eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Meniere Disease, Surgical Treatment: Treatment

Author: John C Li, MD, Private Practice in Otology and Neurotology; Medical Director, Balance Center
Contributor Information and Disclosures

Updated: Sep 3, 2008

Treatment

Medical Therapy

Medical therapy is directed toward the mitigation of symptoms and/or their prevention.

Medical treatment of Ménière disease is tailored to the individual during the quiescent phase. Lifestyle and dietary changes are usually the first steps. Avoidance of identifiable triggers (eg, chocolate, caffeine) could be sufficient. Usually, smoking cessation is also recommended.

If medications are required, prescribe a 3-month trial of a diuretic (eg, hydrochlorothiazide and triamterene [Dyazide]) in conjunction with dietary management. Typically, vestibulosuppressants and antinausea medications (eg, meclizine [Antivert], prochlorperazine [Compazine]) are used on an as-needed basis. Chronic use of these medications can be harmful and is not recommended.

For patients with acute vertigo, treatment is directed at controlling the vertigo. Intravenous or intramuscular diazepam provides excellent vestibular suppression and antinausea effects. Steroids can be given for their anti-inflammatory effects in the inner ear. Intravenous fluid support can also help prevent dehydration and replace electrolytes.

Aminoglycosides are a class of antibiotics that were serendipitously discovered to be preferentially toxic to the vestibular (balance) end organ. Destruction of the vestibular end organ renders the brain insensitive to fluctuations in inner ear pressure brought on by Ménière disease. Given systemically, aminoglycosides affect both ears. Although aminoglycosides can be used to treat extremely severe bilateral Ménière disease, such treatment leaves the patient with little or no balance function. The resulting Dandy syndrome can be debilitating.

One recent innovation in the treatment of Ménière disease is the Meniett device. Its use is not precisely a medical treatment, and the device itself does not require surgical installation. It does, however, require insertion of tympanostomy tube so that the device can work; therefore, its use may qualify as a surgical treatment. The Meniett device delivers pulses of pressure to the inner ear via the tympanostomy tube. Although no one knows exactly why this works, some patients have symptomatic relief when the device is used on a daily basis. Because it is new, long-term results have not been fully evaluated.

For information about medical management, see Inner Ear, Ménière Disease, Medical Treatment.

Surgical Therapy

Surgical management of Ménière disease is reserved for medical treatment failures. Typically, failure of a 3- to 6-month trial of diuretics and dietary control justifies a more aggressive approach. Patient factors (eg, severity, occupation, degree of disability, individual tolerance) also affect the timing of surgery.

Surgical therapy has significantly evolved over the past 30 years. In general, most experts agree that surgical therapy for Ménière disease is reserved for cases in which medical treatment fails. Beyond this, considerable controversy exists. Historically, multitudes of clever surgical procedures have been invented, tested, and discarded. This article covers the 4 most generally accepted management options: endolymphatic sac decompression or shunt placement, transtympanic medication perfusion, vestibular nerve sectioning, and labyrinthectomy.

Surgical procedures are divided into 2 major classifications: destructive and nondestructive.

The rationale for using destructive procedures to control vertigo is as follows: Endolymphatic hydrops causes fluid pressure to build up in the inner ear, causing temporary malfunction and misfiring of the inner ear. Abnormal signals traveling to the brain cause vertigo. Therefore, destruction of the inner ear or nerve prevents abnormal signals from reaching the brain. The brain eventually compensates for the loss of the damaged labyrinth, provided that the other inner ear is working properly.

Destructive procedures have several problems. The decision to destroy the function of a misfiring inner ear is predicated on the adequate function of the opposite ear. However, Ménière disease can be bilateral in 7-50% of patients, according to the literature. Therefore, the author recommends avoidance of using destructive procedures in patients with bilateral Ménière disease. Because balance and hearing are closely intertwined in the labyrinth, destruction of the balance portion poses a high risk of destroying hearing as well. Destructive procedures are irreversible and are reserved for severe cases.

