Clinical Features

Traditionally called glomus tumors, jugulotympanic paragan-gliomas are second only to vestibular schwannomas as the most common neoplasms of the temporal bone. Replicating normal paraganglia, the tumors are characterized histologically by a rather vascular framework that surrounds nests of specialized cells.1 Paragangliomas that arise in the jugular fossa are more common than those that originate within the middle ear. I have found the following clinical characteristics useful in making diagnostic or management decisions:

• Paragangliomas grow very slowly and usually have an extended clinical course.

• The most common presenting symptoms are a hearing loss and pulse-synchronous tinnitus in the affected ear.

• A middle ear mass with a vascular hue is almost always detected on a microscopic examination of the affected ear.

• Other symptoms and signs include progressive facial nerve paralysis and isolated or combined neuropathies of the basal cranial nerves.

• A person with a family history of paraganglioma is at a higher risk of having multicentric tumors.

• Less than 1% of jugular paragangliomas are functional or cat-echolamine-secreting.

• Few jugulotympanic paragangliomas are malignant.

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