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Autoimmune Inner Ear Disease - Causes, Symptoms and Treatment

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Autoimmune sudden sensorineural hearing loss is due to inner ear cell destruction caused by the immune system. Refer to this article to know more in detail.

Published At December 19, 2022
Reviewed AtJanuary 29, 2024

Introduction:

Autoimmune sudden sensorineural hearing loss, also known as autoimmune inner ear disease, is a condition caused due to the body’s immune reaction against its own cells, mainly affecting the inner ear wall. This cell-mediated immune reaction acts against the cells of the inner ear or protein present in the inner ear. It is a rare disease that progresses gradually over months and is hard to diagnose. It can be present in association with other disorders as secondary autoimmune conditions. Hence, it is important to rule out other autoimmune diseases to confirm the diagnosis. Its occurrence is mainly involving both ears, though not necessarily at the same time. The peculiar characteristic of progression over weeks or months rejects the other differential diagnoses like hearing loss due to trauma or old age. Thorough patient history, evaluation, medical tests, and some imaging is necessary to confirm the autoimmune sudden sensorineural hearing loss. Treatment of this disease mainly includes immunosuppressive agents to stop the progression of the disease and assistive hearing devices and cochlear implants to improve hearing ability.

What Is Autoimmune Sudden Sensorineural Hearing Loss?

It is an immune system malfunction, where the body’s own immune cells attack the inner ear causing hearing loss. It is a rare, rapidly progressing disease with unknown etiology. It can happen primarily or in association with other autoimmune diseases (a secondary autoimmune inner ear disease) and usually involve both ears eventually.

What Are the Types of Autoimmune Sudden Sensorineural Hearing Loss?

  • Primary: It is present independently and not associated with any other condition.

  • Secondary: It is present in association with other autoimmune disorders.

What Are the Other Autoimmune Diseases Associated With Secondary Autoimmune Sudden Sensorineural Hearing Loss?

  • Cogan's Syndrome: It is a rare autoimmune disease that affects both eyes and ears that is characterized by recurrent ocular inflammation and hearing loss, which can result in deafness if not treated.

  • Wegener’s Granulomatosis: It is a rare disorder that results in inflammation of blood vessels of the nose, throat, sinuses, lungs, and kidneys.

  • Relapsing Polychondritis: It is an uncommon degenerative condition of the cartilage in the body.

  • Lyme Disease: It is considered a tick-borne illness, and the causative agent is borrelia bacteria. This disease is characterized by a bull’s eye rash, flu-like symptoms, joint pain, and weakness.

  • Systemic Lupus Erythematosus (SLE): SLE (systemic lupus erythematosus) is an autoimmune inflammatory condition that affects joints, skin, blood cells, kidneys, heart, and lungs.

  • Polyarteritis Nodosa: It is a not-so-common disorder that is characterized by generalized inflammation, weakness, and damaged arteries. Mostly the small and medium-sized arteries are affected.

  • Sjogren’s Syndrome: It is an immune disorder that predominantly causes dry eyes and dry mouth.

What Are the Symptoms of Autoimmune Sudden Sensorineural Hearing Loss?

Common symptoms of autoimmune sudden sensorineural hearing loss are:

  • Progressive hearing loss in both ears over weeks to months, which may and may not be at the same time.

  • Loss of balance or dizziness.

  • Tinnitus or ringing sound in the ears.

  • Fluctuating hearing.

  • Ear fullness.

  • Eustachian tube obstruction due to inflammation of the inner ear causing conductive hearing loss.

  • Secondary symptoms due to associated autoimmune disease (tiredness, muscle pain, swelling, redness, fever, etc.).

What Are the Causes of Autoimmune Sudden Sensorineural Hearing Loss?

The causes of autoimmune sudden sensorineural hearing loss can be:

  • Autoimmunity Against the Inner Ear: The body’s malfunctioning immune system forms autoantibodies and immune complexes against the inner ear. This will eventually cause the destruction of inner ear cells and result in bilateral hearing loss.

