Endolymphatic Shunt

Definition
Purpose
Demographics
Description
Diagnosis/Preparation
Aftercare
Risks
Normal results
Morbidity and mortality rates
Alternatives

Definition

An endolymphatic shunt is a surgical procedure in which a very small silicone tube is placed in the membranous labyrinth of the inner ear to drain excess fluid.

Purpose

An endolymphatic shunt is placed as part of the treatment of Meniere’s disease, a disorder of the inner ear whose causes are still unknown. Ménière’s disease is characterized by the following symptoms:

• a rise in the level of endolymphatic fluid in the labyrinth of the inner ear
• hearing loss that comes and goes

• a sensation that the environment or oneself is revolving or spinning (vertigo)
• ringing, buzzing, or hissing noises in the ears (tinnitus)
• a feeling that the ears are blocked or plugged

Endolymphatic shunt surgery is one of the surgical procedures available to treat Meniere’s disease, which is also known as endolymphatic hydrops. The surgery is based on the theory that the disorder causes the inner ear to become overloaded with fluid and that draining this fluid will relieve the symptoms. The fluid is drained by opening the endolymphatic sac, a pouch located next to the mastoid bone at the end of the endolymphatic duct. The endolymphatic duct is a canal that leads to the inner ear.

Demographics

According to the National Institute on Deafness and Other Communication Disorders (NIDCD), there were an estimated three to five million cases of Ménière’s disease in the United States in 1998, with nearly 100,000 new cases diagnosed annually. In most cases only one ear is affected, but as many as 15–40% of patients are affected in both ears. The onset of Méniere’s disease occurs most often in adults between the ages of 20 and 50. Men and women are affected in equal numbers.

Description

An endolymphatic shunt is placed with the patient under general anesthesia. The operation takes about two hours to perform. The patient is usually positioned lying on the back with the head turned to one side and the affected ear lying uppermost. The head is immobilized and supported with a pad or brace. The operation itself begins with opening the mastoid bone and identifying the endolymphatic sac. To find the sac, the surgeon removes the bony cover of the sigmoid sinus, which is an S-shaped cavity behind the mastoid bone. The surgeon leaves intact a small rectangle of thin bone called Bill’s Island (named for Dr. William House). The sigmoid sinus is then collapsed with gentle pressure. The surgeon exposes the endolymphatic sac and makes an incision in it in order to insert the shunt.

Diagnosis/Preparation

The diagnosis of Meniere’s disease is based on the patient’s medical history, a physical examination, and the results of hearing tests, balance tests, an electro-nystagmogram, and imaging studies. An MRI or CT scan is performed to rule out a tumor as the cause of the patient’s symptoms. A hearing test (audiogram) identifies the hearing loss that is typical of Ménière’s disease. Balance function tests are administered to assess the patient’s vertigo.

KEY TERMS

Bony labyrinth— A series of cavities contained in a capsule inside the temporal bone of the skull. The endolymph-filled membranous labyrinth is suspended in a fluid inside the bony labyrinth.

Electronystagmogram— A test that involves the graphic recording of eye movements.

Endolymph— The watery fluid contained in the membranous labyrinth of the inner ear.

Endolymphatic sac— The pouch at the end of the endolymphatic duct that connects to the membranous labyrinth of the inner ear.

Mastoid— A large bony process at the base of the skull behind the ear. It contains air spaces that connect with the cavity of the middle ear.

Membranous labyrinth— A complex arrangement of communicating membranous canals and sacs, filled with endolymph and suspended within the cavity of the bony labyrinth.

Ménière’s disease— Also known as idiopathic endolymphatic hydrops, Méniere’s disease is a disorder of the inner ear. It is named for Prosper Ménière (1799–1862), a French physician.

Shunt— A channel through which blood or another body fluid is diverted from its normal path by surgical reconstruction or the insertion of a synthetic tube.

Sigmoid sinus— An S-shaped cavity on the inner side of the skull behind the mastoid process.

Tinnitus— A sensation of ringing, buzzing, roaring, or clicking noises in the ear.

Vertigo— An illusory feeling that either one’s self or the environment is revolving. It is usually caused either by diseases of the inner ear or disturbances of the central nervous system.

The patient is prepared for surgery by having the hair removed and the skin shaved over an area of at least 1.5 in (3.8 cm) around the site of the incision. A mild solution of soap and water is commonly used to cleanse the outer ear and surrounding skin.

Aftercare

The operated ear is covered with a Glassock dressing, which is a special dressing applied to keep pressure on the site to reduce swelling. There is usually some tenderness and discomfort in the operated ear and the throat (from the breathing tube inserted during

WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?

An endolymphatic shunt is performed in a hospital or ambulatory surgery center on an outpatient basis. It is done by an otolaryngologist, who is a surgeon specializing in disorders of the ear, nose, and throat.

surgery), which can be controlled by such analgesic medications as meperidine (Demerol) or oxycodone (Percocet).

