Can autoimmune disease cause hearing loss?
Hearing loss caused by autoimmune disease is a relatively rare cause of hearing loss accounting for approximately 1 percent of cases.
How do you treat severe hearing loss caused by steroids?
Treatment. After you take the steroids, your doctor may prescribe a medication that can calm down your immune system. Other drugs like azathioprine ( Imuran ), cyclophosphamide ( Cytoxan ), and methotrexate are sometimes used for this. A hearing aid can help you adjust to hearing loss, but in severe cases,...
Which medications are used to treat autoimmune inner ear disease (IAD)?
Cytotoxic medications such as methotrexate and cyclophosphamide may be effective in treating autoimmune inner ear disease when steroids fail or are not an option, however side effects may limit their use.
What medications are used to treat hearing loss?
Other drugs like azathioprine ( Imuran ), cyclophosphamide ( Cytoxan ), and methotrexate are sometimes used for this. A hearing aid can help you adjust to hearing loss, but in severe cases, your doctor might suggest a cochlear implant.
Can autoimmune ear disease be cured?
When you get treatment for AIED-related hearing loss early on, your hearing can often be restored. If hearing loss is significant, you may need a hearing device. Though AIED has no known cure, with ongoing care and regular monitoring it can often be a manageable condition.
Can autoimmune hearing loss be reversed?
Introduction. Autoimmune inner ear disease (AIED) is a reversible form of sensorineural hearing loss when immunosuppressive treatment is given.
What autoimmune disease can cause hearing loss?
The autoimmune disease most directly connected to hearing is autoimmune inner ear disorder (AIED). In this rare disorder, immune cells attack the inner ear, leading to progressive hearing loss that may fluctuate.
How do you test for autoimmune inner ear?
To diagnose AIED, your doctor will ask questions about your health and medical history, do a physical exam, and give you a hearing test. They'll also test your balance, which can show how well your inner ear is "talking" to your brain. You might also have blood work done.
What is the treatment for autoimmune inner ear disease?
Steroids (dexamethasone, prednisone, prednisolone) are often used for the treatment of AIED. High doses, often in doses of 40-80 gm/day, may be useful in the initial management of AIED.
How long does it take prednisone to work for hearing loss?
It often arises without an obvious cause and occurs in one ear all at once or over a period of up to 3 days. Oral steroids, such as prednisone, are usually prescribed over the course of 2 weeks to restore hearing. There is only a 2- to 4-week window of time for treatment before hearing loss becomes permanent.
What causes autoimmune ear disease?
What Causes AIED? The causes of AIED can include: The body's uncontrolled immune system attacks the inner ear protein, forming immune complexes and antibodies and causing progressive hearing loss in both ears. Cochlin is a protein located in the inner ear that is attacked by the immune system.
Is Meniere's autoimmune disease?
The immune response in Meniere's disease is focused on inner ear antigens. Approximately one-third of Meniere's disease cases seem to be of an autoimmune origin although the immunological mechanisms involved are not clear.
Is tinnitus a symptom of autoimmune disease?
Vestibular symptoms, tinnitus, and aural fullness can be found in patients with systemic autoimmune diseases; they often mimic primary inner ear disorders such as Menière's disease and mainly affect both ears simultaneously.
Is sensorineural hearing loss autoimmune?
Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity characterized by a progressive fluctuating bilateral asymmetric SNHL that develops over several weeks to months. Vestibular symptoms, tinnitus and aural fullness are present in up to 50% of patients.
Can Hashimoto's cause deafness?
Having an autoimmune disease like Hashimoto's increases your risk of developing other autoimmune diseases, including several that can cause hearing loss.
Can autoimmune disease cause sensorineural hearing loss?
Autoimmune sensorineural hearing loss (SNHL) is a rare clinical entity characterized by a progressive fluctuating bilateral asymmetric SNHL that develops over several weeks to months. Vestibular symptoms, tinnitus and aural fullness are present in up to 50% of patients.
Is hearing loss a symptom of lupus?
Conclusion: Aural symptoms are prevalent among patients with lupus. Asymmetric symptoms and hearing loss are most common. The cause may relate to immune-complex disease and/or vasculitis.
