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NORD is very grateful to Terry D. Fife, M.D, University of Arizona College of Medicine, Barrow Neurological Institute, for the assistance in the preparation of this report.
Benign paroxysmal position vertigo (BPPV) is a disorder characterized by brief, recurrent bouts of vertigo. Vertigo is a sensation of spinning, whirling or turning. Individuals often feel as if the room is moving or spinning and they can lose their balance and have difficulty standing or walking. During the bouts of vertigo, affected individuals often have abnormal eye movements as well (nystagmus). BPPV is most often triggered by rapid, sometimes unexpected changes in head position. The severity of the disorder varies. In some cases, it only causes mild symptoms, while in others it can potentially cause more severe, even debilitating symptoms. BPPV may disappear on its own only to return weeks or months later. Most affected individuals can be easily and effectively treated by non-invasive methods such as canalith (or canalolith) repositioning maneuvers. However, BPPV may recur even after effectively treated. BPPV is believed to be caused by the displacement of small calcium carbonate crystals within the inner ear. These tiny crystals become dislodged from their normal location and fall into one of three semicircular canals, which are tiny, interconnected, looped tubes that serve to detect movements of the head and that play a role in helping the body maintain balance. The exact, underlying cause of this displacement is not always known (idiopathic). Recurrences are possible because additional calcium can become dislodged. The treatment maneuvers remove the calcium but do not prevent the shedding of additional calcium crystals in the future.
BPPV has been identified as a clinical entity since the late 1800s. The term benign means that the disorder is not progressive and is not considered serious. Although labeled benign, BPPV can disrupt a person's daily activities and affect quality of life. The term paroxysmal means that episodes arise suddenly and often unpredictably. The term positional means the disorder is contingent on a change of the position of the head. BPPV is one of the most common causes of vertigo.
The onset of an episode of BPPV is usually sudden following changes in head position. Often, ordinary movements such as turning over on one's side, lying down, looking up or stooping or bending over can cause an episode. The severity of the disorder can vary greatly from one person to another. Factors that may affect the severity include the speed of head movement, the volume of calcium crystals that are moved, and a person's innate sensitivity to motion. For some people, only a slight positional change of the head can cause symptoms. In such cases, this extreme sensitivity can cause near constant vertigo. In other individuals, the disorder despite a rapid change of head position may only produce mild symptoms. In some cases, symptoms may only be caused by very precise, specific movements. The duration of symptoms of BPPV may also vary and can potentially persist for days, weeks, or months or become recurrent over many years.
Vertigo in individuals with BPPV usually lasts less than 60 seconds. Vertigo can lead to unsteadiness and a loss of balance. Additional symptoms can develop including lightheadedness, dizziness, nausea, vomiting, and blurred vision. Nausea or a feeling of queasiness can persist for a short time even after the sensation of vertigo has passed.
A common associated finding with BPPV is nystagmus, an eye movement disorder characterized by rapid, involuntary movements of the eye. The eyes may be described as jumping or twitching in certain directions. Nystagmus associated with BPPV is fatigable meaning that if one repeats the position change that induced the original vertigo and nystagmus, after time nystagmus lessens in frequency and severity.
The type of nystagmus, defined by the direction of the abnormal eye movements, can vary depending upon which of the three semicircular canals of the inner ear is involved. The three canals are known as the posterior, horizontal and anterior canals. Some researchers subdivide BPPV into posterior BPPV, horizontal BPPV and anterior BPPV based on the specific canal involved. Most cases of BPPV involve the posterior canal, which is also referred to as classic
In some cases, the exact underlying cause of BPPV is unknown (idiopathic). Researchers believe that most cases of BPPV are caused by abnormalities affecting the inner ear. The inner ear contains the cochlea, which converts sound pressure from the outer ear into nerve impulses that are sent to the brain via the auditory canal. The inner ear also contains the semicircular canals. Fluid moves through these canals enabling the brain to detect movements of the head.
Two additional structures found in the inner ear are the utricle and saccule (otolith organs). The utricle and saccule are fluid-filled sacs or cavities that monitor the various movements of one's head and detect the head's position in relation to gravity. The utricle and saccule contain small calcium carbonate crystals. For unknown reasons, in individuals with BPPV these crystals become dislodged and fall into the semicircular canals. Within the canals, these crystals may stimulate nerve endings called cupula, resulting in the body being sensitive to certain head position changes that normally would not cause a response. Basically, the brain is sent powerful asymmetric nerve signals that resemble the kind of asymmetry associated with spinning. This gives a patient the same sensation that would occur with spinning.
Two specific theories proposed in regard to the underlying cause of BPPV are the canalithiasis and cupulolithiasis theories. These proposed mechanisms are not mutually exclusive and there is scientific evidence that both occur, but that canalithiasis is more common than cupulolithiasis. Canalithiasis refers to calcium crystals that are freely mobile within the semicircular canals and, whenever the head changes position, these crystals move through the canal. As these crystals move, they are believed to drag the fluid within the canals, known as endolymph, behind them. As the endolymph moves through the canals, it stimulates the hair cells of the cupula causing vertigo and nystagmus. When the head is not moving, the crystals (and therefore the endolymph) do not move as well. Consequently, there is no stimulation of the cupula and no associated vertigo or nystagmus. It is believed that these crystals eventually dissolve or fall back into the vestibule (the cavity at the entrance to one of the canals). Canalithiasis appears to best explain most cases of BPPV.
