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Endolymphatic hydrops is a disorder of the vestibular system in the inner ear. It is thought to stem from abnormal fluctuations in the fluid called endolymph which fills the hearing and balance structures of the inner ear. This condition results in a distended endolymphatic space and is referred to as endolymphatic hydrops. Endolymphatic hydrops is referred to as primary or secondary.
Primary (idiopathic) endolymphatic hydrops
Primary idiopathic endolymphatic hydrops, known as Ménière’s disease, occurs for no known reason.
Secondary endolymphatic hydrops
Secondary endolymphatic hydrops occurs in response to an event, such as head trauma, allergy or an underlying condition, such as an autoimmune disorder.
The symptoms of endolymphatic hydrops include tinnitus, dizziness, fluctuating hearing loss, imbalance and a feeling of fullness/pressure in the ear.
Who is affected by endolymphatic hydrops?
Ménière’s disease and endolymphatic hydrops are often used synonymously. Ménière’s disease is idiopathic, meaning that if the cause of vertigo is known the diagnosis cannot be Ménière’s disease. People diagnosed with Ménière’s disease are thought to have endolymphatic hydrops; however not all people diagnosed with endolymphatic hydrops have Ménière’s disease.
How is endolymphatic hydrops diagnosed?
Endolymphatic hydrops is diagnosed clinically by an ENT consultant. The diagnosis will be based on a patient’s medical history, their symptoms and tests and investigations undertaken by the consultant.
Managing symptoms/treating endolymphatic hydrops
As with Ménière’s disease, reducing caffeine and alcohol may be helpful in managing your symptoms. Keeping hydrated and maintaining a balanced diet (lowering salt and sugar intake) is also considered beneficial. Medication may also be given to help control dizziness, nausea, and vomiting, if those symptoms are a problem. Diuretics may also be prescribed. Vestibular rehabilitation can help with problems with balance. Vestibular rehabilitation, or balance retraining, involves carefully practicing movements which make you dizzy. The brain learns to cope with these movements and, in time, they no longer cause dizziness and imbalance. This retraining can reduce dizziness and imbalance between major attacks of vertigo. Lifestyle changes and stress reduction techniques can help as these may also have an impact on your symptoms. In severe cases, your consultant may advise inner ear surgery to relieve the symptoms.
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1. What is endolymphatic hydrops?
Endolymphatic hydrops is a disorder of the vestibular system, which is part of the inner ear. Endolymphatic hydrops consists of abnormal fluctuations in the endolymph fluid, which fills the hearing and balance structures of the inner ear. These fluctuations cause an increase in pressure in the endolymphatic system of the inner ear. Endolymph fluid, which is regulated by the endolymph sac, flows through the inner ear and is critical to hearing. In addition to water, endolymph fluid contains salts such as sodium, potassium, chloride and other electrolytes. If the inner ear is damaged by disease or injury, the volume and composition of the endolymph fluid can also change, causing the symptoms of endolymphatic hydrops.
2. Symptoms of endolymphatic hydrops?
The symptoms of endolymphatic hydrops include the feeling of pressure or fullness in the ears, hearing loss , tinnitus , (ringing in the ears), hearing loss, and balance problems. Individuals who have Meniere’s disease have a very severe form of endolymphatic hydrops, but individuals with endolymphatic hydrops do not always progress to Meniere’s disease.
3. What are the causes of endolymphatic hydrops?
Endolymphatic hydrops may occur as a result of trauma such as a blow to the head, infection, degeneration of the inner ear, allergies, dehydration and loss of electrolytes or in rare circumstances a benign tumor. In many cases, it is not clear what causes the disorder. Meniere’s attacks occur when there is an increase in endolymphatic pressure in the inner ear, causing a leak in the membrane separating the perilymph (potassium poor extracellular fluid) and the endolymph (potassium rich intracellular fluid). The mix of these two fluids surrounding the vestibular nerve receptors creates an electrical blockade and a loss of function. The sudden change in the rate of the vestibular nerve firing creates acute vestibular imbalance otherwise known as vertigo.
