eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Meniere Disease, Surgical Treatment

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

Updated: Sep 3, 2008

Introduction

This article focuses on surgical management of Ménière disease. For information about medical management, see the eMedicine article Inner Ear, Mèniére Disease, Medical Treatment.

History of the Procedure

The term endolymphatic hydrops is often used synonymously with Ménière disease and Ménière syndrome. Endolymphatic hydrops refers to a condition of increased hydraulic pressure within the inner ear endolymphatic system.

Problem

Excess pressure accumulation in the endolymph can cause a tetrad of symptoms: (1) fluctuating hearing loss, (2) occasional episodic vertigo (usually a spinning sensation, sometimes violent), (3) tinnitus or ringing in the ears (usually low-tone roaring), and (4) aural fullness (eg, pressure, discomfort, fullness sensation in the ears).

Frequency

Although the frequency is probably underestimated, a reasonable estimate is approximately 1,000 cases per 100,000 people. A familial predisposition seems to exist; approximately one half of all patients have a family history of the disease.

Mortality/Morbidity: Although the disease itself is not lethal, significant morbidity can arise from various manifestations of the disease. Vertigo can cause devastating accidents and falls. Hearing loss is often progressive over time. Many patients are unable to work and are forced to claim disability.

Sex: Although sex-related rates are nearly equal, a slight female preponderance may exist, in the range of 50-65%.

Age: In some studies, the mean age among treatment groups was 49-67 years. Ménière disease has been seen at almost all ages. Typical onset begins at early-to-middle adulthood.

Etiology

The syndrome may be idiopathic or may be secondary to various processes interfering with normal production or resorption of endolymph. Examples of these processes are endocrine abnormalities, trauma, electrolyte imbalance, autoimmune dysfunction, medications, parasitic infections, and hyperlipidemia.

Pathophysiology

Two fluids fill the chambers of the inner ear: endolymph and perilymph. These fluids are separated by thin membranes, which house the nervous tissue of hearing and balance. Fluctuations in pressure of these fluids stress the nerve-rich membranes and can cause hearing disturbance, ringing in the ears, vertigo, imbalance, and a pressure sensation in the ear.

More specifically, episodes of hydrops are probably caused by an increase in endolymphatic pressure that causes a break in the membrane separating the perilymph (a potassium-poor fluid) from the endolymph (a potassium-rich fluid). The resultant chemical mixture bathes the vestibular nerve receptors, leading to depolarization blockade and transient function loss. Sudden change in the vestibular nerve firing rate creates an acute vestibular imbalance, giving the sense of vertigo.

Physical distention caused by the increase in endolymphatic pressure leads to mechanical disturbance of the otolithic organs. Because the utricle and saccule are responsible for linear and translational motion detection, rather than angular and rotational acceleration, irritation of these organs may produce nonrotational vestibular symptoms.

This physical distention causes mechanical disturbance of the organ of Corti as well. Distortion of the basilar membrane and of the inner and outer hair cells may cause hearing loss and tinnitus. Because the apex of the cochlea is wound tighter than the base, the apex is more sensitive to pressure changes than the base. This explains why hydrops affects low frequencies (located at the apex) more than high frequencies (located at the relatively wider base of the cochlea).

Symptoms improve after the membrane is repaired and normal sodium and potassium concentrations are restored.

Presentation

History

The typical history involves episodic attacks of true whirling vertigo. These attacks are usually preceded by a variable sense of ear pressure and fullness; decreased hearing; and a low-tone, roaring tinnitus. Episodes of vertigo usually last minutes to hours and are often associated with severe nausea and vomiting. In general, patients feel "run down," imbalanced, and nauseated for several hours to days after an acute attack.

The timing and frequency of the attacks vary. Some patients can regularly predict when they will have an attack. Others note a completely random pattern. Attacks have been linked to triggers such as foods, menstrual cycles, and psychosocial stress.

Some patients are completely asymptomatic between episodes. However, many notice progressive deterioration of hearing and balance function with each successive attack.

Physical

Results of the physical examination vary depending on the disease phase. Physical examination results may be completely normal during remission, particularly if the patient is asymptomatic.

During an acute attack, patients generally have severe vertigo and are often in significant distress. Many present to the physician's office with signs of recent vomiting. Patients are occasionally diaphoretic and pale. Vital signs may show elevated blood pressure, pulse, and respiration.

