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Otology, Neurotology, and Skull Base Surgery

Neurotology is a clinical subspecialty within the field of otolaryngology (ear, nose, and throat medicine) which focuses on the neurology and neurosurgery of the ear. This includes the diagnosis, treatment, and rehabilitation of hearing and balance disorders, as well as the treatment of the facial nerve, the ear and temporal bone, and related structures, such as the skull base and brainstem. Now a well-recognized field within the specialty of otolaryngology, it is represented by a dedicated society, the American Neurotologic Society (ANS) and a peer reviewed journal, Otology & Neurotology. As a comprehensive medical center, ENT neurotology provides the full spectrum of care within this new and challenging field, including many of the following disorders.

• Cholesteatoma
• Otosclerosis
• Implantable Hearing Technologies
• Cochlear Implants
• Baha® Systems
• Acoustic Neuromas and Skull Base Surgery
• Benign Paroxysmal Positional Vertigo (BPPV)
• Meniere's Disease

Cholesteatoma

Cholesteatomas were first described in early medical literature as "pearly tumors" in the middle ear but their name is actually something of a misnomer. These growths contain no cholesterol and are not a true "tumor," per se. Rather, they are more cyst-like in nature and most commonly arise in patients with a history of frequent ear infections, usually during childhood. Symptoms then return, often years after the last infection. Most often, gradual hearing loss, with or without persistent drainage from the affected ear, leads to the identification of a cholesteatoma. Detection can be difficult in some cases. Proper evaluation should include a careful review of the patient's medical history, examination of the ear, usually with a microscope, and a hearing test. A CT scan might be helpful in some cases if the diagnosis is not clear, or if complications from the cholesteatoma are suspected. The hearing loss is typically conductive in nature, meaning that sound is not transmitted properly through the eardrum and middle ear bones (ossicles). There might be some damage to the hearing nerve in selected cases.

The cholesteatoma is actually an ingrowth of skin that collects in small pockets in the middle ear, then may extend to the bone behind the ear known as the mastoid. The sloughed skin acts like a foreign body in the closed environment of the middle ear and becomes chronically infected, thereby creating the pearly growth that typifies this condition. These space-occupying growths can be congenital but are most often acquired over time. They affect children and adults alike and surgery is the preferred treatment. If left untreated, cholesteatomas can cause damage to the delicate structures of the middle ear and hearing loss. In rare cases, dizziness and facial paralysis or weakness may occur. Surgery for this procedure is almost always performed on an outpatient basis. The recovery period for both children and adults is relatively quick, usually within five to ten days.

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Otosclerosis

Otosclerosis is an inherited disease that is progressive in nature and causes conductive hearing loss. The condition can begin in early adulthood and is more prominent in women than men. Research also indicates that there is some connection between pregnancy and the onset or acceleration of otosclerosis. For patients with this condition, hearing loss occurs due to fixation of the middle ear bones (or ossicles) and most specifically, the stapes. This fixation is caused by new, spongy growths of bone around the stapes, which hinders the transmission of sound.

Surgery is the preferred treatment for otosclerosis and the procedure is called stapedotomy or, in some cases, stapedectomy. The surgeon bypasses the bony growth by removing some of the stapes bone with a laser and attaching a microscopically small metallic prosthesis. This prosthesis is barely the size of an eyelash and is non-magnetic, making it safe for patients to have MRI scans in the future, if needed. The surgery is performed on an outpatient basis and the recovery period is typically brief, usually within five to ten days. Stapedotomy is a highly effective way to correct conductive hearing loss in patients with otosclerosis. Results in some patients can be dramatic. Hearing aids are typically an alternative to surgery.

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Implantable Hearing Technologies

Our office is pleased to offer several types of surgically implantable hearing technologies including cochlear implants for severe to profound hearing loss and Baha® systems for conductive hearing loss or single-sided deafness.