Overview
Treatment
- Often the first line of treatment is a course of oral steroid medication such as prednisone, dexamethasone, or even aldosterone. They are usually used for a period of about 1 month. Steroids are usually not used in individuals with diabetes, peptic ulcer disease, glaucoma, or high blood pressure. Steroids are effective about 60 percent of the time. Steroids should never be dis…
- Ultimately, many diagnose AIED by the positive response to steroid treatment. Steroids (dexamethasone, prednisone, prednisolone) are often used for the treatment of AIED. High doses, often in doses of 40-80 gm/day, may be useful in the initial management of AIED. Unfortunately, hearing improvement is rarely sustained and unacceptable side effects from the corticosteroid t…
Causes
- Autoimmune diseases that affect the inner ear are not all well understood, however, they generally involve components of the immune system (immune cells or antibodies) which for unknown reasons begin to attack the structures that make up the inner ear. There are several theories about how this happens but this usually occurs in relation to another co-existing autoimmune di…
- AIED occurs when the body's immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occuring in less than 1% of the 28 million Americans with a hearing loss. It can happen in isolation (as just labyrinthine disease) or as part of other systemic autoimmune disorders. Twenty percent of patients have other autoimmune diseases, such as rh…
Prognosis
- Autoimmunity isnt involved in most cases of vestibular disorders; however, it can cause large losses when continuing unchecked. Effective treatments are available when the diagnosis is timely.
Diagnosis
- If you have symptoms of autoimmune inner ear disease your doctor may use a combination of several tests to help confirm this diagnosis. Here are some of the tests your doctor may choose to order: None of the tests listed above are specific for autoimmune inner ear disease but are used to help rule out or confirm associated conditions. A diagnosis is based on a combination o…
- Diagnosing an autoimmune disorder as the cause of inner ear symptoms can be difficult. To succeed, a physician must have training and experience in these disorders. Most otolaryngologists are not trained or experienced in autoimmune disorders in general, and a rheumatologist trained in autoimmune disorders is unlikely to be highly familiar with vestibular f…
- Because of the difficulty in the differential diagnosis of AIED, many have proposed the use of lab tests to assist in the medical diagnosis. McCabe originally recommended an \"immune screen\" to include Later, Campbell and Klemens (2000) listed other medical tests they commonly use to detect AIED:
Epidemiology
- Hearing loss caused by autoimmune disease is a relatively rare cause of hearing loss accounting for approximately 1 percent of cases.
- In general, autoimmune disorders occur more frequently in women than men and less frequently in children and the elderly. When the ear is attacked, the progression of damage and functional loss is rapid, occurring over weeks to months and usually progressing rapidly to the second ear.
- As mentioned above, 20% of patients have other autoimmune diseases. Here is a list of some of the systemic immune diseases that have been known to have AIED:
Signs And Symptoms
- The most characteristic symptom of autoimmune inner ear disease is sudden hearing loss which usually occurs in both ears (bilaterally). This rapid hearing loss is usually classified as sensorineural and often accompanied by vestibular symptoms such as dizziness or loss of balance. The hearing loss usually occurs over a period of a few months.
- The exact clinical description can vary somewhat; however, most agree that the syndrome follows a fairly typical course. AIED will manifest as rapidly progressing, sensorineural hearing loss that is most commonly bilateral. Typically, it begins unilaterally and ultimately affects the other ear, and it can occur suddenly. Word recognition is usually disproportionately poor. Vestibular symptom…
History
- Brian McCabe, MD, first described autoimmune sensorineural hearing loss in his landmark 1979 paper. McCabe described a series of 18 patients whose \"clinical pattern did not fit with known entities and thus seemed to merit distinctive categorization.\" Since that time, autoimmune inner ear disease (AIED) or immune-mediated sensorineural hearing loss, has taken its place as a cau…
Research
- General immune system research and study of autoimmune disorders is underway in many locations. Scientists are also conducting research related to autoimmune disorders and the inner ear. Most inner ear research has centered on finding an identifiable inner ear \"marker\" or chemical that can be tested for so that diagnosis can be faster and more accurate. Other resear…
Classification
- Hearing loss has been viewed historically as the main inner ear effect of an autoimmune problem, but the vestibular system can also be attacked. Several factors determine the type of vestibular symptoms that may be experienced. Those factors include the speed with which the vestibular loss occurred, the degree of loss, whether one side or both sides are affected, and whether the d…