Neurotologic examination may reveal significant nystagmus. Pneumo-otoscopy of the affected ear may elicit symptoms or cause nystagmus. Generally, the Romberg test (ie, observation of sway or loss of balance while the patient is standing with his or her eyes closed) reveals significant increase in instability and worsening with the eyes closed. If the patient is able to stand with his or her eyes closed, the Fukuda marching step test (ie, observation of directional drift while the patient is marching with eyes closed) may reveal significant deviation. The Dix-Hallpike test (ie, observation of nystagmus while moving a patient from sitting to supine with the head angled 45° to 1 side and then the other) may be positive, indicating coexisting benign positional vertigo.

Hearing is frequently affected. The Weber tuning fork test usually shows results lateralized away from side of the affected ear. In general, the Rinne test indicates that air conduction remains better than bone conduction.

Performing a complete neurologic evaluation during the physical examination is important. New-onset vertigo may be an early sign of stroke, migraine, or brainstem compression, which require emergency care vastly different from those of Ménière disease.

Indications

Surgery is indicated for Ménière disease that is refractory to medical management. Typically, failure to respond to 3-6 months of medical therapy is an indication of surgery. However, patients with severe debility may undergo surgery sooner. Any underlying medical causes for Ménière disease should be treated before surgical therapy is undertaken. The diseased ear must be clearly identified.

Relevant Anatomy

The relevant anatomy revolves around the petrous bone and the inner ear. The ear is divided into 3 sections: external, middle, and inner. The external ear consists of the auricle, external ear canal, and tympanic membrane. The tympanic membrane separates the external ear from the structures of the middle ear. The middle ear is an air-containing space that houses the 3 hearing bones: the malleus, the incus, and the stapes. The inner ear is completely encased in bone and consists of the cochlear vestibular apparatus and its associated nerves.

The cochlear vestibular apparatus is a complex organ arranged in a complex yet elegant spatial orientation. Because it is completely encased in bone, the organ is housed in a series of winding tunnels and interconnecting spaces. The mazelike orientation of these tunnels is appropriately named the labyrinth. The bony labyrinth is the space that encases the membranous labyrinth.

The cochlear portion is a snail-shaped organ that houses the organ of Corti. It is responsible for translating mechanical vibrations into electrical impulses and sending them to the brain through the cochlear nerve. The vestibular system consists of a large chamber (ie, vestibule), from which 3 semicircular canals protrude. Within the vestibule, 2 sensors, ie, the utricle and the saccule, detect linear acceleration, while the semicircular canals detect rotational movements in the 3 planes of rotation.

The cochlear and vestibular segments are joined in the middle and share a dual-chambered hydraulic system. These hydraulic chambers are bathed by endolymph and perilymph. A membrane (ie, membranous labyrinth) separates the fluids and completely surrounds the endolymph like a balloon.

The system may be visualized as a water balloon floating in a pool. The water balloon is the membranous labyrinth that contains the endolymph. Surrounding pool water is the perilymph, which supports the delicate nerve tissues of the membranous labyrinth. In this analogy, the walls of the pool represent the limits of the bony labyrinth space. The ground encasing the pool is the bone that encases the labyrinthine space.

The endolymphatic sac is a reservoir pouch that resides on the posterior surface of the petrous bone against the posterior fossa dura. It is connected via the vestibular duct to drain into the endolymphatic space of the cochlea.

The vestibular apparatus gives off 2 nerves: the superior and the inferior vestibular nerves. Together with the cochlear and facial nerves, the vestibular nerves travel through the internal auditory canal to the cerebellopontine angle.

Contraindications

Patients should be in good health and able to withstand surgery and anesthesia. Perform careful history taking and physical examination before the procedure to detect any medical contraindications.

Surgery is not necessarily contraindicated in elderly patients. Patients in their 80s have tolerated labyrinthectomy fairly well.

Otitis media and mastoiditis are contraindications for surgery. Resolve these infections before proceeding to avoid an increased risk of meningitis.

One principle of ear surgery is to avoid operating on an ear that provides all of the patient's hearing because of the risk of creating profound bilateral deafness. This rule applies to most otologic surgical procedures.

Bilateral vestibular disease is the relative contraindication for destructive procedures because of risk of complete loss of inner ear function (ie, Dandy syndrome).

Hypersensitivity or allergy to the target medication is a contraindication for aminoglycoside perfusion.

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