PRIMER ON VERTIGO

**A Short Primer on Vertigo

**Vertigo refers to the spinning symptom. Vertigo occurs when there is an acute loss of vestibular function (with accompanying hearing loss, such as in Meniere’s Disease or Sudden Hearing Loss with Vertigo or without hearing loss in Vestibular Neuritis) or in cases of over stimulation of vestibular function such as in Positional Vertigo. Sudden Hearing Loss was discussed in a previous Newsletter. This issue will describe the other entities.

**The Vestibular System refers to the balance system, which involves the inner ear balance organs (semicircular canals, and the utricle and saccule which are located in the vestibule of the inner ear), the Vestibular Nerve, which exits the inner ear and ends in the brain stem, and the central connections of the system.

The Diagnosis and Treatment of Dizziness and Imbalance

This page focuses on dizziness and imbalance as they relate to disorders of the inner ear and its immediate central nervous system connections. Certain terms must be understood, particularly the term “Vestibular”. This refers to the vestibular system, which is the system responsible for maintaining balance and spatial orientation. Symptoms of dizziness and imbalance, more often than not, are related to disorders of this system. “Inner Ear” refers to that portion of the ear, which contains the nerve receptors for balance and hearing. Information from the inner ear is forwarded to the brain through the “Eighth Nerve”, which is made up by two nerves, the nerve of balance (Vestibular Nerve) and the nerve of hearing (Cochlear Nerve).
Dizziness related to problems with the vestibular system, which is the system related to balance function, can be described by patients in several ways. The symptoms will lead to evaluation of these patients for specific treatable disorders, which will be described below.

The Spinning Symptom - Patients may experience the spinning symptom from four specific ear disorders and one non-specific disorder.

**Positional Vertigo-This disorder arises from the inner ear, and is directly caused by small calcium-type particles or clots of particles or inner ear debris, which move around in the fluids of the inner ear. The spinning usually occurs when the patient lies down in bed or gets up from bed. It can also occur after bending or turning. It is violent and frightening, and is usually of short duration. The patient may become nauseated and may vomit, and is often frightened enough to seek medical attention from their doctor or from a hospital Emergency Room. The condition may improve without treatment over a period of days, weeks or even months. Treatment is available at any stage of this disorder. The treatment is a positioning maneuver, in which the head is turned, non-violently, into a position in which the particles or clot will be removed from the part of the inner ear that is affected. The patient must stay relatively upright for 48 hours after the treatment. Ninety percent of patients improve, with most being permanently cured of this condition. There may be evidence, in some patients, of a possible problem in the brain that is the cause of the positional vertigo. Appropriate tests would then be recommended. These cases, fortunately, are few in number. Predisposing causes of inner ear positional vertigo can be sinus infections and head trauma. This condition is in the balance portion of the inner ear and not in the hearing portion and, therefore, there is no hearing loss associated with it.

**Vestibular Neuritis-This disorder arises from an inflammation in the Vestibular Nerve itself. The spinning symptom comes on suddenly, and is quite severe. It is usually accompanied by nausea, and often by vomiting. The patient cannot move at all without feeling dizzy. Dizziness occurs with any movement, and is not related to position. Dizziness can be present even when the patient is lying quietly in bed, as opposed to positional vertigo, which occurs with a change in the position of the head. This disorder may last from 24 hours to days and even weeks. It is treated with cortisone-type drugs. There is no hearing problem associated with Vestibular Neuritis, which is confined to only the balance portion of the Eighth Nerve.

