Eagle syndrome: toward a clinical delimitation.

INTRODUCTION: Orofacial and cervical pain are a frequent reason for neurology consultations and may be due to multiple pathological processes. These include Eagle syndrome (ES), a very rare entity whose origin is attributed to calcification of the stylohyoid ligament or elongation of the temporal styloid process. We present a series of five patients diagnosed with ES. METHODS: We describe the demographic and clinical characteristics and response to treatment of 5 patients who attended the headache units of 2 tertiary hospitals for symptoms compatible with Eagle syndrome. RESULTS: The patients were 3 men and 2 women aged between 24 and 51, presenting dull, intense pain, predominantly in the inner ear and the ipsilateral tonsillar fossa. All patients had chronic, continuous pain in the temporal region, with exacerbations triggered by swallowing. Four patients had previously consulted several specialists at otorhinolaryngology departments; one had been prescribed antibiotics for suspected Eustachian tube inflammation. In all cases, the palpation of the tonsillar fossa was painful. Computed tomography scans revealed an elongation of the styloid process and/or calcification of the stylohyoid ligament in 3 patients. Four patients improved with neuromodulatory therapy (duloxetine, gabapentin, pregabalin) and only one required surgical excision of the styloid process. CONCLUSIONS: Eagle syndrome is a rare and possibly underdiagnosed cause of craniofacial pain. We present 5 new cases that exemplify both the symptoms and the potential treatments of this entity.