Traditionally called glomus tumors, jugulotympanic paragangliomas 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 catecholamine-secreting.
  
• Few jugulotympanic paragangliomas are malignant.

Diagnosis

A jugulotympanic paraganglioma has such a characteristic otoscopic appearance that there is seldom an indication for a biopsy. A high jugular bulb might be mistaken for the superior pole of jugular paraganglioma; however, its smooth, pale blue margin helps distinguish it from a tumor. Although another middle ear neoplasm can mimic a paraganglioma, there is frequently something in the clinical history or examination that just does not fit that of the more common paraganglioma. Thus far, I have not had to biopsy a middle ear mass that I believed was a paraganglioma in order to formulate a treatment plan. Instead, I have frequently complemented the clinical findings with a radiologic evaluation that might include computed tomography (CT), magnetic resonance imaging (MRI), and angiography. However, not every patient suspected of having a jugulotympanic paraganglioma requires neuroradiologic evaluation. I believe, for example, that a patient with a small tym-panic paraganglioma that can be seen in its entirety requires no additional evaluation. When the margins of a tumor are obscure, however, I do recommend a radiographic examination. The frequency with which improvements are made in imaging technology preclude insistence on one imaging technique over another. From my perspective, the choice of an initial imaging modality should be based on the information needed, the availability of imaging equipment, and the level of knowledge and experience of the consulting radiologist.

I still use high-resolution computed tomography (HRCT) of the cranial base with contrast enhancement as an initial imaging tool. Whether the lesion is a large tympanic paraganglioma or a sizable jugular variant is not therapeutically important to me. I have found that the study gives me a reliable assessment of the integrity of bone in the area of the jugular fossa, the carotid canal, and the facial canal, as well as an estimate of the extent of the neoplasm.

If the information derived from CT is equivocal or incomplete, I then use gadolinium-enhanced MRI for its superior soft tissue contrast resolution. MRI, for example, can distinguish between tumor and a serous or mucoid effusion, fat, muscle, and brain. I also use MRI to screen for synchronous tumors when clinically indicated. Finally, I use this imaging technique to screen for residual or recurrent disease, particularly if the patient has had prior treatment.

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