What Autoimmune Disease Causes Hearing Loss

What Causes Susac Syndrome

Treatment of Sensorineural Hearing Loss – Dr. Girish Rai

Susac syndrome is the result of your own immune system attacking endothelial cells the cells that line the inner walls of your blood vessels in your brain, retina and inner ear. When attacked, the endothelial cells swell and can partially or completely shut off blood flow through the vessel. The blockage prevents needed nutrients and oxygen from reaching these areas.

Its not known what causes your immune system to attack the endothelial cells.

Arthritis And Hearing Loss

People with arthritis may experience hearing loss due to the disease or to the drugs used to treat it.

Several studies, including a 2018 study in Clinical Rheumatology, found higher rates of sensorineural hearing loss a type of hearing loss usually caused by poor function of the hair cells in the cochlea in patients with rheumatoid arthritis . Some evidence also links hearing loss with psoriatic arthritis , juvenile idiopathic arthritis , systemic lupus erythematosus and Sjogrens syndrome.;

Medication-Related Causes

For most people with arthritis the likely culprits behind hearing problems are pain medications.

High doses of aspirin and other salicylates may cause reversible hearing loss; or ringing in the ears . A 2010 study published in the American Journal of Medicine and a 2012 study in the American Journal of Epidemiologyconfirmed the risks of aspirin and NSAIDs on hearing and the risks of other NSAIDs and acetaminophen on hearing as well.

The 2010 study, which examined data from nearly 27,000 men, found that regular; aspirin use increased the chances of hearing loss by 50%, and regular NSAID use made hearing loss 61%;more likely in men younger than 50. Among regular users of acetaminophen, the likelihood of hearing loss was doubled.;

In both studies regular use was defined as at least twice a week and the link between analgesic use and hearing loss was generally greater among those younger than 50.;;

How Medications Hurt Hearing

Disease-Related Causes

What You Can Do

How Can You Prevent Hearing Loss

Avoid loud noise, such as noise from machines at your work, power tools, very loud music, and very loud motorcycles.
Turn down the volume on anything that you listen to through ear buds or headphones, such as MP3 players.
Wear hearing protection, such as earplugs or earmuffs.
Avoid putting objects in your ear.

What Is The Prognosis For Someone With Susac Syndrome

Your experience with Susac syndrome is unique to you. Some people have symptoms only once in their life. Others have symptoms that appear and disappear several times and then completely go away. In most people, symptoms come and go over two to four years on average. A small percentage of people have long-term, continuous flare-ups and remissions of their symptoms.

Most people recover from Susac syndrome with no or few long-term symptoms. However, some people continue to have problems with brain function , and may have some degree of hearing and vision loss. A cochlear implant may help some people with severe hearing loss.

Tests That Diagnose Autoimmune Diseases

Current Menieres Disease Research What You Should Know

No single test can diagnose most autoimmune diseases. Your doctor will use a combination of tests and a review of your symptoms and physical examination to diagnose you.

The antinuclear antibody test is often one of the first tests that doctors use when symptoms suggest an autoimmune disease. A positive test means you may have one of these diseases, but it wont confirm exactly which one you have or if you have one for sure.

Other tests look for specific autoantibodies produced in certain autoimmune diseases. Your doctor might also do nonspecific tests to check for the inflammation these diseases produce in the body.

BOTTOM LINE: A positive ANA blood test may be indicative of an autoimmune disease. Your doctor can use your symptoms and other tests to confirm the diagnosis.

Treatments are also available to relieve symptoms like pain, swelling, fatigue, and skin rashes.

Eating a well-balanced diet and getting regular exercise may also help you feel better.

BOTTOM LINE: The main treatment for autoimmune diseases is with medications that bring down inflammation and calm the overactive immune response. Treatments can also help relieve symptoms.

More than 80 different autoimmune diseases exist. Often their symptoms overlap, making them hard to diagnose.

Autoimmune diseases are more common in women, and they often run in families.

