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Laryngological presentations and patient-reported outcome measures in Ehlers–Danlos syndrome

Published online by Cambridge University Press:  10 December 2021

C M Lam ,
G Wood  and
M A Birchall
C M Lam
Affiliation:
Royal National Throat, Nose and Ear Hospital, London, UK
G Wood
Affiliation:
Royal National Throat, Nose and Ear Hospital, London, UK
M A Birchall*
Affiliation:
Royal National Throat, Nose and Ear Hospital, London, UK
*
Author for correspondence: Prof Martin A Birchall, Royal National Throat, Nose and Ear Hospital, 47–49 Huntley Street, LondonWC1E 6DG, UK E-mail: m.birchall@ucl.ac.uk
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Abstract

Objective

This study aimed to characterise the laryngological presentations of Ehlers–Danlos syndrome and conduct a preliminary exploration of patient-reported outcome measures.

Methods

This paper describes a retrospective case series of patients with Ehlers–Danlos syndrome seen by the senior author between 2005 and 2019. A literature review was conducted to summarise the existing findings.

Results

Twenty-one patients met the inclusion criteria. All reported symptoms were grouped; this showed that swallowing, voice and hyolaryngeal skeletal complex problems were commonest. Patient-reported outcome measures were available for eight patients, which showed large variations in: the Reflux Severity Index (median = 25.5; range = 0–33), Eating Assessment Tool score (median = 21.5; range = 0–35) and Voice Handicap Index (median = 21.5; range = 0–104). Twelve studies met our literature review inclusion criteria, involving at least 91 patients with laryngological presentations of Ehlers–Danlos syndrome.

Conclusion

Ehlers–Danlos syndrome patients experience musculoskeletal issues, which in the throat manifest as hyolaryngeal skeletal complex problems. Future studies with larger patient numbers are required to validate laryngological patient-reported outcome measure tools in Ehlers–Danlos syndrome.

Keywords

Ehlers-Danlos SyndromePatient Reported Outcome MeasuresLarynxConnective Tissue
Type
Main Article
Information
The Journal of Laryngology & Otology , Volume 136 , Issue 10 , October 2022 , pp. 947 - 951
Copyright
Copyright © The Author(s), 2021. Published by Cambridge University Press on behalf of J.L.O. (1984) LIMITED.

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Footnotes

Prof M Birchall takes responsibility for the integrity of the content of the paper

Presented at the virtual Ehlers-Danlos Society ECHO Summit, 2–3 October 2020 (online event).

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