Cupulolithiasis refers to crystals that have become stuck (adhered) to the cupula in one of the three semicircular canals, usually the posterior canal. BPPV caused by cupulolithiasis is believed to account for the more persistent cases of BPPV that do not respond as well to positioning treatments.
Neither the canalithiasis nor the cupulolithiasis theories address why the crystals become dislodged. There are many different theories as to what conditions can cause crystals to become dislodged and enter the semicircular canals. Such conditions include head trauma, surgery, chronic middle ear infections (otitis media), a severe cold or infection or vestibular neuritis. There are some weak but possible associations with osteoporosis.
Additional factors that may predispose individuals to BPPV include alcoholism, inactivity, age, and certain central nervous system disorders. In many cases, no such precipitating cause can be identified (idiopathic).
The lifetime prevalence of BPPV is about 2.4% and other estimates range from 10-64 per 100,000 people in the general population. However, many physicians believe that the disorder is often misdiagnosed and that the true frequency may be higher. BPPV most often affects older adults with a peak age of onset in the sixth decade. The disorder may affect individuals of any age, but is quite uncommon in those under 20 years of age. Women are believed to be affected at least twice as often as men.
Symptoms of the following disorders can be similar to those of BBPV. Comparisons may be useful for a differential diagnosis.
Meniere's disease is a disorder characterized by recurrent vertigo, unilateral hearing loss and ringing sounds in the affected ear (tinnitus). It is associated with swelling (dilation) of the membranous labyrinth (endolymphatic hydrops) in the ear. The attacks of vertigo in Meniere's disease appear suddenly and usually last a few hours. Vertigo consists of the sensation that the room or objects are rotating around an affected individual. The dizziness often subsides gradually. The attacks may be of vertigo are often associated with nausea and vomiting. Affected individuals may have a recurrent feeling of fullness or pressure in the affected ear, and hearing tends to fluctuate. Over the years, hearing may progressively worsen. Tinnitus often develops on the affected side and may be more intense before or during an attack of vertigo. Usually, one ear is affected, but both ears are involved in 10 to 15 percent of individuals with Meniere's disease. (For more information on this disorder, choose "Meniere's" as your search term in the Rare Disease Database.)
Labyrinthitis is a disorder characterized by inflammation and swelling of the inner ear. The membranous labyrinth is the series of interconnected tubes and cavities within the inner ear. Labyrinthitis is an inflammatory condition, probably caused by one of many possible viruses and leads to vertigo that is evoked by head movements. Vertigo can result in loss of balance. Nausea and vomiting may also occur. Hearing loss in the affected ear is typical. Labyrinthitis is usually caused by viral infection and much less commonly by bacterial infection.
Vestibular neuritis is very similar to labyrinthitis except that hearing is unaffected. Like labyrinthitis, it is usually caused viral infection of the vestibular nerve of the ear. Since some viral infections may cause patchy inflammation of the vestibular nerve and labyrinth, some have proposed the term neurolabyrinthitis be used to include both labyrinthitis and vestibular neuritis. The vestibular nerve carries information from the inner ear to the brain regarding head movement. There are two vestibular nerve branches and when either one is affected by an infection it leads to imbalance and vertigo. Vestibular neuritis may follow a viral upper respiratory tract infection.
An acoustic neuroma, also known as a vestibular schwannoma, is a rare benign (non-cancerous) growth that develops on the eighth cranial nerve. This nerve runs from the inner ear to the brain and is responsible for hearing and balance (equilibrium). There is no standard or typical pattern of symptom development, although hearing loss in one ear (unilateral) is the initial symptom in approximately 90 percent of cases. Additional findings include ringing in the ears (tinnitus) and dizziness. The symptoms of an acoustic neuroma occur from the tumor pressing against the eighth cranial nerve and disrupting its ability to transmit nerve signals to the brain. An acoustic neuroma is not cancerous (malignant); it does not spread to other parts of the body. The reason an acoustic neuroma forms is unknown. (For more information on this disorder, choose "acoustic neuroma" as your search term in the Rare Disease Database.)
There are additional disorders or conditions that can cause vertigo including migraines, head trauma, and reduced blood flow in the brain as might be caused by stroke, cerebellar hemorrhage or other similar conditions. Mal de Debarquement syndrome, a rare and poorly understood disorder, is characterized by a rocking sensation and/or imbalance that persist for an excessive length of time after an ocean cruise, airplane flight or other motion experience.
A diagnosis of BPPV is based upon identification of characteristic symptoms, a detailed patient history and a thorough clinical evaluation. Affected individuals usually have a history of episodes of vertigo.
