Secondary Endolymphatic Hydrops

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By Susan Pesznecker, RN; Legacy Clinical Research & Technology Center, Portland, Oregon with the Vestibular Disorders Association

Endolymphatic hydrops is a disorder of controls are believed to be lost or the vestibular system. Although its damaged. This may cause the volume and underlying cause and natural history are concentration of the endolymph to unknown, it is believed to result from fluctuate in response to changes in the abnormalities in the quantity, body’s circulatory fluids and electrolytes. composition, and/or pressure of the endolymph (the fluid within the Symptoms endolymphatic sac, a compartment of the Symptoms typical of hydrops include inner ear). pressure or fullness in the ears (aural fullness), tinnitus (ringing or other noise Causes in the ears), hearing loss, dizziness, and Endolymphatic hydrops may be either imbalance. primary or secondary. Primary idiopathic endolymphatic hydrops (known as Diagnosis and testing Ménière’s disease) occurs for no known There is no vestibular or auditory test reason. Secondary endolymphatic that is diagnostic of endolymphatic hydrops appears to occur in response to hydrops. Diagnosis is clinical—based on an event or underlying condition. For the physician’s observations and on the example, it can follow head trauma or ear patient’s history, symptoms, and surgery, and it can occur with other inner symptom pattern. The clinical diagnosis ear disorders, allergies, or systemic may be strengthened by the results of disorders (such as diabetes or certain tests. For example, certain autoimmune disorders). abnormalities in electrocochleography (which tests the response of the eighth In a normal inner ear, the endolymph is cranial nerve to clicks or tones presented maintained at a constant volume and with to the ear) or audiometry (which tests specific concentrations of sodium, hearing function) may support a hydrops potassium, chloride, and other diagnosis. electrolytes. This fluid bathes the sensory cells of the inner ear and allows them to Treatment goals function normally. In an inner ear Ménière’s disease (primary idiopathic affected by hydrops, these fluid-system endolymphatic hydrops) is discussed in

detail in a separate publication of the Fluctuations in body fluids and electrolyte Vestibular Disorders Association (see the levels may affect the amount and list at the end of this document). In brief, composition of the endolymph, leading to Ménière’s disease is characterized by hydrops symptoms. Stabilizing the fluid sudden, violent attacks or episodes of and electrolyte levels may help reduce or vertigo, tinnitus, hearing loss, and aural relieve the symptoms. Modifications in fullness. The attacks typically occur at diet may be necessary in order to achieve intervals of weeks to months, with such stabilization. symptom-free periods between attacks. Over several years, there is partial A hydrops diet regimen (HDR) often destruction of hearing and sometimes of makes many people with SEH feel balance function. Ménière’s disease affects significantly better without any other both ears in up to half of those who treatment. The HDR is the cornerstone of experience it. Treatment is intended to stabilizing overall fluid levels. The most improve symptoms, manage acute attacks, important aspect of this regimen is deal with the damage to hearing and constancy. Eating a balanced diet in balance, and maintain quality of life. moderate amounts at regular intervals— with meals and snacks of a consistent The treatment of secondary size, eaten at about the same time every endolymphatic hydrops (SEH) is day, without skipping meals or somewhat different. Since SEH is alternating tiny snacks with huge meals— secondary to (that is, results from) an helps the body’s fluid and electrolyte underlying disorder, the symptoms tend levels remain stable. to be present more continuously, rather than occurring in spontaneous attacks. A second key element of the HDR is mini- However, they are often less violent, and mizing the use of solutes (salts and

SEH may cause less damage to hearing and balance than does Ménière’s disease. The five goals of treating secondary endolymphatic Treatment of SEH has five goals: to hydrops stabilize the body’s fluid and electrolyte levels; to identify and treat the 1. Stabilize the body’s fluid and electrolyte levels. underlying condition that is driving the 2. Identify and treat the SEH; to improve daily symptoms; to underlying condition. manage persistent symptoms and 3. Improve daily symptoms. changes; and to maintain quality of life. 4. Manage persistent symptoms Goal 1: Stabilizing the body’s fluid and changes. and electrolyte levels 5. Maintain quality of life.