4. What is the treatment for endolymphatic hydrops?
A specialized interdisciplinary team of professionals is necessary to treat all of the issues associated with Endolymphatic hydrops. This group of individuals should include an Otologist or Otolaryngologist, and an audiologist, and possibly a vestibular rehabilitation expert.
Over our forty-year history, CEI neurotologists have developed and refined a three level treatment strategy for patients with Endolymphatic hydrops. The three levels begin with dietary and lifestyle modifications, then medication, and in some cases, a physical therapy and exercise program. In those patients who fail to respond to first measures, more aggressive treatment is needed and may include surgery to stop the attacks of vertigo. Over 90 % of CEI patients successfully have their vertigo controlled with this strategy.
The California Ear Institute is a leading global center for otological, otolaryngological and audiological treatment. Dr. Joseph Roberson , a board certified neurotologist, has performed thousands of successful hearing related surgeries, including many on patients whose hearing impairment is the result of Endolymphatic hydrops.
5. What are the consequences of avoiding treatment?
Patients must seek medical treatment for any change in hearing or balance regardless of whether or not Endolymphatic hydrops is suspected to be the source of the hearing loss. Evaluation for beneficial drug treatment or surgery cannot be established without a comprehensive medical examination and associated testing. Avoiding medical evaluation for Endolymphatic hydrops or any other hearing or balance impairment related condition is not recommended, and can lead to permanent hearing loss.
Patients searching the internet for information about Endolymphatic hydrops or any other medical issue should know when reading about individual case histories, that generally it is the patients with the most severe symptoms who either post their own experiences or are included in medical review journals. As a result, it is easy to become overly alarmed and assume the worst. Patients should keep this in mind as they explore the available resources, and look for sources that are certified by HON or other reputable health reviewing organizations. As with all medical conditions, prompt treatment by experienced medical personnel give the best chance for a positive outcome.
If you suspect that you have Endolymphatic Hydrops, or you have noticed a change in your hearing, consult a CEI otologist. The outlook for treatment is excellent, once the diagnosis has been made and appropriate treatment is initiated.
Click here to make an appointment with the California Ear Institute to consult with one of our board certified otologists regarding your ear-related condition.
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Secondary Endolymphatic Hydrops (SEH)
What is Endolymphatic Hydrops?
Endolymphatic hydrops is a disorder of the inner ear and can affect the endolymphatic fluid of the cochlea, the vestibular apparatus, or both. Although its underlying cause and natural history are unknown, it is believed to result from abnormalities in the quantity, composition, and/or pressure of the endolymph (the fluid within the endolymphatic sac, a compartment of the inner ear).
In a normal inner ear, the endolymph is maintained at a constant volume and with specific concentrations of sodium, potassium, chloride, and other electrolytes. This fluid bathes the sensory cells of the inner ear and allows them to function normally. In an inner ear affected by hydrops, these fluid-system controls are believed to be lost or damaged. This may cause the volume and concentration of the endolymph to fluctuate in response to changes in the body’s circulatory fluids and electrolytes.
Endolymphatic hydrops may be either primary or secondary. Primary idiopathic endolymphatic hydrops (known as Ménière’s disease) occurs for no known reason. Secondary endolymphatic hydrops appears to occur in response to an event or underlying condition. For example, it can follow head trauma or ear surgery, and it can occur with other inner ear disorders, allergies, or systemic disorders (such as diabetes or autoimmune disorders).
Symptoms typical of hydrops include pressure or fullness in the ears (aural fullness), tinnitus (ringing or other noise in the ears), hearing loss, dizziness, and imbalance.
