EDS can harm the voice box of patients, affecting their life’s work

Report highlights need for interdisciplinary approach to treatment

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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Some people with Ehlers-Danlos syndrome (EDS) may experience symptoms affecting the larynx (voice box), according to a report of nine patients in the U.S., including six who were singers.

“Recent years have seen an increase in reports on the laryngeal manifestations of Ehlers–Danlos Syndrome,” researchers wrote. “Here, we attempt to view patients with EDS from a holistic lens, highlighting the need for interdisciplinary assessment and management between physicians, [speech-language specialists], and other healthcare professionals to maximize quality of life.”

The report, “Laryngological Symptomatology in Patients with Ehlers–Danlos Syndrome,” was published in The Laryngoscope.

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EDS can cause symptoms in the larynx in some patients

EDS is a group of genetic diseases that affects the connective tissues in the body, leading to symptoms such as more flexible joints, stretchier skin, and sometimes joint dislocations.

While it is rare, EDS can cause symptoms in the larynx. These include a hoarse voice, difficulty speaking, trouble swallowing, and bleeding in the vocal folds, which are the structures that produce sound and protect against choking.

Now, researchers from Case Western Reserve University School of Medicine and the Cleveland Clinic described the cases of nine patients (eight women, one man) who experienced symptoms in the larynx related to EDS.

Their ages ranged from 22 to 50 years, with a mean of 33. Eight patients had hypermobile EDS, the most common type of the disease. Six patients were singers, and one was a mental health counselor, so voice was essential to their work.

The most common complaints were a hoarse or strained voice (eight patients), followed by trouble swallowing (five patients) and laryngospasm, or sudden tightening of the vocal cords (three patients).

Also common among these patients was gastroesophageal reflux disease, which occurs when stomach acid flows back up into the esophagus (the tube connecting the mouth to the stomach), causing heartburn and trouble swallowing.

It may be beneficial to view patients with EDS and laryngological complaints through a holistic lens as many may need interdisciplinary assessment and management.

Seven patients also experienced postural orthostatic tachycardia syndrome, which causes dizziness and a fast heart rate when transitioning from lying down to standing up.

All but one patient underwent videostroboscopy, a procedure used by doctors to look at the vocal cords and larynx in detail. It involves using a long, thin tube with a camera and light to record a slow-motion video.

Of the eight patients who had a hoarse or strained voice, four showed abnormal videostroboscopy findings in the mucosa, or the lining of the larynx. For these four patients, voice was essential to their work.

Three had swelling on both sides of the larynx. One patient had swelling in a vocal fold and had undergone surgery to remove nodules, or growths that formed on the vocal cords. Voice therapy and two rounds of steroid injections mildly improved her voice.

The other patients were diagnosed with muscle tension dysphonia, a voice disorder caused by muscle tightness around the larynx, and improved with voice therapy. However, one patient suddenly lost her voice after 3.5 months of starting voice therapy.

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Patients referred to specialists to manage their specific symptoms

Four patients out of five who had trouble swallowing underwent a test called modified barium swallow, which showed their swallowing function was normal. Two of them got better after swallowing therapy.

The other two had problems related to their digestive system and were referred to a gastroenterologist, a specialist of the esophagus, stomach, and intestines.

The other patient who had trouble swallowing experienced painful “popping” or dislocation of her hyoid (neck) bone when yawning or laughing. She was referred to a speech-language specialist.

Two patients out of the three who had laryngospasm were diagnosed with chronic (long-lasting) cough and a sensitive vagus nerve, a long nerve that carries information between the brain and internal organs. They were treated with triamcinolone, a corticosteroid, nearly every two months.

One patient who had a hoarse or strained voice, trouble swallowing, and laryngospasm was managed successfully with voice and swallowing therapy.

“It may be beneficial to view patients with EDS and laryngological complaints through a holistic lens as many may need interdisciplinary assessment and management,” the researchers wrote.