Laryngeal Manifestations of Ehlers-Danlos Syndrome

Objectives: The identification of voice and airway manifestations of Ehlers-Danlos Syndrome (EDS), diagnoses, and potential treatment modalities.

Study design: Single institution retrospective case series.

Methods: We examined all patients presenting to our institution over a span of 10 years with a history of EDS or who were subsequently diagnosed with EDS after their evaluation. Demographic and clinical data were collected.

Results: Four patients were identified with an underlying diagnosis of EDS. All four patients were heavy voice users. All four patients had history and/or stroboscopy findings suggesting vocal hyperfunction, which we suspect is due to EDS-related hypermobility of the cricoarytenoid joint or fragility of the superficial lamina propria. Two patients also had respiratory symptoms - one with respiratory muscle weakness and sensation loss and one with inducible laryngeal obstruction. All patients were treated with voice therapy with subsequent improvement in their symptoms.

Conclusions: Patients with EDS may present to laryngology clinics with symptoms of dysphonia or dyspnea secondary to their underlying condition. Voice therapy is a low-risk and potentially beneficial treatment in this patient population.