DIAGNOSIS AND TESTING
Diagnosis is often clinical—based on the physician’s observations and on the patient’s history, symptoms, and symptom pattern. The clinical diagnosis may be strengthened by the results of certain tests. For example, certain abnormalities in electrocochleography (which tests the response of the eighth cranial nerve to clicks or tones presented to the ear) or audiometry (which tests hearing function) may support a hydrops diagnosis. New research has shown that MRI with contrast in the inner ear can give a definitive diagnosis of endolymphatic hydrops, but likely would not be able to differentiate between primary (Meniere’s) and secondary. This is most commonly not used because clinical diagnosis is often accurate.
Ménière’s disease (primary idiopathic endolymphatic hydrops) is discussed in detail in a separate publication of the Vestibular Disorders Association (see the list at the end of this document). In brief, Ménière’s disease is characterized by sudden, violent attacks or episodes of vertigo, tinnitus, hearing loss, and aural fullness. The attacks typically occur at intervals of weeks to months, with symptom-free periods between attacks. Over several years, there is partial destruction of hearing and sometimes of balance function. Ménière’s disease affects both ears in up to half of those who experience it. Treatment is intended to improve symptoms, manage acute attacks, deal with the damage to hearing and balance, and maintain quality of life.
The treatment of secondary endolymphatic hydrops (SEH) is somewhat different. Since SEH is secondary to (that is, results from) an underlying disorder, the symptoms tend to be present more continuously, rather than occurring in spontaneous attacks. However, they are often less violent, and SEH may cause less damage to hearing and balance than does Ménière’s disease.
Treatment of SEH has five goals: to stabilize the body’s fluid and electrolyte levels; to identify and treat the underlying condition that is driving the SEH; to improve daily symptoms; to manage persistent symptoms and changes; and to maintain quality of life.
Goal 1: Stabilizing the body’s fluid and electrolyte levels
Stabilizing the fluid and electrolyte levels may help reduce or relieve the symptoms. Modifications in diet may be necessary in order to achieve such stabilization.
A hydrops diet regimen (HDR) often makes many people with SEH feel significantly better without any other treatment. The HDR is the cornerstone of stabilizing overall fluid levels. The most important aspect of this regimen is consistency. Eating a balanced diet in moderate amounts at regular intervals—with meals and snacks of a consistent size, eaten at about the same time every day, without skipping meals or alternating tiny snacks with huge meals—helps the body’s fluid and electrolyte levels remain stable.
A second key element of the HDR is minimizing the use of solutes (salts and sugars) in the diet. Solutes require the body to use large amounts of fluid for dilution and digestion. This causes large fluctuations in body fluids and consequently in the endolymph, which can trigger hydrops symptoms. An important starting point of this diet is to avoid adding salt or sugar to food and to avoid eating prepared foods (those that come out of a can or other container).
Adequate fluid intake is another mainstay of the HDR. In order to function at its best, the body needs lots of water—six to eight glasses spaced evenly throughout the day. It is also important to anticipate and replace the additional fluid lost through perspiration during exercise, fever, or hot weather. Other fluids—for example, low-sugar sodas, herbal teas, and low-sugar fruit and vegetable juices—may also make up part of the daily allotment. Caffeine (found in coffee, tea, some herbal teas, colas, chocolate, and some medications) and alcohol have strong diuretic properties and may need to be restricted, because they can cause the body to lose more fluid than it has taken in.
Physicians may prescribe diuretics as part of treatment, not to cause fluid loss, but rather to “push” the kidneys to excrete a constant amount of urine throughout the day, thus helping to minimize large swings in the body’s fluid content. With diuretic use, drinking lots of water is important to avoid dehydration. Certain diuretics require the use of a potassium supplement to replace potassium lost through the urine. It is important to discuss proper use of medication and intake of food and water with the prescribing physician.
Goal 2: Identifying and treating the underlying condition
This goal is complex and will likely involve both the otologist (ear specialist) and the primary care provider (internist, family practice physician, nurse practitioner, etc.). Once an underlying condition is identified and treated, SEH symptoms tend to improve over time with proper management. Hydrops associated with head trauma or ear surgery usually improves over the course of one to two years following the causative event.
