Laryngeal dysfunction common in hEDS patients, study shows

Hypermobile EDS patients report more voice, upper airway, swallowing problems

Andrea Lobo, PhD avatar

by Andrea Lobo, PhD |

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Patients with hypermobile Ehlers-Danlos syndrome (hEDS) report more voice, upper airway, and swallowing problems than the general population, a study found.

A similar higher prevalence was found in people with hypermobility spectrum disorders (HSD).

That’s according to the study, “Laryngological Complaint Prevalence in Hypermobile Ehlers-Danlos or Hypermobility Spectrum Disorders,” published recently in the journal The Laryngoscope.

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EDS is a group of genetic disorders affecting the connective tissues, which provide structure to joints, skin, blood vessels, and other tissues and organs.  The most common type, hEDS, is characterized by highly mobile joints and soft, fragile skin.

Other tissues also may be affected, leading to muscular, vascular, and digestive symptoms. Also, given the amount of connective tissue in the neck and larynx (voice box), symptoms affecting the voice, the upper airways, and swallowing have been reported in people with EDS.

However, few studies have reported these symptoms in people with hEDS or HSD. A diagnosis of HSD is made when patients have joint hypermobility, but do not fulfill all hEDS criteria.

Aiming to quantify the prevalence of voice, upper airway, and swallowing complaints among these patient populations, researchers at the Ehlers-Danlos Syndrome Clinic in Jacksonville, Florida, analyzed 363 patients who answered the REDCap EDS Questionnaire between July 2020 and July 2022. This tool was used as standard care before the patients’ first appointments at the clinic to determine who needed a referral to laryngology and speech therapy.

Of these participants, 41 (11.3%) were diagnosed with hEDS, 248 (68.3%) with HDS, and 74 (20.4%) were not diagnosed with any of these diseases, being used as controls. Diagnosis was based on the 2017 International Classification.

hEDS and HSD patients report much more laryngeal dysfunction

Significantly more participants with hEDS or HSD reported laryngeal dysfunction than controls (27% vs. 14.9%). Moreover, 33.9% of the patients reported symptoms related to difficulty swallowing, particularly coughing more when eating or drinking, compared to 24.3% of the controls

In addition, 65 patients (22.5%) reported hoarseness that affected their ability to communicate, compared to 20.3% of the controls. Among those affected, most patients (52.3%) said hoarseness was an issue in communication more than two days per month (vs. 46.7% of the controls). Most of these patients (83.9%) reported functional deficits related to voicing, assessed by their Voice Handicap Index (VHI-10) scores. No significant difference was seen in VHI-10 scores between patients and controls.

However, “controls in this study were sick patients that were seen in the EDS Clinic, which may explain why they had many voice, upper airway, and swallowing complaints,” the researchers noted.

Looking at published studies to compare the prevalence of these symptoms to that in the general population, hEDS/HSD patients seen at the clinic had a higher prevalence of hoarseness (22.5% vs. 0.98-6.6%), laryngeal dysfunction (27% vs. 3-23%), and swallowing difficulties (33.9% vs. 2.8-15.2%).

According to the researchers, a major finding in this study was that there were no significant differences in the prevalence of voice, upper airway, or swallowing problems between patients with hEDS and those with HSD.

“Although the 2017 diagnostic criteria distinguish hEDS from HSD, many comorbidities [coexisting conditions] are shared between the two conditions,” they wrote.

“Our data suggest that voice, upper airway, and swallow issues may also be experienced similarly between the conditions. Future studies should examine this question in more detail,” they added. “Additionally, research into appropriate voice, upper airway, and swallow treatment modalities/therapy for patients with hEDS or HSD should also be explored.”