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Why Does The Immune System Attack The Body

Doctors dont know exactly what causes the immune-system misfire. Yet some people are more likely to get an autoimmune disease than others.

According to a 2014 study, women get autoimmune diseases at a rate of about 2 to 1 compared to men 6.4 percent of women vs. 2.7 percent of men. Often the disease starts during a womans childbearing years .

Some autoimmune diseases are more common in certain ethnic groups. For example, lupus affects more African-American and Hispanic people than Caucasians.

Certain autoimmune diseases, like multiple sclerosis and lupus, run in families. Not every family member will necessarily have the same disease, but they inherit a susceptibility to an autoimmune condition.

Because the incidence of autoimmune diseases is rising, researchers suspect environmental factors like infections and exposure to chemicals or solvents might also be involved.

A Western diet is another suspected risk factor for developing an autoimmune disease. Eating high-fat, high-sugar, and highly processed foods is thought to be linked to inflammation, which might set off an immune response. However, this hasnt been proven.

A 2015 study focused on another theory called the hygiene hypothesis. Because of vaccines and antiseptics, children today arent exposed to as many germs as they were in the past. The lack of exposure could make their immune system prone to overreact to harmless substances.

How Is Mnires Disease Treated

Ménières disease does not have a cure yet, but your doctor might recommend some of the treatments below to help you cope with the condition.

Medications. The most disabling symptom of an attack of Ménières disease is dizziness. Prescription drugs such as meclizine, diazepam, glycopyrrolate, and lorazepam can help relieve dizziness and shorten the attack.

Salt restriction and diuretics. Limiting dietary salt and taking diuretics help some people control dizziness by reducing the amount of fluid the body retains, which may help lower fluid volume and pressure in the inner ear.

Other dietary and behavioral changes. Some people claim that caffeine, chocolate, and alcohol make their symptoms worse and either avoid or limit them in their diet. Not smoking also may help lessen the symptoms.

Cognitive therapy. Cognitive therapy is a type of talk therapy that helps people focus on how they interpret and react to life experiences. Some people find that cognitive therapy helps them cope better with the unexpected nature of attacks and reduces their anxiety about future attacks.

Injections. Injecting the antibiotic gentamicin into the middle ear helps control vertigo but significantly raises the risk of hearing loss because gentamicin can damage the microscopic hair cells in the inner ear that help us hear. Some doctors inject a corticosteroid instead, which often helps reduce dizziness and has no risk of hearing loss.

Pressure pulse treatment.

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What Is Mnires Disease

Ménières disease is a disorder of the inner ear that causes severe dizziness , ringing in the ears , hearing loss, and a feeling of fullness or congestion in the ear. Ménières disease usually affects only one ear.

Attacks of dizziness may come on suddenly or after a short period of tinnitus or muffled hearing. Some people will have single attacks of dizziness separated by long periods of time. Others may experience many attacks closer together over a number of days. Some people with Ménières disease have vertigo so extreme that they lose their balance and fall. These episodes are called drop attacks.Ménières disease can develop at any age, but it is more likely to happen to adults between 40 and 60 years of age. The National Institute on Deafness and Other Communication Disorders estimates that approximately 615,000 individuals in the United States are currently diagnosed with Ménières disease and that 45,500 cases are newly diagnosed each year.

Obstructions In The Middle Ear

Autoimmune Inner Ear Disease (AIED) – Boys Town Ear, Nose & Throat Institute

Blockages in the ear canal can cause pressure to build up in the inner ear, affecting the operation of the ear drum. Moreover, objects directly touching the ear drum can irritate the organ and cause the perception of tinnitus symptoms. Common obstructions include:

Excessive ear wax
Loose hair from the ear canal
Dirt or foreign objects

In many cases, the removal of the blockage will alleviate tinnitus symptoms. However, in some situations, the blockage may have caused permanent damage that leads to chronic tinnitus.