  • Autoimmunity Against Cochlin: The body’s immune system forms antibodies against the cochlin protein, which is present in the inner ear wall. Destruction of this protein leads to damage to the inner war wall.

  • Dilation of Endolymphatic Sac: As an immune response, the endolymphatic sac (structure present in the inner ear) present in the inner ear may dilate and cause hearing loss.

How to Diagnose Autoimmune Sudden Sensorineural Hearing Loss?

Apart from clinical signs and symptoms, different medical tests are done to diagnose autoimmune sudden sensorineural hearing loss, such as,

  • Erythrocyte Sedimentation Rate: It is used as a general indicator in case of inflammation.

  • Rheumatoid Factor: It is used as a marker for rheumatoid arthritis and other autoimmune disorders.

  • Antinuclear Antibody Titer: It is used to detect lupus and other related autoimmune disorders.

  • Quantitative Immunoglobulin Determination: This is a test used to find immunodeficiencies by assessing the quantitative determination of different immunoglobulins.

  • A Leukocyte Migration Inhibition Test: This test helps reveal the sensitivity of lymphocytes and the cell-mediated immunity to a particular antigen.

To rule out any other underlying cause for sensorineural hearing loss, other commonly used medical tests are:

  • Complete Blood Count: It is helpful to rule out hemolytic disorders or leukemia.

  • The Fluorescent Treponemal Antibody Absorption (Fta-Abs) Test: It is done for the identification of antibodies against the bacteria which causes syphilis.

  • Magnetic Resonance Imaging (MRI): For brain and cerebellopontine angle help to detect the presence of vascular lesions and space-consuming lesions.

  • Lipid Profile: It is done to check for any dyslipidemias. Dyslipidemia refers to abnormally elevated levels of lipids or cholesterol in the circulation.

What Is the Treatment for Autoimmune Sudden Sensorineural Hearing Loss?

A team of different specialists should work and treat autoimmune sudden sensorineural hearing loss. Different treatment approaches should be evaluated based on the progression of the disease, and care should be taken to evaluate the side effects of immunosuppressant agents.

Different therapies used are:

  • Steroids: Corticosteroids are mainly used in the treatment of immunosuppression and their effect on the modulation of sodium transport. Although, they also possess the risk of steroid-associated adverse effects.

  • Non-steroidal Immunosuppressants: In non-responsive patients with steroid therapy or with unacceptable side effects of steroid therapy, alternative immunosuppressive approaches are used. Cyclophosphamide is used as an effective drug in such cases; however, more research is needed to evaluate its side effects.

  • Methotrexate: It is used in Prednisone-sparing treatment as an adjunct to other drug therapies.

  • Cytokine Modulation: Blocking of tumor necrosis factor-alpha (TNF - alpha), both systemically and locally, has been used successfully as a treatment for autoimmune sudden hearing loss.

  • Cell Modulation: Rituximab has been used as an effective drug in the treatment.

  • Plasmapheresis: The procedure has shown some success in improving the hearing capacity of the ears.

  • Hearing Aids: Assistive hearing devices can be used to improve hearing ability.

  • Cochlear Implantation: Patients who can no longer hear from hearing devices are evaluated for cochlear implantation.

Conclusion:

Autoimmune sudden sensorineural hearing loss happens because of the body’s immune reaction against inner ear cells. Clinical symptoms, history of disease progression, and certain tests can help to diagnose the autoimmune sudden sensorineural hearing loss, also known as autoimmune inner ear disease. It can be treated efficiently with Corticosteroids, though other drug therapies are also found to be effective to some extent. Hearing aids or cochlear implantation can be helpful in some cases to improve hearing ability.

Frequently Asked Questions

1.

Can Autoimmune Inner Ear Disease Be Treated?

The treatment can manage the symptoms and decrease the progression of the disease. Treatment involves the combination of steroids and immunosuppressive drugs to suppress the immune response, reducing inflammation in the inner ear. The doctor may recommend cochlear implants or hearing aids to improve hearing function. Vestibular rehabilitation therapy can manage balance problems. 

