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Overview

 

Glomus Jugulare Tumor

A glomus jugulare tumor is a tumor that can affect the ear, upper neck, base of the skull, and the surrounding blood vessels and nerves.


General Information

  • Arising from the glomus bodies located within the ear, they are the most common tumors of the middle ear.
  • These tumors are slow-growing and very vascular, with blood supply from the external carotid artery and internal carotid artery.
  • These tumors are most common in women.


Causes

A glomus jugulare tumor grows in the bones of the skull, where nerves are located. Specifically, the tumor occurs in part of the jugular vein (a large vein in the neck) or along a certain nerve on the side of the skull (temporal bone).

Both of these areas contain glomus bodies, which are nerve fibers that normally respond to change in body temperature or blood pressure. A person with a glomus jugulare tumor may have hearing loss, dizziness, or a feeling of a loud pulsing in the ear.

These tumors usually occur later in life, around age 60 or 70, but 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 succinate dehydrogenase (SDHD), an enzyme.


Symptoms

Symptoms may include: -
  • Difficulty swallowing (dysphagia)
  • Hearing problems
  • Hoarseness
  • Pain
  • Weakness or loss of movement in the face (cranial nerve palsy)
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Exams And Tests

Glomus jugulare tumors are diagnosed by physical examination and various scans including: -
  • Cerebral angiography (injection of dye so that the blood vessels of the neck and brain can be seen with x-rays)
  • CT scan
  • MRI scan


Treatment

  • Medical therapy
    • For tumors that actively secrete hormones and neurotransmitters, medical therapy can ease the symptom and be useful prior to surgery.
    • Alpha and beta blockers are given before surgery to block possibly lethal blood pressure abnormalities and heart arrhythmias.
  • Surgery
    • These tumors are very vascular; therefore, pre-operative blockage of the blood supply to the tumor is often performed.
    • Possible complications of surgery include persistent leakage of cerebrospinal fluid (CSF) from the ear, and also damage to one of the nerves controlling face movement, sensation or hearing.
    • Even though the surgery may be successful glomus jugulare tumors are somewhat problematic because they have a high recurrence rate and may require multiple operations.
  • Radiation
    • Radiation may relieve symptoms and stop growth in spite of persistent tumor mass.
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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 facial paralysis, hearing loss, and difficulty swallowing.




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