Exposure To Therapeutic Drugs

Various chemicals and drugs adversely affect the auditory system; the main ones in clinical use are aminoglycoside antibiotics and cisplatin, both of which are toxic to sensory hair cells. Hearing loss develops in approximately 20% of patients receiving aminoglycosides,, and the prevalence is as high as 56% among patients with cystic fibrosis,, a population exposed to repeated courses of aminoglycoside therapy. Among adults who have received cisplatin, clinically significant hearing loss develops in approximately 60% of patients with testicular cancer and 65% of patients with head and neck cancer. Susceptibility to cisplatin-induced hearing loss depends on the cumulative dose of the drug, the age of the patient , and status with respect to concurrent cranial irradiation. Patients who have severe hearing loss caused by ototoxic drugs are likely to be identified and referred for follow-up auditory testing, but many more patients with mild-to- moderate drug-induced hearing loss are not identified and hence do not receive treatment for their hearing loss.

Treating Tinnitus Caused By Lupus

If you have tinnitus, it is likely you also have hearing loss. Your doctor or a specialist should test your hearing.1

Many treatments that are used for SNHL in people with lupus are also used for tinnitus. These include:1,10-12

Corticosteroids. These can reduce vasculitis and keep the immune system from attacking the inner ears.
Immunosuppressants and monoclonal antibodies. These drugs can also keep the immune system in check.
Plasma transfer . This process removes the antibodies that attack phospholipids from the blood.
Hearing aids and cochlear implants. These devices can help someone hear over the background noise of tinnitus.

Some people have also found acupuncture, which is not used for hearing loss, helpful.13

Unfortunately, tinnitus is not just a physical disease. It can also be a mental health concern for those who find it distressing. Cognitive behavioral therapy can help people emotionally cope with tinnitus.4,14

Finally, antidepressants can help people living with tinnitus feel less depressed and anxious. A doctor will need to go over your symptoms carefully to find the right antidepressant. Some can make tinnitus sounds louder or more noticeable. Others can worsen other lupus symptoms like dry eye and mouth, fatigue, and light sensitivity.15-17

If you are experiencing lupus and tinnitus, talk to your doctor about what treatment may be right for you.

Burden Of Hearing Loss

The Global Burden of Disease Study measured years lived with disability and found that hearing loss is the fourth leading cause of disability globally. In the United States, the prevalence of hearing loss doubles with every 10-year increase in age. Approximately half of persons in their seventh decade and 80% who are 85 years of age or older have hearing loss that is severe enough to affect daily communication. Because of the aging population in this and other developed countries, hearing loss is likely to become an increasingly prevalent disability.

Living With Hearing Loss

If you have hearing loss, you may find that it takes extra effort and energy to talk with others. Hearing may be especially difficult in settings where there are many people talking or there is a lot of background noise. The increased effort it takes to be with other people may cause stress and fatigue. You may begin to avoid social activities, feel less independent, and worry about your safety.

Hearing devices you may want to use include:

Hearing aids.Hearing aids make all sounds louder , including your own voice. Common background noises, such as rustling newspapers, magazines, and office papers, may be distracting. When you first get hearing aids, it may take you several weeks to months to get used to this.
Hearing Loss: Should I Get Hearing Aids?

Assistive listening devices. These devices make certain sounds louder by bringing the sound directly to your ear. They shorten the distance between you and the source of sound and also reduce background noise. You can use different types of devices for different situations, such as one-on-one conversations and classroom settings or auditoriums, theatres, or other large public spaces. Commonly used listening devices include telephone amplifiers, personal listening systems , and hearing aids that you can connect directly to a television, stereo, radio, or microphone.

Alerting devices. These devices alert you to a particular sound by using louder sounds, lights, or vibrations to get your attention.

How Is Susac Syndrome Treated

Susac syndrome is best treated as soon as possible with an aggressive, long-term course of drugs that suppress the immune system. Medications may include:

Prednisone or other corticosteroids.
Azathioprine.
Biological therapies, such as rituximab , etanercept , infliximab .

Women should avoid oral contraceptives and estrogen-replacement therapies because certain hormones may increase the risk of blood vessel blockage.

