Papillary endolymphatic sac tumor: Catastrophic presentation in a child.

This was a 9-year-old female patient from a rural area, admitted to emergency service due to rapidly progressing deterioration of the state of consciousness. In the clinical examination, she was comatose, with bilateral nonreflective mydriasis, grade IV (House-Brackmann scale) right facial paralysis, absence of right corneal reflex and bilateral extensor motor responses to pain. When asked, the relatives did not report the presence of symptoms before her admission; in fact, they denied that the patient has any symptoms referring to the vestibular-cochlear system, such as vertigo, hypoacusis, hyperacusis, weakness of the facial muscles or instability in walking. The patient presented no other clinical signs that would suggest von Hippel--Lindau (VHL) disease.