Cochlear Implants

Cochlear implant technology has advanced significantly over the past several years and the U.S. Food and Drug Administration has relaxed the candidacy criteria for both children and adults. When severe or profound bilateral hearing loss is no longer treatable with traditional hearing aids, cochlear implants can be an important option for the right type of patient. There is a battery of tests we must perform to determine if a patient is an implant candidate. A tiny array of electrodes is surgically placed within the cochlea and provides stimulation to the neural fibers that transmit hearing impulses. An external "speech processor" that resembles a behind the ear hearing aid is worn in conjunction with the internal components. While cochlear implants are not a "quick fix" treatment for hearing loss and require a sincere commitment to frequent audiologic follow-up, they have become one of the most rewarding procedures we perform. It is an outpatient procedure and recovery time is very brief, usually five to ten days.

Baha® Systems

Baha® systems are for an entirely different type of patient. There are several specific conditions that could warrant the use of the Baha® system:

• Patients who have conductive or mixed hearing loss that has not been remedied by other surgeries
• A chronically draining ear that, despite medical intervention, cannot tolerate a traditional hearing aid
• Patients who have no ear canal (atresia) or small, malformed outer ears (microtia)
• Normal hearing in one ear with a permanent, profound hearing loss in the other (also called single-sided deafness or SSD)

A Baha® system requires surgical placement of a titanium screw fixture in the mastoid bone behind the ear and under the skin while a small abutment protrudes outward, allowing the attachment of a sound processor. For patients with medical conditions that prohibit the fitting of conventional hearing aids, this sound processor serves as a way to direct sound toward the inner ear using the efficiency of bone conduction while bypassing the outer and middle ear. Patients with single-sided deafness receive a Baha® on the side with more significant hearing loss so the sound processor can route the auditory signals coming from that side across the skull via bone conduction to ear with better hearing. Baha® placement is an outpatient procedure and recovery time for both children and adults is approximately five to seven days, however, there is a three to four month post-operative waiting period before the sound processor can be activated.

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Acoustic Neuromas and Skull Base Surgery

An acoustic neuroma is a benign tumor growth that occurs on the nerve for hearing and balance function, between the inner ear and the brainstem. It is very slow growing and most often presents with gradual hearing loss and/or tinnitus (ringing in the ear), but sometimes may cause subtle disturbances of equilibrium or outright dizziness. The hearing loss is typically in the higher frequencies and might be harder for the individual to notice until it becomes more severe. Occasionally, a sudden loss of hearing might occur, prompting more immediate attention and evaluation. A medical history, physical examination, and audiogram (hearing test) are needed to suspect an acoustic neuroma. Its presence is best detected with an MRI scan. Rarely, a CT scan can be used to identify these lesions if an MRI is contraindicated, such as in patients with pacemakers or other medical implants that are not compatible with the magnetic fields used in MRI scans.

Traditionally, surgical removal has been the treatment of choice, but often at the expense of residual balance or hearing function in the affected ear. Hearing conservation techniques are available, but depend on the size and location of the tumor for success. Hearing does not improve with removal, which is a common misperception. Also, newer techniques in radiation treatment have emerged as viable options to surgery. The goal of radiation is to prevent further growth of the tumor and further loss of hearing and balance, but the tumor does not shrink significantly or resolve. Follow-up scanning is necessary to document stability of the tumor in this situation. Acoustic neuromas are uncommon and usually occur in one ear only. A rare genetic variant can cause both ears to be involved, usually at a relatively young age. Consultation with a neurotologist is very important for a patient identified with an acoustic neuroma.

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Benign Paroxysmal Positional Vertigo (BPPV)

This type of dizziness is provoked when the head and inner ear structures are moved into certain positions. Tipping back in the shower, rolling over in bed, and bending over at the waist can initiate sensations of imbalance and floating, but most often spinning vertigo. The dizziness increases and decreases over time. Patients often report that staying still helps quell the dizziness once it has begun. These dizzy spells can last from a few seconds to usually no longer than a few minutes.