Risks

There are few risks associated with endolymphatic shunt surgery. The operation is considered the first-line surgical treatment for Meniere’s disease precisely because it is very safe. The chance of hearing loss from the procedure is about 0.5%.

Normal results

Endolymphatic shunt surgery relieves the vertigo associated with Ménière’s disease, with restoration of hearing dependent on the severity of the disease. The patient’s ear may protrude slightly shortly after surgery but usually returns to its original position within two to three weeks after the operation. Numbness around the ear is a common complication that may last for several months.

Morbidity and mortality rates

Endolymphatic shunt surgery is considered a low-morbidity procedure. It has been reported to achieve complete or substantial control of vertigo in 81% of patients, with significant improvement in hearing in about 20%. Overall, there is a 60% chance of curing the vertigo, a 20% chance that the attacks will remain at the same level of severity, and a 20% chance that the attacks will get worse. The patient’s vertigo usually improves even if hearing does not improve.

Alternatives

Nonsurgical alternatives

There are several nonsurgical treatments recommended for patients with Meniere’s disease:

Vestibular suppressants. These are drugs designed to control vertigo attacks; they include mechzine (Antivert), diazepam (Valium), and dimenhydrinate (Dramamine).

QUESTIONS TO ASK THE DOCTOR

• What are the alternatives to an endolymphatic shunt procedure?
• Will I regain my hearing if I have this surgery?
• Can I expect improvement in any of the other symptoms of Ménière’s disease?
• How long will it take to recover from the surgery?
• How many endolymphatic shunts do you perform each year?

• Diuretics. Medications that increase the body’s output of urine can also help reduce the frequency of vertigo attacks in some patients by lowering the amount of fluid in the body.
• Dietary changes. Although the benefits of a low salt diet have not been confirmed by formal scientific research, many patients with Ménière’s disease have noted that their symptoms improve when they restrict their salt intake.
• Steroids. Prednisone and other steroids have been used to treat patients in the early stages of Ménière’s disease. Their use in this disorder, however, is still considered experimental as of 2003.

Surgical

Surgical alternatives to the placement of an endolymphatic shunt include:

• Selective vestibular neurectomy. In this procedure, the surgeon cuts the vestibular nerve, which relays balance, position and movement signals from the inner ear to the brain. Vestibular neurectomy prevents the transmission of faulty information from the affected ear and eliminates attacks of vertigo in many patients.
• Labyrinthectomy. In this procedure, the membranous labyrinth of the inner ear is removed. Labyrin-thectomy is more successful than other surgeries in eliminating vertigo, but the patient suffers complete and permanent loss of hearing in the operated ear.

Resources

BOOKS

Graham, M. D. Treatment Options for Ménière’s Disease: Endolymphatic Sac Surgery: Do It or Don’t Do It and Why? San Diego: Singular Publishing, 1998.

Haybach, P. J., and J. Underwood. Ménière’s Disease: What You Need to Know. Portland, OR: Vestibular Disorders Association, 19

“Ménière’s Disease.” Section 7, Chapter 85 in The Merck Manual of Diagnosis and Therapy, edited by Mark H. Beers, MD, and Robert Berkow, MD. Whitehouse Station, NJ: Merck Research Laboratories, 1999.

PERIODICALS

Brookler, K. H. “A Patient with Endolymphatic Hydrops Refractory to Shunt Surgery.” Ear Nose Throat Journal 79 (July 2000): 493.

Goksu, N., Y. A. Bayazit, A. Abdulhalik, and Y. K. Kema-loglu. “Vestibular Neurectomy with Simultaneous Endolymphatic Subarachnoid Shunt.” European Archives of Otorhinolaryngology 259 (May 2002): 243–246.

Mason, T. P. “What is Ménière’s Disease, and How is it Treated?” Health News 9 (April 2003): 12.

Ostrowski, V. B., and J. M. Kartush. “Endolymphatic Sac-Vein Decompression for Intractable Ménière’s Disease: Long Term Treatment Results.” Otolaryngology Head and Neck Surgery 128 (April 2003): 550–559.

Welling, D. B., and H. N. Nagaraja. “Endolymphatic Mastoid Shunt: a Reevaluation of Efficacy.” Otolaryngology Head and Neck Surgery 122 (March 2000): 340–345.

ORGANIZATIONS

American Academy of Otolaryngology—Head and Neck Surgery. One Prince Street, Alexandria, VA 22314. (703) 806-4444. www.entnet.org.

National Institute on Deafness and Other Communication Disorders (NIDCD). 31 Center Drive, MSC 2320, Bethesda, MD 20892-2320. (800) 241-1044. www.nidcd.nih.gov/.

Vestibular Disorders Association (VEDA). PO Box 4467, Portland, OR 97208-4467. (800) 837-8428. www.vestibular.org.

OTHER

“Meniere’s Disease—Surgical Therapy.” UPennHealth. www.uphs.upenn.edu/balance/patient%20education%20brochures/Meniere’s-Surgical%20Treatment.htm.

Monique Laberge, Ph.D.

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