Goal 3: Improving daily symptoms
With the use of the HDR and possibly also a diuretic, balance symptoms may improve dramatically. Other medications may be used to help with persistent dizziness, nausea, or vomiting.
Other strategies to reduce the symptoms of SEH include:
- Maintaining normal weight, or losing any excess weight. When a person is overweight, the vestibular system must struggle to deal with a larger-than-normal and displaced center of gravity.
- Avoiding aspirin in high doses, which can cause temporary tinnitus.
- Avoiding ibuprofen and other NSAIDS (nonsteroidal anti-inflammatory drugs), which can have a direct effect on fluid balance and may increase symptoms in some SEH patients.
- Stopping smoking. Smoking constricts the single, tiny artery that feeds the ear, thus depriving it of oxygen and nutrients.
- Maintaining general health by getting adequate exercise and sleep, both can help with cardiovascular health to support the inner ear. Exercise can help the musculature needed for balance stay active.
Vestibular rehabilitation, a type of specialized physical therapy for vestibular patients, can improve tolerance for activity, overall energy level, and symptoms of dizziness and imbalance. The cognitive symptoms that often accompany vestibular disorders—for instance, trouble with concentration, short-term memory, reading, or prioritizing tasks—may diminish as the hydrops is brought under control, but occupational and speech therapists can help if any symptoms remain.
Goal 4: Managing persistent symptoms and changes
If dizziness and vertigo become intractable, more aggressive measures may be considered. Medications or surgery can be used to selectively destroy the problematic inner ear structures.
There are various surgeries that may be recommended. Endolymphatic decompression procedures aim at relieving fluid pressure in the inner ear. Another type of surgery is a labyrinthectomy, which destroys the membranous structures of the inner ear that detect gravity and motion changes. Semi-circular canal plugging and neurectomy (cutting the vestibular nerve between the ear and the brain) create mechanical changes that prevent abnormal inner ear signals from reaching the brain, thus reducing symptoms. These procedures do not cure the underlying disorder and are not without risk, but they may improve symptoms in some cases.
SEH does not usually result in significant hearing loss. If it does occur, modern hearing aids and other assistive devices may be useful. In addition, tinnitus-masking devices can be used to help deal with annoying tinnitus.
Very often, people with inner ear disorders attempt to avoid aggravating their symptoms by restricting their activity and becoming reclusive. This is counterproductive. In fact, remaining as active and busy as possible (within safe limits) helps the brain adjust to changes in inner ear function and helps control symptoms. The physician may recommend a course of vestibular rehabilitation therapy to help the brain compensate for changes in balance function.
Goal 5: Maintaining quality of life
As with any chronic disorder, maintaining a healthy outlook and as normal a routine as possible is essential. Creating a safe physical environment in the home is also important, as well as taking into consideration whether one should undertake potentially hazardous activities such as driving a car, climbing ladders, or participating in sports.
As an “invisible” disability, an inner ear disorder can be frustrating to manage. A person may feel miserable, yet “look normal” to friends and family. Educating others about the illness can help them better understand the difficulties and consequences of having a vestibular disorder. Counseling or participating in a support group for people with inner ear disorders may help to deal with the confusion or secondary depression that often accompanies these conditions. In any case, the physician and the physician’s staff remain the patient’s primary resource in understanding and dealing with SEH.
Authors: Susan Pesznecker, RN with the Vestibular Disorders Association. Edits by Jeremy Hinton, DPT.
Some helpful documents available from the Vestibular Disorders Association include the following:
- Diagnostic Tests for Vestibular Problems
- Vestibular Rehabilitation: An Effective, Evidence-Based Treatment
- Vestibular Injury: Compensation, Decompensation, and Failure to Compensate
- Benign Paroxysmal Positional Vertigo—BPPV
- Dietary Considerations with Endolymphatic Hydrops, Ménière’s Disease, and Vestibular Migraine
Click here to download the “Secondary Endolymphatic Hydrops” publication.
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