Nondestructive procedures are aimed at improving the state of the inner ear. Nondestructive procedures are less invasive than destructive ones and do not preclude use of other treatment modalities.

Preoperative Details

Surgeons must definitively decide which ear is affected and rule out other medical causes of Ménière syndrome before proceeding to surgery.

Appropriate laboratory tests are done to identify other problems. Surgery should proceed only after these results rule out other problems.

MRI is helpful to rule out acoustic neuroma as the source of vertigo.

Electrocochleography (ECoG) can help in localizing the problematic ear. Electronystagmography (ENG) can be helpful to demonstrate reduced vestibular responses and is essential to establish vestibular function in the nonsurgical ear when a destructive procedure is contemplated.

Document function of the inner ear before surgery. Because hearing fluctuates, several preoperative audiograms may be required to capture the range of hearing function. Audiograms can help in identifying the diseased side.

After destructive procedures, patients may initially feel worse than ever and require substantial preoperative and postoperative counseling to adequately prepare for the possible sequelae.

Intraoperative Details

Intraoperative monitoring of the cranial nerves is useful in endolymphatic sac surgery, labyrinthectomy, and vestibular nerve section. Most physicians routinely monitor the facial nerves in all 3 of the procedures mentioned above. Intraoperative monitoring of auditory brainstem responses (ABRs) or direct monitoring of the cranial nerve VIII is crucial for optimal hearing preservation when the vestibular nerve is sectioned. Replacement of the bone flaps in retrosigmoid nerve sections can often prevent dural traction headaches.

Endolymphatic sac decompression and/or shunt placement

In theory, the endolymphatic sac procedure decreases pressure buildup of the endolymph by removing petrous bone that encases the endolymph reservoir. This allows the reservoir sac to expand more freely than before and allows the pressure to dissipate. In addition, some surgeons insert a drain or valve from the endolymphatic space to the mastoid or subarachnoid space to further reduce pressure.

Exposure of the endolymphatic sac is essentially extended mastoidectomy. Special care is taken to skeletonize the sigmoid sinus, posterior fossa dura, and posterior semicircular canal. Thin egg-shelled bone is removed from the posterior fossa dura and sigmoid sinus. The endolymphatic sac is distinguished from the dura by differences in its color and texture (ie, sac is thicker and whiter than surrounding tissue).

The location of the sac can vary somewhat, but it is generally immediately posterior or posteroinferior to the posterior semicircular canal. In endolymphatic sac decompression, the procedure is terminated all of the bone overlying the sac is removed. Shunt procedures involve incising the lateral leaf of the sac and inserting a drainage tube into the internal lumen of the endolymphatic duct. Shunts can drain into the subarachnoid space or the mastoid space.

Success rates of 60-90% have been reported for vertigo control and stabilizing hearing acuity. Success rates for endolymphatic sac decompression and for shunt procedures do not seem to differ substantially.

Morbidity and mortality risks with endolymphatic sac decompression are relatively small. The risk of hearing loss and facial nerve damage is minimal when the procedure is done by experienced surgeons. Severe postoperative pain is unusual, and the recovery period is usually short and uneventful.

The endolymphatic sac procedure is perhaps one of the most controversial issues in neurotology. Critics state that the procedure is completely worthless. Authors of the famous Danish study argue that endolymphatic surgery is as useless as sham surgery and that any benefit is a placebo effect.¹ Proponents of sac surgery argue that patients really do improve. Compared with destructive procedures, endolymphatic sac procedures have low risk and morbidity and can provide relief to patients in whom medical therapy fails.

Transtympanic medication perfusion, intratympanic perfusion

Transtympanic medication perfusion (see Image 1) is becoming an established treatment modality. Popularized by Dr John Shea in 1995, the procedure is still evolving. Although the techniques, insertion devices, indications, and pharmacologic compositions vary, the basic concept remains the same. Medications that may exert some effect on Ménière disease are delivered to the middle ear cavity through a myringotomy. From there, medication is absorbed into the inner ear, presumably through the round window membrane. This method facilitates the application of high concentrations of medication with minimal systemic effects.