2.

Which Autoimmune Diseases Lead to Ear Problems?

The autoimmune diseases that lead to ear problems are as follows:
- Autoimmune inner ear disease affects the inner ear and leads to hearing loss.
- Systemic lupus erythematosus can cause hearing loss and inflammation of the middle ear.
- Rheumatoid arthritis can lead to inflammation of the ear.
- Sjogren syndrome is an autoimmune disease that affects the salivary and tear glands. It causes ear problems and leads to hearing loss.

3.

What Blood Test Diagnoses Autoimmune Disease?

The blood test used to diagnose the autoimmune disease is as follows:
- Antinuclear Antibody Test: The test detects foreign antibodies against proteins. 
- Rheumatoid Factor Test: The test detects the presence of an antibody (rheumatoid factor) in the blood of people with rheumatoid arthritis.
- Complement Test: A low level of complement may indicate an autoimmune disease.

4.

How to Diagnose Autoimmune Diseases?

The autoimmune disease can be diagnosed in the following ways:
- Physical Examination: The doctor will perform a physical examination/
- Blood Test: The doctor may prescribe a blood test for antibodies.
- Imaging Test: Tests such as X-rays, computed tomography (CT) scan, and magnetic resonance testing (MRI) helps in the detection of organ damage caused by autoimmune diseases.
- Biopsy: A sample from the affected area is taken and examined under the microscope to check for signs of damage.

5.

Can a Person Lose Hearing With an Autoimmune Disease?

A person can lose hearing with an autoimmune disease. Autoimmune diseases in which the human body kills its cells and lead to inflammation and damage. Inner ear damage can cause hearing loss. The other symptoms include dizziness and ringing in the ears. 

6.

How to Decrease Inflammation in the Inner Ear?

There are some ways to decrease inflammation in the inner ear as follows:
- The doctor may prescribe corticosteroids and immunosuppressive drugs to reduce inflammation.
- Some dietary changes, such as nuts, fish, and seeds, can reduce inflammation.
- Yoga, meditation, and deep breathing exercises can reduce stress.

7.

Are Inner Ear Diseases Serious?

Inner ear disease can be serious and affect the individual quality of life. It can cause hearing problems. The common inner ear diseases are as follows:
- Autoimmune inner ear disease.
- Vestibular neuritis (vestibular nerve inflammation).
- Minieres disease affects the inner ear and causes dizziness and hearing loss.

8.

Are Inner Ear Problems Temporary?

Inner ear problems can be temporary or permanent. Some inner ear problems cause vertigo and can be resolved on their own. Some inner ear problems cause permanent hearing loss which can be permanent. 

9.

Can Inner Ear Damage Be Treated?

Treatment of inner ear damage depends on the severity and cause of damage. The temporary ear damage can be cured with supportive treatment. Some inner ear damage may be permanent and cannot be treated. The doctor may prescribe cochlear implants or hearing aids to manage hearing loss.

10.

Which Doctor Treats Inner Ear Problems?

Healthcare professionals can treat inner ear problems. An otolaryngologist which is also known as an ENT doctor, can diagnose inner ear problems and manage symptoms such as hearing loss and tinnitus. An ENT doctor focuses on the nose, ear, and throat. An audiologist specialized in hearing and balance disorders can treat the condition.

11.

Can Inner Ear Problems Hurt the Brain?

Inner ear problems affect the brain because the ear is connected to the brain through a vestibulocochlear nerve. It carries hearing information to the brain. It can affect the nerves and break the brain's connection with the inner ear. The symptoms include vertigo and dizziness.

12.

How Much Time Did the Inner Ear Problem Take To Recover?

The time taken depends on the cause of the underlying problem. The inner ear viral infection may last for several days to weeks. The symptoms, such as vertigo, can resolve within on few weeks. Chronic inner ear problems may take several months to recover. The problems can be temporary or permanent. 
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Dr. Syed Peerzada Tehmid Ul Haque
Dr. Syed Peerzada Tehmid Ul Haque

Otolaryngology (E.N.T)

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