The dizziness is initiated when small crystals of calcium carbonate inside the inner ear, called otoconia, become dislodged from their normal resting place and begin to float freely in the fluids of the inner ear. They settle in the lowest part of the inner ear, which is a motion sensor for vertical head movements. The free-floating motion of the crystals in this area causes them to push on this motion sensor, sending abnormal messages to the brain. These messages tell the brain that that the body is moving, even when it is not. The crystals can become dislodged by head injury, aging, or even without any known or explicable cause. A very simple analogy for BPPV is that of a water-filled toy snow globe. When the globe is in its resting position, the particles are inactive on the floor. Move the globe just a little and the disturbed particles suddenly begin to move around in the water until gravity returns them to a resting position. BPPV can often be treated by maneuvering the otoconia crystals into a repository place in the inner ear that prevents them from free-floating and sending false messages to the balance receptors and brain.

BPPV is most commonly diagnosed by doctors using the Dix Hallpike maneuver. This maneuver puts the patient in a reclined position with the head hanging slightly lower than the rest of the body. If BPPV is present, the patient's eyes will begin to move in a reflexive pattern called nystagmus. It is easily observable by the doctor and is one of the classic signs of BPPV.

The particle repositioning treatment can be done in a doctor's office with no anesthesia and no surgery. It involves a series of maneuvers that place the head and body in various positions so the crystals can travel through the inner ear fluid to a new resting place. The doctor will guide you through these positions and the entire process takes just a few minutes. Aftercare is quite specific and involves limited head movement and resting on several pillows for a few days and nights. The repositioned crystals need time to settle and following the aftercare instructions is key to this. Most patients experience relief after just one treatment but, occasionally, a second treatment may be needed weeks, months, or even years afterward.

There are surgical treatments available for the rare patient with ongoing BPPV that fails to respond to conventional particle repositioning therapies. Your specialist can help you determine if surgical intervention is appropriate for you and can discuss the risks, benefits, and expected outcome for this type of treatment.

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Meniere's Disease

Meniere's disease is a disorder that arises from an abnormality of inner ear fluids. Although it commonly affects only one ear, the American Academy of Otolaryngology estimates that approximately 15 percent of all diagnosed cases may involve both ears. The classic trio of symptoms comprising Meniere's disease are episodic spinning vertigo, fluctuating hearing loss, and ringing of the ears. Feelings of fullness or pressure in the ear or ears may also be a symptom. Perhaps the most disconcerting and, at times, disabling component of Meniere's disease is vertigo. Episodes typically start with little or no warning and may last just a few minutes or several hours, but rarely longer. Nausea and even vomiting can also occur. Many patients report a sense of drowsiness or fatigue following a Meniere's attack that can linger for several days. Fluctuations in hearing are common and there is often a sense of distortion or lack of clarity that makes it difficult to understand speech or tolerate louder sounds. These symptoms vary from patient to patient and can manifest themselves as an occasional annoyance or become very disabling. It is important for patients to establish care with an experienced specialist who can help them manage these symptoms and minimize the interruptions to their lives.

In addition to a complete medical history and thorough examination by a physician, a hearing test can be very helpful in diagnosing Meniere's disease. There are very classic attributes of an audiogram that help physicians identify Meniere's disease. These include hearing loss in the low frequencies and reduced ability to recognize and repeat words. The doctor may order additional tests, as needed, to assess the patient's sense of balance. In some cases, the doctor may suggest a CT scan or MRI to rule out other conditions.

Meniere's disease can be managed with a combination of medication, diet modification, and lifestyle changes. Patients are advised to eat a low-sodium diet and take a prescription diuretic. Other medications may be prescribed to help manage dizziness, vertigo, and nausea. As always, it is important to maximize a healthy lifestyle by eating properly and remaining physically active. By doing so, the patient's balance capabilities are maintained as much as possible. Extended bed rest is not typically recommended. Patients are asked to limit the intake of caffeine and alcohol, as well as quit smoking.

If episodes cannot be managed by conservative therapies, the doctor may suggest surgery. There are several procedures that can minimize or control vertigo attacks and in many cases hearing can be preserved. Risks, benefits, and reasonable expectations for the outcome of these procedures should be carefully discussed with a specialist.

Contact the Oral and Maxillofacial Surgery Service

If you would like to learn more about otology, neurotology, and skull base surgery at Legacy Emanuel Hospital and Health Center, contact Head and Neck Surgical Associates.

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