Transtympanic medication perfusion is relatively low risk and simple to perform. Essentially, the procedure is similar to the placement of tympanostomy tubes, which can be done in an office or outpatient setting. Innovations such as the round window microcatheter and Silverstein MicroWick were designed to channel medication flow directly to the round window niche. Theoretically, this method reduces dosing inconsistencies due to loss of medication down the eustachian tube. The round window microcatheter has lost favor and is no longer being produced.

Transtympanic perfusion is considered a nondestructive procedure when steroids are used. Transtympanic steroid application is useful, particularly when patients have poor tolerance for the systemic adverse effects of steroids. Furthermore, high concentrations can be administered by using this approach. Although results of long-term studies are not yet available, success rates appear to be favorable.

Transtympanic perfusion is considered a destructive procedure when aminoglycosides are used. When aminoglycosides are given transtympanically, their effects are concentrated in the affected ear rather than in both ears. Because streptomycin is difficult to obtain in the United States because of restrictions by the U.S. Food and Drug Administration (FDA), gentamicin is more widely used. Early studies show an efficacy of about 90%. Some authors report substantial worsening of hearing in 5-25% of patients.

Vestibular nerve sectioning

Sectioning the diseased balance nerve can be the ultimate solution for patients with useful hearing in the affected ear. Although hearing and balance functions are housed in 1 common chamber within the inner ear, their neural connections to the brain separate themselves into distinct nerve bundles as they course through the internal auditory canal. This anatomical separation facilitates the isolation and ablation of balance function without disturbing hearing function.

Vestibular nerve sectioning is similar to approaches used for acoustic neuroma, because it involves the opening of the internal auditory canal. Vestibular nerve sections are typically performed through a retrosigmoid or middle fossa approach. The translabyrinthine approach would not spare hearing and is used only as added insurance when labyrinthectomy is intended.

Surgeons generally agree that the retrosigmoid approach is less technically difficult than the middle-fossa approach. The retrosigmoid approach is performed through a small craniotomy posterior to the sigmoid sinus. Exposure of the cerebellopontine angle allows visualization of the eighth cranial nerve. Proper identification of the vestibular nerve through observance of proper anatomic relationships is mandatory to avoid severing facial and cochlear nerves. Because the nerve bundle rotates as it exits the internal auditory canal, the vestibular nerve moves from its lateral position to a more superior location. The vestibular nerves are those closest to the tentorium. Monitoring of the facial and cochlear nerves by means of intraoperative ABR monitoring is helpful.

The middle-fossa approach for vestibular nerve sectioning involves creating a 5 X 5-cm craniotomy in the middle fossa just superior to the temporal line above the external ear canal. The dura of the middle fossa is retracted superiorly to expose the bone. After the proper anatomical landmarks are identified, the internal auditory canal is identified and opened, and the vestibular nerve is sectioned as laterally as possible. Fat is packed into the internal auditory canal and held in position by the temporal lobe dura. The craniotomy is then closed to terminate the procedure.

The middle fossa approach has slightly more complete ablation of vestibular function. Because the vestibular fibers are cut immediately as they exit the vestibular and organ, stray vestibular fibers have little opportunity to cross over and travel along the facial and cochlear nerves. The vestibular nerve is sectioned much more medially in the retrosigmoid approach, possibly after stray vestibular fibers have crossed. Although unusual, failure of the retrosigmoid approach may be attributed to these crossed fibers.

Difficulty of locating the internal canal and limited exposure in the canal because of the position of the facial nerve are disadvantages of using the middle-fossa approach. Cutting the vestibular nerve without exerting pressure on cochlear and facial nerves is difficult because the vestibular bundle lies deep within the canal. Furthermore, the risk of damaging the cochlear artery, which causes hearing loss, is higher via the middle fossa than the retrosigmoid approach.

