A Systematic Literature Review of Hypermobility Spectrum Disorders (HSD) and hypermobile Ehlers Danlos Syndrome (hEDS). Effects on Laryngeal Functioning, Voice, and Swallowing.

Objective: This review investigated the effects of HSD and hEDS on Voice. Hypermobility is an asset in the performing arts, with a range of movement of joints beyond the typical range of movement, and a vocal range of up to four octaves. HSD and hEDS are hereditary disorders of the connective tissue, caused by mutations in the genes that encode collagen proteins, resulting in a collagen defect. This defect in collagen leads to joint hypermobility, hyperextensibility of the skin, and tissue fragility, and can be a risk factor for injury. Their effect on multiple systems leading to musculoskeletal, vascular, and gastrointestinal complications, has been well documented. However the effect on the voice has been neglected, this is reflected by a paucity of research in the literature.

Methods/Design: A mixed methods systematic review of the literature was carried out according to the PRISMA 2020 guidelines of multiple databases including Pub Med, Scopus, and Ovid Medline for a comprehensive coverage. Search items involved the keywords: collagen defect, connective tissue, lamina propria, hypermobility spectrum disorders, hypermobile Ehlers-Danlos syndrome, pain, laryngeal symptoms, dysphonia, dysphagia. Studies from 1967-2023 were included in this systematic review.

Results: Laryngeal symptoms reported were; vocal fatigue, pain, cricoarytenoid dislocation, unreliable voice, and dysphonia. Menton (2023) reported an increased prevalence of voice, upper airway, and dysphagia symptoms in hEDS/HSD patients. Symptoms of acid reflux, dysphonia, and dysphagia were reported by Williams (2023). Jeffrey (2021) reported a wide vocal range, vocal fatigue, intermittent hoarseness, unreliable voice, and inability to sing due to pain. Arulanandam et al (2016) observed tightness of closure of the vocal folds or “scissor effect”, reduced mobility of the cricoarytenoid joint, dislocation of the cricoarytenoid joint, and vocal fold paralysis.

Conclusions: HSD and hEDS known for their impact on joint hypermobility, joint dislocation, and musculoskeletal symptoms, also affect the voice significantly. Voice problems are secondary to the collagen defect in the connective tissue. Inclusion of a laryngeal exam in the evaluation of HSD and hEDS, could yield key findings contributing to the final diagnosis. Further research is needed on how the voice is affected by Hypermobility Spectrum Disorders.