Laryngeal Manifestations of Ehlers-Danlos Syndrome
Abstract
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.
How strong is the evidence?
Evidence Hub reads this as a case series, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
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