sugars) in the diet. Solutes require the supplement to replace potassium lost body to use large amounts of fluid for through the urine. dilution and digestion. This causes large fluctuations in body fluids and Goal 2: Identifying and treating the consequently in the endolymph, which underlying condition can trigger hydrops symptoms. An This goal is complex and will likely involve important starting point of this diet is to both the otologist (ear specialist) and the avoid adding salt or sugar to food and to primary care provider (internist, family avoid eating prepared foods (those that practice physician, nurse practitioner, come out of a can or other container). etc.). Once an underlying condition is identified and treated, SEH symptoms Adequate fluid intake is another mainstay tend to improve over time. Hydrops of the HDR. In order to function at its associated with head trauma or ear best, the body needs lots of water—six to surgery usually improves over the course eight glasses spaced evenly throughout of one to two years following the the day. It is also important to anticipate causative event. and replace the additional fluid lost through perspiration during exercise, Goal 3: Improving daily symptoms fever, or hot weather. Other fluids—for With the use of the HDR and possibly also example, low-sugar sodas, herbal teas, a diuretic, balance symptoms may and low-sugar fruit and vegetable improve dramatically. Other medications juices—may also make up part of the may be used to help with persistent daily allotment. Caffeine (found in coffee, dizziness, nausea, or vomiting. tea, some herbal teas, colas, chocolate, and some medications) and alcohol have Other strategies to reduce the symptoms strong diuretic properties and may need of SEH include: to be restricted, because they can cause  Maintaining normal weight, or losing the body to lose more fluid than it has any excess weight. When a person is taken in. overweight, the vestibular system must struggle to deal with a larger- Physicians may prescribe diuretics as part than-normal and displaced center of of treatment—not to cause fluid loss, but gravity. rather to “push” the kidneys to excrete a  Avoiding aspirin in high doses, which constant amount of urine throughout the can cause temporary tinnitus. day, thus helping to minimize large swings  Avoiding ibuprofen and other in the body’s fluid content. With diuretic NSAIDS (nonsteroidal anti- use, drinking lots of water is important, in inflammatory drugs), which can have order to avoid dehydration. Certain a direct effect on fluid balance and diuretics require the use of a potassium may increase symptoms in some SEH patients.

 Stopping smoking. Smoking mechanical changes that prevent constricts the single, tiny artery that abnormal inner ear signals from reaching feeds the ear, thus depriving it of the brain, thus reducing symptoms. oxygen and hemoglobin. These procedures do not cure the  Maintaining general health by getting underlying disorder and are not without adequate exercise and sleep. risk, but they may improve symptoms in some cases. Vestibular rehabilitation, a type of specialized physical therapy for vestibular SEH does not usually result in significant patients, can improve tolerance for hearing loss. If it does occur, modern activity, overall energy level, and hearing aids and other assistive devices symptoms of dizziness and imbalance. may be useful. In addition, tinnitus- The cognitive symptoms that often masking devices can be used to help deal accompany vestibular disorders—for with annoying tinnitus. instance, trouble with concentration, short-term memory, reading, or Very often, people with inner ear prioritizing tasks—may diminish as the disorders attempt to avoid aggravating hydrops is brought under control. their symptoms by restricting their activity and becoming reclusive. This is Goal 4: Managing persistent counterproductive. In fact, remaining as symptoms and changes active and busy as possible (within safe If dizziness and vertigo become limits) helps the brain adjust to changes intractable, more aggressive measures in inner ear function and helps control may be considered. For example, the symptoms. The physician may hair-cell structures of the inner ear may recommend a course of vestibular be selectively destroyed with the careful rehabilitation therapy to help the brain use of ototoxic (ear-poisoning) compensate for changes in balance medication. function.

In rare cases, surgery may be Goal 5: Maintaining quality of life recommended. Endolymphatic As with any chronic disorder, maintaining decompression procedures aim at a healthy outlook and as normal a routine relieving fluid pressure in the inner ear. as possible is essential. Creating a safe Another type of surgery is a physical environment in the home is also labyrinthectomy, which destroys the important, as well as taking into membranous structures of the inner ear consideration whether one should that detect gravity and motion changes. undertake potentially hazardous activities Semi-circular canal plugging and neu- such as driving a car or climbing ladders. rectomy (cutting the vestibular nerve between the ear and the brain) create

As an “invisible” disability, an inner ear  Diagnostic Tests for Vestibular disorder can be frustrating to manage. A Problems person may feel miserable, yet “look  Vestibular Rehabilitation: An normal” to friends and family. Educating Effective, Evidence-Based others about the illness can help them Treatment better understand the difficulties and  Vestibular Injury: Compensation, consequences of having a vestibular dis- Decompensation, and Failure to order. Counseling or participating in a Compensate support group for people with inner ear  Benign Paroxysmal Positional disorders may help to deal with the Vertigo—BPPV confusion or secondary depression that  Dietary Considerations with often accompanies these conditions. In Endolymphatic Hydrops, Ménière’s any case, the physician and the Disease, and Vestibular Migraine physician’s staff remain the patient’s primary resource in understanding and © Vestibular Disorders Association dealing with SEH. VEDA’s publications are protected under Further reading copyright. For more information, see our permissions guide at www.vestibular.org. Some helpful documents available from the Vestibular Disorders Association include the This document is not intended as a substitute following, identified by title and catalog for professional health care. number.  Ménière’s Disease—What You Need to Know

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