Advantages of vestibular nerve sectioning are a vertigo-control rate of about 95-98%, and hearing preservation in the surgically treated ear, which is successful in about 95% of patients. Risks of this procedure are those inherent to craniotomy and acoustic neuroma surgery. Facial-nerve damage, hearing loss, exacerbation of tinnitus and dizziness, CSF leakage, headaches, hemorrhage, and infection (meningitis) are possible but rarely develop.

After surgery, patients generally require 3-5 days of inpatient care. Adaptation to the surgical loss of 1 vestibular apparatus usually takes weeks to months. Vestibular rehabilitation during this period is often helpful.

Labyrinthectomy

Labyrinthectomy has the advantage of a high cure rate (>95%) and is useful in patients in whom Ménière disease has destroyed their hearing on the affected side. Labyrinthectomy involves ablation of the diseased inner-ear organs but does not require entry into the cranial cavity. Therefore, it is less complex than vestibular nerve sectioning.

Labyrinthectomy can be accomplished through 2 approaches: transcanal and basic mastoidectomy. The transcanal approach takes place through the external ear canal. First, a tympanomeatal flap is elevated. Next, a right angle pick is inserted through the oval window and maneuvered to disrupt and scramble the nerve tissues of the labyrinth. Sometimes, a drill is used to connect the round and oval windows to improve exposure to the neuroepithelium.

Labyrinthectomy can also be performed with a basic mastoidectomy approach. Extension of the mastoidectomy by drilling through the semicircular canals allows for more complete ablation of the labyrinthine neuroepithelium than does the transcanal approach.

Labyrinthectomy is a bit less invasive than vestibular nerve sectioning. Craniotomy is not required; therefore, the risk of CSF leakage and meningitis is reduced. Patients typically require a few days of inpatient care. Adaptation to the surgical loss of 1 vestibular apparatus usually takes weeks to months. Vestibular rehabilitation during this period is also helpful.

Image 2 shows an intraoperative view of a left ear treated with labyrinthectomy.

Postoperative Details

Postoperative care is different for each of the surgeries. Patients undergoing destructive surgery can expect severe vertigo and imbalance for the first few days. Liberally dispense medications to alleviate nausea and vomiting during this time. Early vestibular rehabilitation is helpful in achieving rapid compensation for loss of unilateral vestibular input.

Follow-up

In general, outpatient follow-up requires substantial supportive care. Patients are often dizzy and report feeling worse than ever before. Outpatient vestibular rehabilitation has been helpful in patients undergoing destructive procedures.

Patients who have undergone endolymphatic sac decompression notice conductive hearing loss due to blood that accumulates behind the eardrum after surgery. Some patients have dizziness immediately after surgery.

Long-term follow-up can be scheduled for every 3-6 months initially and then changed to an as-needed schedule.

See the eMedicine patient education articles Ménière Disease and Tinnitus.

Complications

Many potential risks and complications exist, but they develop relatively rarely. As with any ear surgery, hearing loss, tinnitus, dizziness, facial paralysis, hematoma, bleeding, CSF leak, taste disturbance, and mouth dryness are possible.

Transtympanic injections are the least risky treatments among those discussed here. Transtympanic injections are equivalent to tympanostomy tube placement in terms of risk. Risks vary with the type of medication applied. Steroids have been fairly safe when given transtympanically. Aminoglycosides can adversely affect hearing. Hearing loss statistics range from 5-25%. Some authors have noted otitis media, otorrhea, and prolonged perforations.

Endolymphatic sac decompression incurs the same risks as standard mastoidectomy does. Endolymphatic sac shunts can increase the risk of CSF complications (eg, leakage, meningitis) when they penetrate the subarachnoid space.

Vestibular nerve sectioning involves craniotomy and therefore carries the highest risk of CSF complications and increases the risk of damage to the facial and cochlear nerves compared with standard mastoidectomy. Other complications depend on the approach used. The retrosigmoid approach is associated with a high incidence of traction headaches, which has been attributed to postoperative adhesion of the scalp to the dura, particularly when the bone window is not replaced. Violation of the tracts of the labyrinthine air cells can also be a pathway for CSF leakage if these openings are not covered with bone wax.

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