Vernet syndrome: intracranial extension of a slow-growing mass

Vernet syndrome, often referred to as jugular foramen syndrome, is a rare clinical entity characterised by a set of signs and symptoms caused by dysfunction of IX, X and XI cranial nerves. Although paraganglioma of the head and neck is the most frequent aetiology, it may also be caused by meningioma, VIII cranial nerve schwannoma, pontocerebellar cistern metastases, head and neck trauma, infections and very rarely by cholesteatoma which extends to the petrous apex. The authors describe a case of a patient with a jugulotympanic paraganglioma in which evolution ends up in Vernet syndrome. The patient preferred a 'wait and scan' strategy. With the lack of data available to develop an unequivocal algorithm for paraganglioma management, we always consider not only age but also comorbidities, prior treatment and progression of the lesion. Each case has to be addressed individually and treatment should be discussed in detail with every patient.