**Meniere’s Disease - This disorder arises in the entire inner ear, so that hearing loss is associated with the dizziness. The dizziness is described as spinning, and is violent. Nausea occurs with the dizziness, and vomiting occurs frequently. The symptoms of tinnitus (ringing in the ear) and ear fullness are usually associated with the disease. The disease is usually confined to one ear, but may involve both ears in about 10% of cases. The dizziness and associated symptoms can last for hours, but the attack usually ends within a days’ time. Patients may experience attacks with various frequencies. Attacks can occur several times a week in the most severe cases. The patient is completely incapacitated during an attack, and fearful of attacks, especially if they are occurring frequently. A medical evaluation is necessary to detect any evidence of a general medical problem, such as thyroid disease, high blood cholesterol and triglycerides, syphilis, a pre-diabetic state or a history of head trauma. The specific problem in the ear is a swelling of one of the two inner ear fluid compartments, in this case, the endolymph. Treatment is directed at any specific underlying disorder or disease and at the swelling of the endolymph. The swelling can be treated with diuretics, dilute histamine or surgery to decompress the endolymphatic space. Other surgical procedures are available for use in specific situations, with the aim of destroying the abnormal balance organ that is causing the attacks. This might also be accomplished by an office procedure, in which an antibiotic, which is toxic to the balance organ, is instilled into the middle ear and allowed to penetrate into the inner ear. Gentamycin and Streptomycin are the drugs most frequently used for this purpose. Pumps to deliver these drugs into the inner ear are now being employed for carefully selected patients. This technique requires a surgical procedure for the implantation, which is usually maintained for 10 to 14 days.

**If there are questions concerning these diseases, please call our office at 201-837-2174 or 201-445-2900. Patients referred for evaluation may also call these numbers.

Recurrent attacks of spinning without hearing loss - Fortunately, this is an unusual condition. It is difficult to diagnose and treat. It may be an atypical Meniere’s Disease, a migraine equivalent, an exacerbation of a chronic mild imbalance, a metabolic dysfunction or be from an unknown cause. A complete evaluation is performed, but may not be diagnostic. It may be necessary to actually observe an attack in order to facilitate a diagnosis.

Non-Spinning Symptoms - Patients with vestibular disorders may experience such non-spinning symptoms as mild or severe imbalance, lightheadedness which can be severe enough to make the patient feel faint (some patients have reported actually fainting), fuzziness or blurring of vision (some patients have reported seeing double), clogging of the ears, pain in the ears, disorientation and motion sickness. There can be associated symptoms of difficulty with memory and concentration. Patients will often have difficulty with dizziness and disorientation in wide-open spaces, such as malls or in crowded spaces, such as stores and supermarkets. Nausea is uncommon, and vomiting is rare.
Some patients feel that they are tilting and that the floor is uneven. Many patients do have difficulty walking on uneven surfaces. Hearing loss and tinnitus can occur in some of these patients. There are two specific disorders that account for over 90% of such cases.

The Nucleo-Reticular Vestibular Syndrome (NRVS)-This syndrome was described by Italian ear specialists, and is not well known in the USA. The symptoms are confined to the vestibular system. There is no hearing loss or tinnitus present, although patients may experience ear fullness along with the symptoms of mild imbalance, lightheadedness and disorientation. Spinning is unusual and, if present, is not a major symptom. NRVS usually comes on gradually, but can follow an episode of Vestibular Neuritis. It can be caused by head or neck trauma, but can occur spontaneously, most probably from a viral cause in these cases. Careful physical examination will reveal mild imbalance on the Quix test, a test for imbalance performed by having the patient stand with feet together, chin up, eyes closed and with the arms outstretched with index fingers pointing outwards. The doctor looks for imbalance, particularly a lateral sway, on this test. The accuracy of the test is improved by testing the patient after lying down, as the transition from lying down to standing up stresses the vestibular system. The hearing is checked (and is unaffected by this disorder), fistula testing (a test performed by presenting positive and negative pressures to the ear canal) is performed as well as a special test of the Stapedial reflex (a reflex test, which involves examination of the brain stem pathway of the vestibular nerve). This test, described as the Brain Stem Arc Study (BSAS), evaluates the latency, amplitude and slope of the reflex as per the method of Professor Bosatra of Italy (developed in the 1970s). If NRVS is diagnosed, treatment with medication (antiserotonin) is begun (with an 80-85% cure rate). Patients, who do not improve, can be given other medications or referred for Physical Therapy, but the rate of improvement is lower in this group. Patients, who do not improve, are routinely re-evaluated, particularly for the Perilymphatic Fistula Syndrome in which the vestibular symptoms are often identical to NRVS.

