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Glomus jugulare tumor


Definition:

A glomus jugulare tumor is a tumor of a part of the temporal bone (which involves the middle and inner ear structures) in the skull. This tumor can affect the ear, upper neck, base of the skull, and the surrounding blood vessels and nerves.

Alternative Names:

Paraganglioma - glomus jugulare

Causes:

A glomus jugulare tumor grows in the temporal bone of the skull, in an area called the jugular foramen. The jugular foramen is also where the jugular vein and several important nerves exit the skull.

This area contains nerve fibers, called glomus bodies, which normally respond to changes in body temperature or blood pressure.

These tumors usually occur later in life, around age 60 or 70, but they can appear at any age. The cause of a glomus jugulare tumor is unknown. Usually, there are no known risk factors. Glomus tumors have been associated with changes (mutations) in a gene responsible for the enzyme succinate dehydrogenase (SDHD).

Symptoms:

Symptoms may include:

  • Difficulty swallowing (dysphagia)
  • Dizziness
  • Hearing problems or loss
  • Hearing pulsations in the ear
  • Hoarseness
  • Pain
  • Weakness or loss of movement in the face (facial nerve palsy)
Exams and Tests:

Glomus jugulare tumors are diagnosed by physical examination and imaging tests, including:

Treatment:

Glomus jugulare tumors are rarely cancerous and do not tend to spread to other parts of the body. However, treatment may be needed to relieve symptoms. The main treatment is surgery. Surgery is complex and is usually done by a neurosurgeon, head and neck surgeon, and ear surgeon (neurotologist).

In some cases, a procedure called embolization is performed before surgery to prevent the tumor from bleeding too much during surgery.

After surgery, radiation therapy may be used to treat any part of the tumor that could not be removed completely.

Some glomus tumors can be treated with stereotactic radiosurgery.

Outlook (Prognosis):

Patients who have surgery or radiation tend to do well. More than 90% of those with glomus jugulare tumors are cured.

Possible Complications:

The most common complications are due to nerve damage, which may be caused by the tumor itself or damage during surgery. Nerve damage can lead to:

  • Change in voice
  • Difficulty swallowing
  • Hearing loss
  • Paralysis of the face
When to Contact a Medical Professional:

Call your health care provider if you:

  • Are having difficulty with hearing or swallowing
  • Develop pulsations in your ear
  • Notice a lump in your neck
  • Notice any problems with the muscles in your face
References:

Rucker JC. Cranial neuropathies. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Neurology in Clinical Practice. 5th ed. Philadelphia, PA: Butterworth-Heinemann; 2008:chap 74.

Marsh M, Jenkins HA. Temporal bone neoplasms and lateralcranial base surgery. In: Flint PW, Haughey BH, Lund LJ, et al, eds. Cummings Otolaryngology: Head & Neck Surgery. 5th ed. Philadelphia, PA: Mosby Elsevier; 2010:chap 176.


Review Date: 2/3/2014
Reviewed By: Ashutosh Kacker, MD, FACS, Professor of Clinical Otolaryngology, Weill Cornell Medical College, and Attending Otolaryngologist, New York-Presbyterian Hospital, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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