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.