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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 sometime in the past, usually 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 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 know 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 cholesteatomas. 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 hearing loss and damage the delicate structures of the middle ear. In rare cases, dizziness and facial paralysis or weakness may occur. Surgery for this procedure is almost always done on an outpatient basis. The recovery period for both children and adults is relatively quick, usually within 5-10 days. The surgery requires thorough knowledge of the complex anatomy of the ear and regular experience performing this type of surgery to best avoid complications. In many cases the conductive hearing loss is amenable to reconstruction with modern techniques, leading to restoration of some or all of the hearing loss.
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Otosclerosis
Otosclerosis is an inherited disease that is progressive in nature and creates a 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 done on an outpatient basis and the recovery period is typically brief, usually within 5-10 days. Stapedotomy is a highly effective way to correct the conductive hearing losses of patients with otosclerosis and 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 losses and Baha systems for conductive hearing losses or single-sided deafness.
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Cochlear Implants
Cochlear implant technology has advanced significantly over the past several years and the Food & Drug Administration has relaxed the candidacy criteria for both children and adults. When severe or profound bilateral hearing losses are 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 5-10 days. For more information about cochlear implants please visit our audiology services page. We also invite you to visit the link for our supplier, Cochlear Americas.
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Baha Systems
Baha systems are for an entirely different type of patient. There are several specific conditions that could warrant the placement of a Baha:
- Patients who have a 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 "deaf" side so the sound processor can route the auditory signals coming from the "deaf" side across the skull via bone conduction to the better hearing ear. Baha placement is an outpatient procedure and recovery time for both children and adults is approximately 5-7 days, however there is a 3-4 month post-op waiting period before the sound processor can be activated. For additional information about Baha systems, please visit our audiology services page or the home page for our Baha supplier, Cochlear Americas.. |
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Acoustic Neuromas & 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 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, a common misperception in many cases. 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 preserve as much hearing and balance function as possible, 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 exists that causes 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 true spinning vertigo. The dizziness demonstrates a "crescendo/decrescendo" effect as it increases and then decreases over time. Patients often report that remaining still will help quell the dizziness once it has begun. These "spells" can last for a few seconds but usually not longer than a few minutes.
The dizziness is initiated when tiny crystal inside the inner ear (or "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, a motion sensor for vertical head movements. The motion of the crystals as they push on the motion sensor sends an abnormal message to the brain 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 thereby sending false messages to the balance receptors and brain.
BPPV is mostly commonly diagnosed by doctors using the Dix Hallpike maneuver which 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 presentations 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 sleeping 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% 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 tinnitus (or ringing in the ears). Feelings of fullness or pressure in the ear(s) may also be a symptom. Perhaps the most disconcerting and, at times, disabling component of Meniere's is vertigo. Episodes typically start with little to no warning and may last minutes to several hours, rarely longer. Nausea and even vomiting can 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 come in varying degrees and patterns from patient to patient and can manifest themselves as an occasional annoyance or become very disabling. It is important to establish care with an experienced specialist who can help patients manage these symptoms and minimize the interruptions to their lives.
In addition to a complete history and thorough examination by a physician, a hearing test can be very helpful in diagnosing Meniere's. 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 balance function. 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 balance organs and the patient's own 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 and to 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 your specialist.
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