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Surgical Options for Meniere's Disease

Ménière's Disease

There is controversy surrounding the treatment of ménière's disease, a condition characterised by mystique and misunderstanding. However, there is a definite role for surgery in a certain group of patients.

History

In 1861, Dr Prosper Ménière, head of The Institute for Deaf Mutes in Paris described a series of patients with episodic vertigo, tinnitus and fluctuating hearing loss. The first surgical treatments were undertaken in 1904 when Lake and Milligan attempted to relieve vertigo by destroying the semi-circular canals in the inner ear and Perry proposed intracranial division of the vestibular (balance) nerve.

In 1923, Portmann experimentally produced balance symptoms in fish by obliterating the endolymphatic sac, and four years later proposed the drainage of excess fluid from the endolymphatic sac into the mastoid as a treatment for ménière's disease.

The surgical decision

The decision to operate on a patient with ménière's disease often involves a consideration of the uninvolved ear. The potential for ménière's disease to involve both ears, in the long term, is between 20 and 34.4%. Over 90% of patients with both ears affected have better long term hearing in the second ear. Therefore, the risk that the second affected ear will be ultimately worse than the first is around 1%.

Patients selected for surgery in the author's practice are those who suffer incapacitating attacks of vertigo despite maximal medical treatment including medications, and diet and lifestyle changes. Selection for surgery depends more on the decree of incapacity than the frequency of attacks.

Therefore, surgery is indicated in only 10-20% of patients.

The decision to proceed with surgery is a personal one based on a number of factors. The debility suffered from recurrent attacks of vertigo varies among patients and often relates to their general lifestyle and expectations. A young, busy executive with frequent attacks will request surgery much sooner than a retired, elderly patient with occasional attacks.

The decision with regard to the type of surgery should be based on presently available facts rather than unsubstantial evidence. Proven facts are hard to obtain so patients and their families are advised to carefully assess treatments and to be aware that there is no perfect treatment for ménière's disease.

Endolymphatic Saccus Surgery (ESS)

This surgery aims to abolish vertigo but to preserve hearing. The operation has been carried out since 1926 with many variations which include; removal of the bone around the sac (decompression); the insertion of a shunt or tube into the sac; and the removal of the sac altogether.

An incision is made behind the ear and a mastoidectomy is then performed. This involves the removal of the mastoid bone to expose the facial nerve and semi-circular canals. The bone is then removed from around the endolymphatic sac. The surgical treatment of the sac, including drainage techniques or decompression, varies among different surgeons as there is no singe established or proven technique.

Before surgery, patients frequently have hearing in the affected ear but its usefulness may be limited by the symptoms of distortion. Following surgery, patients may suffer from dizziness but are often well enough to walk around after one or two days. Pain relief and anti-nausea agents are sometimes required but patients are generally comfortable.

The major risks of ESS include facial nerve damage; complete hearing loss, and leakage of spinal fluid, although these are rare.

Following surgery, hearing may fluctuate or even improve in a few cases; however in the natural course of Ménière's, the trend is a gradual decline in hearing. Successful relief of vertigo occurs in about 60-80% of patients in the short term but most well performed studies indicate a recurrence of symptoms over longer periods and vestibular nerve section may be required. For these reasons, SS is not frequently carried out in Professor Atlas’ practice.

Vestibular Nerve Section (VNS)

This procedure involves cutting the balance nerve supplying the inner ear affected by ménière's disease, but preserving the hearing nerve. The patients selected for VNS usually have residual, sometimes fluctuating hearing loss. However, some patients have severe hearing loss and may be selected for both vestibular (balance) and cochlea (hearing) nerve section if tinnitus is a major problem.

The patients who cannot undergo this type of procedure include those with no hearing in the opposite ear and those with central nervous system disease, such as stroke, poor health, and aged patients. Surgery may also not be available option for patients with hip, knee or back disorders which affect balance.

VNS is carried out by surgically dividing the balance nerve as it passes from the brain to the inner ear. Following an incision made behind the ear, a small bone disk is removed, giving access to the vestibular nerve. Gentle but minimal brain retraction is required before the nerve section.

The patient is monitored in a high dependency unit for one night following surgery. Pain relief and anti-nausea medications are required in the first 24-48 hours as dizziness usually occurs. Patients are encouraged to get out of bed on the second or third day to begin balance rehabilitation and the majority of patients are home by the 5th to 7th day.

A marked worsening of hearing following surgery occurs in up to 4% of cases, which is not significantly different from ESS. Most patients experience no change but hearing will worsen over time in the natural progression of ménière's.

There have been no cases of permanent facial weakness following vestibular nerve section in the author's series but this is a potential complication in all forms of ménière's disease surgery. Other risks include leakage of spinal fluid. Severe complications, such as meningitis or stroke have been reported, but are extremely rare.

The disadvantage of VNS is the potential for incomplete balance compensation. The loss of balance following surgery requires the opposite ear to compensate. In some patients, compensation is incomplete and patients have symptoms of veering and staggering. Significant symptoms of this type are uncommon, and balance physiotherapy is very useful. After surgery, more than 90% of patients consider themselves to be more active than before. ESS is considered a less complicated procedure than VNS but is less successful in the abolition of acute attacks of dizziness, especially over the long term.

However, it may be considered as a first option with VNS left in reserve.

Destructive surgery

There are a number of so-called destructive procedures carried out to relieve vertigo in patients without hearing. These are used when disabling attacks of vertigo occur and there is no evidence of disease in the opposite ear.

When patients are carefully assessed before surgery, surgical destruction of the labyrinthe almost always relieves vertigo. The most common type of labyrinthectomy is carried out using a similar approach to endolymphatic surgery but the entire balance organ is removed. This type of surgery is rarely used but may be reserved for patients in poor health who would probably not tolerate the stresses of a longer general anaesthetic and post-operative recovery.

Results of surgery

There is great controversy regarding the assessment and reporting of results following surgery. The assessment of surgery requires careful, standardised studies and long term evaluation. This is frequently missing from many studies of ménière's disease. The fact that surgeons are still undecided with regard to the correct operation creates a problem for the patient. However, the patient should be interested in knowing long-term results of the various surgical treatments offered.

Long term studies of endolymphatic sac surgery indicate that vertigo symptoms return over prolonged periods. Torok in 1977 showed that endolymphatic sac surgery and many other treatments led to a 60-80% success rate of controlling vertigo. There has not been a single report published that convincingly proves that hearing improvement is a direct result of the surgical treatment and not merely a result of time and the natural course of the disease.

The future

The future is bright for the understanding of ménière's disease. Genetic mapping and engineering may find applications for treatment. Inner ear manipulation with electrodes, lasers, and medications may be possible in the future to alleviate or even reverse the progress of ménière's disease.

Summary

Ménière's disease is a disabling condition. Some patients will not respond to medical treatment and will consider surgery. The major forms of surgical treatment include ESS and VNS. Each procedure has its own advantages, risks, and long term results and surgeons need to discuss these with their patients to allow an informed decision to be made.