Clinical Testing and Work-Up
Affected individuals will undergo a Dix-Hallpike test. During this test, an affected individual sits down, with legs extended, on an examination table. The doctor will rotate the head approximately 30 to 45 degrees and then help the person quickly lie on his or her back (supine position). In individuals with BPPV, this will prompt a characteristic episode of nystagmus and/or vertigo. The timing and appearance of the eye movements will help a physician determine the cause of vertigo. The Dix-Hallpike test can differentiate vertigo caused by a problem in the brain from vertigo caused by a problem in the inner ear. The specific pattern of nystagmus will tell a physician which of the three semicircular canals of the inner ear is involved in an individual case.
In some cases in which a diagnosis is in doubt, affected individuals may undergo a test such as magnetic resonance imaging (MRI) to rule out other conditions. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues such as in the brain or ear.
In some cases, a physician may use a test called a videonystagmography (VNG) or videonystagmography (VNG), which records the voluntary and involuntary movements of the eyes. During this exam, electrodes are placed around the eyes (ENG) or goggles with cameras are placed over the eyes (VNG). Both techniques record eye movements following different stimuli such as staring at a light, moving the head to different positions, and stimulating the inner ear and nearby tissue, usually by cold or warm water (or air). The information can then be analyzed by a computer and this can determine if there is a disturbance in the normal functioning of the inner ear balance.
Individuals with BPPV may be treated with canalith repositioning maneuvers, in which the head is put through a series of specific movements designed to shift the crystals (otoliths) out of the semicircular canals and back into the vestibule. Once the crystals are back in the vestibule, they are usually reabsorbed in a matter of days. The maneuvers may need to be repeated. Different maneuvers are required depending upon which of the three semicircular canals is involved. Canalith repositioning maneuvers are often highly effective in treating BPPV, although the condition can recur often within one year. Canalith repositioning maneuvers are initially performed at a physician’s office, but affected individuals may be taught the maneuvers in to order to perform them at home. During therapy, crystals may occasionally move from one semicircular canal to another, which is referred to as canal switch.
The Epley maneuver is a common canalith repositioning maneuver. The Epley maneuver is a five position cycle that is repeated until no signs of nystagmus are observed. Patients are placed in a sitting position on an examination table with their head turned 45 degrees toward the affected ear. Patients are then tilted back onto the table with their head hanging off the end. The head is then slowly rotated toward the unaffected ear. Patients are then rolled onto their side and the head is rotated back toward the affected ear. The patient is then brought back to a sitting position. Canalith repositioning maneuvers like the Epley maneuver are relatively simple, noninvasive and effective therapy for individuals with BPPV. Treatment for right sided typical BPPV can be viewed on Youtube at http://www.youtube.com/watch?v=ZqokxZRbJfw. Other canal repositioning maneuvers used to treat individuals with BPPV include the Semont liberatory maneuver and for the less common horizontal canal variant of BPPV, the Lempert or Gufoni maneuver. These maneuvers may have slight variations as well and some of these treatments may be viewed on Youtube at http://www.youtube.com/watch?v=hq-IQWSrAtM.
In some cases, affected individuals may be referred for vestibular rehabilitation therapy (VRT). VRT is the use of specific exercises that are designed to compensate for inner ear deficiencies. While this technique may coincidentally improve BPPV, it is really intended to promote adaptation by the brain to loss of balance function related to inner ear on one side. A physical or occupational therapist will develop a treatment plan tailored to an individual based upon a thorough examination. Basically, affected individuals will perform a series of exercises or postures that over time will lessen their symptoms. Initially, the exercises may temporarily worsen symptoms. However, if affected individuals persist with their instructions, VRT often leads to a decrease in symptom severity or complete disappearance of symptoms. In some cases, no other therapy is necessary.
Some individuals with BPPV may opt for watchful waiting meaning endeavoring no treatment and waiting for symptoms to spontaneously resolve. There seems little point to this since treatment is so quick and easy, however. Furthermore, symptom resolution can take weeks or months in some individuals.
Some affected individuals may receive vestibular suppressant medications (e.g., meclizine or diazepam) that may help relieve certain symptoms of BPPV such as the spinning sensation or nausea. However, drug therapy for BPPV itself is generally ineffective and usually not recommended because canalith repositioning maneuvers are so effective.
In rare cases, individuals with BPPV may be treated with surgery. The frequency of surgery as a treatment for BPPV has dropped in recent years. Surgery for BPPV is reserved for individuals who fail to respond to less invasive treatment options and for whom symptoms are recurrent and problematic (intractable BPPV). The most common procedure used is plugging (occluding) the posterior semicircular canal in order to prevent crystals from causing deflection and stimulation of the cupula. Another procedure, called singular neurectomy, has also been discussed in the medical literature as a potential therapy for intractable BPPV. During a singular neurectomy, a surgeon cuts a specific nerve to the posterior semicircular canal, thereby eliminating the abnormal signals being transmitted from the inner ear. This causes symptoms to improve. However, single neurectomy is a difficult procedure and is rarely performed because it carries a risk of hearing loss. Canal plugging is used more often in the rare cases that require surgical intervention. Although it also has a risk of associated hearing loss, the hearing loss is usually temporary (transient). Surgical therapy is not considered for BPPV except as a last resort.
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Report last updated: 2014/01/28 00:00:00 GMT+0