Perilymphatic Fistula Syndrome (PLF)
Perilymphatic fistulas can be described as a leak of fluid from the inner ear to the middle ear through a weak spot in the thin, bony wall between these two areas. The fluid in question is perilymph, one of the two inner ear fluids, the other being endolymph. Each is in a separate compartment in the inner ear. Perilymph (peri for perimeter) is in the outer compartment, and is therefore closer to the middle ear (which is outside the inner ear). Endolymph is in the inner compartment and is in its own space, a closed space, which does not communicate with the outside or even with internal spaces in the body. Perilymph does not normally communicate with the outside, but does communicate with the body internally, specifically with the cerebrospinal fluid space through a duct (cochlear aqueduct) between inner ear perilymph space and the cerebrospinal fluid space. The vestibular symptoms of a leak of perilymph, a perilymphatic fistula, are similar to those described above for NRVS. This is a non-spinning type of dizziness. However, perilymph fistula patients may also suffer hearing loss, which may even be the dominant and presenting symptom.

The symptoms caused by a leak of perilymph result from a distortion of the inner ear space. As the perilymph leaks, the endolymphatic space swells to fill the space left by the leaking perilymph. This results in distortion of the membranes between the spaces and a disturbance of function of the nerve cells that are in the membranes between these compartments, the nerve cells concerned with hearing and balance.

The diagnosis can be made based upon the presence of the swollen endolymph (Endolymphatic Hydrops) by performing certain tests or by viewing the leaking erilymph at exploratory surgery or by office examination with a fine endoscope to visualize the wall between the middle and inner ears. The causes of this condition may be traumatic (about half the cases) or non-traumatic (the other half). Events such as motor vehicle accidents, in which head or neck trauma may occur can be associated with this condition. Barometric trauma, such as that induced during SCUBA diving can also cause this condition. Internal increases in ear pressure can occur during weight lifting and severe sneezing (especially with the mouth closed) and be associated with perilymphatic fistulas. Perilymphatic fistulas can be a complication of middle ear surgery and direct external trauma to the ear.

Treatment includes observation, as spontaneous healing can occur. The patient is often initially advised to avoid causing internal pressures and to sleep with the head elevated (so as to decrease the cerebrospinal and perilymph pressure). Patients with severe hearing loss should have a shorter period of observation (7-10 days at most), as hearing loss in these cases is more likely to be a permanent condition than the vestibular symptoms. Patients with vestibular symptoms can be observed for three months to allow for spontaneous healing. Surgical repair of perilymphatic fistulas is indicated when the symptoms persist. Treatment with a diuretic may benefit some patients who prefer a non-surgical treatment, but this is not a cure. Physical Therapy directed at restoring balance can be utilized at any stage of the treatment, and can be quite helpful to the patient.

Perilymphatic Hypertension refers to a syndrome in which the inner ear fluids are distorted, but opposite in direction to the Perilymphatic Fistula Syndrome. The symptoms are similar. Treatment is usually medical, with medication that depletes the perilymph, which, in this case, is swollen (rather than the endolymph swelling in perilymphatic fistulas).

Imbalance may occur without the associated vestibular symptoms described above. The vestibular system should be evaluated in these cases. An evaluation of the central nervous system (neurological evaluation) may also be necessary.

Further information can be obtained by calling the practice offices at 201-837-2174 or 201-445-2900. One can inquire by mail at 1 DeGraw Square Teaneck, NJ 07666 or 44 Godwin Avenue Midland Park, NJ 07432. Calls or letters should be addressed to Dr. Joel F. Lehrer, M.D., F.A.C.S.