RESUMEN
Background - Approximately 2% of patients admitted to the emergency department present with headache, which is often associated with vomiting, ocular pain, and earache. In rare cases, the presence of an abnormal communication between a cavernous sinus and the carotid arterial system that creates a carotid cavernous fistula is the main cause of these symptoms. Case presentation - A 32-year-old woman presented at the emergency department with unilateral headache associated with earache on the same side, and pulsating tinnitus. On examination, we observed unusual appearance of our patient (small stature, unusually visible skin, lobeless ears). In the first 5 hours of our observation no neurological symptoms had been present, but after a severe vomiting, exophthalmos, subconjunctival suffusion and moderate ptosis developed. First, regarding the initial general symptoms, otorhinolaryngologist assessed the patient, and did not find any abnormality. Further, we ordered computed tomography and consulted a neurologist. Despite of the negative results we continued the observation because her symptoms did not improve. After appearance of neurological symp-toms, carotid cavernous fistula was suspected. Magnetic resonance imaging and ophthalmologist consultation verified the diagnosis. For therapy, she was transferred to interventional neuroradiology. Because of the unusual appearance and carotic cavernous fistula, we ordered genetic examination. This indicated the presence of Ehlers-Danlos syndrome type IV in the background. The first major manifestation of the syndrome was observed at our department. Conclusions - Carotid cavernous fistula is an uncommon diagnosis in the emergency department; however, the early recognition of symptoms and early treatment can prevent further consequences of this potentially severe condition.