Ear & Hearing Appointments

Our Otologist/Neurotologist specializes in the medical and surgical care of patients with disorders of the ear and lateral skull base. Otology/Neurotology and Audiology work together to offer complete hearing health solutions.

To schedule an appointment with our Ear & Hearing department, please call us at 795-7750.

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What Research Does The Nidcd Support On Sudden Deafness

Since so little is known about the causes of most cases of SSHL, researchers are considering different types, risk factors, and causes of SSHL. For instance, researchers are studying how changes in the inner ear, such as disrupted blood flow or inflammation, may contribute to hearing loss. Researchers are also testing new ways to use imaging to help diagnose SSHL and potentially detect its causes.

NIDCD-funded researchers are also trying to improve ways of dispensing drugs into the inner ear by intratympanic injections. Scientists are developing ways to infuse drugs into tiny microspheres that can slowly release the drug. This would allow doctors to give a single injection of a slow-releasing drug into the ear rather than several injections of a traditional fast-releasing drug. Another team of scientists is studying the use of magnets to push drug-infused particles into and throughout the inner ear, distributing the drug more evenly and effectively. Visit the NIH Clinical Research Trials and You website to read about these and other clinical trials that are recruiting volunteers.

When To Call A Doctor

Risk factors for hearing loss in adults – Dr. Girish Rai

911 or other emergency services immediately if:

Hearing loss occurs with an injury to the head or ear.
Hearing loss occurs suddenly with other symptoms such as:

if you:

Develop sudden, severe hearing loss.

Have hearing loss that you think may be caused by earwax.
Have hearing loss after taking medicine.
Have hearing loss after having cold or influenza symptoms.
Have hearing loss after travelling on an airplane.
Feel your hearing is gradually getting worse.
Wonder if you need hearing aids.
Think your baby or child may not be hearing well.

If you think you have a hearing problem, you might choose to see an audiologist.

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Differential Diagnosis Of Autoimmune Inner Ear Disease

For the most part, this is the differential diagnosis of progressive bilateral sensorineural hearing loss.

How is Autoimmune Inner Ear Disease Treated ?

There are several protocols for treatment.

Anti-TNF drugs such as etanercept/infliximab — Enbrel, Humira, Remicade, Symponi and others.

TNF- is a cytokine — a small molecule that turns on immune responses. Interfering with TNF- is a succcessful treatment for several common autoimmune disorders. Until just recently, these drugs were given off-label through injections or infusions. Recently however, there has been an effort to administer these drugs directly into the ear. We will start with this dissussion.

What Causes Hearing Loss

In adults, the most common causes of hearing loss are:

Noise. Noise-induced hearing loss can happen slowly over time. Being exposed to everyday noises, such as listening to very loud music or using a lawn mower, can damage the structures of the inner ear, leading to hearing loss over many years. Sudden, loud noises, such as an explosion, can damage your hearing.
Age. In age-related hearing loss, changes in the inner ear that happen as you get older cause a slow but steady hearing loss. The loss may be mild or severe, and it is always permanent.

Other causes of hearing loss include earwax buildup, an object in the ear, injury to the ear or head, an ear infection, a ruptured eardrum, and other conditions that affect the middle or inner ear.

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Hearing Aids And Other Devices

A hearing aid is defined by the Food and Drug Administration as a wearable sound- amplifying device that is intended to compensate for impaired hearing. The goal of treatment with well-fitted hearing aids is to improve the audibility of even soft speech or music and other sounds while ensuring that sounds do not become uncomfortably loud. Hearing aids can be sophisticated instruments with a variety of customizable features that contribute to their high costs; whether performance improves with higher-cost devices is uncertain. A variety of noncustomized devices, termed hearing assistive technologies, are also available; they include amplified telephones, visual technologies such as captioning, video conferencing, and visual or vibrotactile alerts. Hearing aids are regulated by the FDA, and state laws may restrict access to them. The devices are sold through audiologists and hearing-aid dispensers. The costs of services related to hearing-aid fitting are often bundled with the cost of the device, so the specific costs of the services and the technology are not transparent.