HISTORY AND CLINICAL FINDINGS: A 73-year-old woman was admitted because of vertigo of recent onset with a tendency to fall down and progressive hearing impairment with tinnitus over the last 2 years. Neurological examination also revealed right recurrent nerve paresis, facial hemispasm and lingual atrophy, pointing to a lesion involving cranial nerves VII, VIII, IX, X and XII. She was found to have spontaneous nystagmus to the left, due to peripheral vestibular function deficit, without otoscopic abnormalities. She was in atrial fibrillation with a blood pressure of 140/80 mm Hg. The suspected cause was a hormonally active glomus jugulare tumour with intermittent hypertension and involvement of several cranial nerves. INVESTIGATIONS: Repeatedly measured plasma and urinary catecholamine concentration was normal. Neuroradiology showed a contrast-rich lesion close to the jugular vein and the hypoglossal nerve, as well as a tumour in the left retromandibular fossa with displacement of the left internal carotid artery. The suspected cause of these findings was a neurologically asymptomatic left carotid body tumour with multiple cranial nerve deficits (VII, VIII, IX and XII) due to their compression at the base of the skull. No abnormal catecholamine activity could be demonstrated. TREATMENT AND COURSE: After complete excision of the right carotid body there were no further hypertensive crises. Later on the left carotid body tumour was embolised because it had continued to grow. CONCLUSION: The repeated hypertensive crises were probably caused by absent blood pressure regulation, the result of destruction of the afferent fibres. This destruction was due to compression of the hypoglossal nerve by the right jugular glomus, at the same time as the contralateral carotid body had been destroyed by tumour.