture (REDCap) survey software and analysed using the SPSS statistical package. Linear regression analysis was performed to investigate the association between HRQoL scores and disease duration. RESULTS: Of fifty participants,60% were male; the median (interquartile range, IQR) age was 60 (15) years. The median (IQR) multi-attribute scores for HUI3 and EQ5D were 0.36 (0.53) and 0.64 (0.33), respectively. The most frequently affected domains (moderate or worse category) were hearing (64%), and pain (48%) for HUI3, and mobility (54%) and pain (50%) for EQ5D. There was a weak association between disease duration and multi-attribute scores for HUI3 (R=0.353; adjusted R2=0.096; b=-0.008; p=0.047) but not EQ5D. DISCUSSION: Our findings demonstrate low HRQoL scores which capture low functional status in several domains typically affected in iSS, suggesting that iSS has a major adverse impact on quality of life in multiple functional domains. Measures of HRQoL in iSS should be included in clinical and research settings, including treatment trials.

Type: Article
Title: Health-Related Quality of Life in Adults With Classical Infratentorial Superficial Siderosis: A Cross-sectional Study
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1212/WNL.0000000000201115
Publisher version: https://doi.org/10.1212/WNL.0000000000201115
Language: English
Additional information: Copyright © 2022 The Author(s). This is an open access article distributed under the terms of the Creative Commons Attribution License 4.0 (CC BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
UCL classification: UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences
UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Maths and Physical Sciences > Dept of Statistical Science
UCL > Provost and Vice Provost Offices > UCL BEAMS
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > The Ear Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
URI: https://discovery.ucl.ac.uk/id/eprint/10154884
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