As medical research advances, increasing bodies of evidence demonstrate that the human body does not always work cohesively. In some cases, different aspects of the body can essentially begin to attack other aspects, resulting in systemic autoimmune diseases. There are over 80 known autoimmune disorders that affect the human body, and some changes to the immune system can specifically affect the inner ear – resulting in potential tinnitus or other hearing changes.
What Is An Autoimmune Disease?
An autoimmune disease (in some cases, systemic autoimmune diseases are present) is a condition in which the body’s immune system can no longer differentiate between its own cells and foreign cells. As a result, the immune system begins to attack cells within the body. Some well-known examples of autoimmune disorders include:
- Lupus
- Thyroid Diseases (Hashimoto’s and Graves Disease)
- Rheumatoid Arthritis
- Psoriasis
- Type 1 Diabetes (previously called Juvenile Diabetes)
- Ulcerative colitis
- Multiple sclerosis
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In each of the conditions mentioned above, the immune system is activated to target and attack a part of the body. Although there are therapies available to manage the symptoms, autoimmune diseases are typically considered chronic or incurable.
Interestingly, some experts do consider them treatable, provided that the body’s immune system dysfunction is addressed and managed. Whether conventional methods of treatment or alternative interventions are used, a targeted or systemic autoimmune disorder can have presenting symptoms that are extremely bothersome, which can significantly impact the overall quality of life.
General Symptoms Of Autoimmune Disease
The precise symptoms of an autoimmune disorder will depend on the type of disorder that is present and the specific organ systems that are affected. Nevertheless, there are several symptoms that are common across each autoimmune condition. The common symptoms of an autoimmune condition include:
- Fatigue
- Joint Pain/swelling
- Skin problems
- Abdominal/Intestinal problems
- Recurring fever
- Swollen glands
Autoimmune conditions are not self-diagnosed, and are not typically among the first suspects when a patient comes to see their doctor with the above listed symptoms and complaints. If someone reports a vestibular dysfunction, for instance, their neurological function will likely be evaluated or balance disorders ruled out, before autoimmune conditions are considered or tested.
An abnormal immune response or immune attack can exhibit one or all of the above-listed symptoms, and they may come and go intermittently.
What Is An Autoimmune Inner Ear Disease?
An autoimmune inner ear disease (AIED) is a specific type of autoimmune disease that results in inflammation of the inner ear structures. This is the result of immune system dysfunction, which involves immune cells attacking those structures as though they are comprised of foreign cells.
AIED is a relatively rare disease, as it affects less than 1% of the American population with hearing loss. Additionally, 20% of patients with AIED have another autoimmune disease, such as lupus or rheumatoid arthritis.
As stated above, AIED may not immediately be a suspect when the sensorineural hearing loss and associated symptoms arise. The symptoms are frequently confused with another type of ear disease, such as acoustic neuroma, Meniere’s Disease, or even age-related hearing loss.
Symptoms Of Autoimmune Inner Ear Disease
The symptoms of an autoimmune inner ear disease differ somewhat from those of a standard autoimmune disease. Although the general symptoms of immune system dysfunction may be present and an underlying autoimmune disease may be at play, the standard symptoms of AIED include:
- Hearing loss that progresses over weeks or months
- Fullness in the ear
- Vertigo
- Ringing, hissing, or roaring sounds in one ear or both
- Sudden hearing loss
Although these symptoms are often associated with an autoimmune sensorineural hearing loss, they can also be attributed to another condition, for instance otitis media. Some physicians believe that an autoimmune disease of the ear must result in severe hearing loss or hearing loss in both ears and therefore may not consider the possibility of AIED unless the symptoms match their expectations.
Types Of Autoimmune Inner Ear Disease
According to the American Speech, Language and Hearing Association (ASHA), some of the systemic immune diseases that have been known to also lead to AIED include:
- Cogan’s syndrome. This rare syndrome is a systemic vasculitis (inflammation of blood vessels) and can have severe impact on vision due to ocular inflammation as well as associated vestibulo-auditory dysfunction.
- Relapsing polychondritis. This autoimmune condition is characterized by recurrent inflammation of the cartilage and other tissues and system throughout the body, including structures of the ear. The condition can eventually lead to a mixed hearing loss. A mixed hearing loss is a type of hearing loss that results from dysfunction in more than one part of the ear.
- Systemic lupus erythematosus (SLE). The most common type of lupus, SLE is a systemic condition that can effect the skin, kidney, neurologic system, and musculoskeletal system. In addition, in some patients it impacts the blood flow, which can lead to a sensorineural hearing loss (inner ear hearing loss).
- Granulomatosis with polyangiitis (GPA, former known as Wegener’s granulomatosis). Another rare autoimmune condition that can impair blood vessel function through a formation of granulomas and inflammation. GPA is known to cause sensorineural hearing loss due to inflammation of the inner ear.
- Polyarteritis nodosa (PAN). Another disorder that leads to a systemic necrotizing blood vessel inflammation. Among other issues, it can result in a significant hearing loss, in most cases sensorineural and of a sudden onset.
- Sjogren’s syndrome. In this syndrome, patients suffer from systemic dysfunction of primarily the salivary and lachrymal glands and exhibit symptoms of dry eyes and dry mouth. Sensorineural hearing loss may be present.
- Lyme disease. Inflammation is the root of the issues that accompany lyme disease and can result in ear fullness, sudden sensorineural hearing loss, and other issues specific to the ears.
The above listed conditions may impact the ears in various ways. Those with blood vessels involved may be more likely to affect the ears and cause hearing loss. In any case, it is vital to seek treatment as early as possible for it to be effective. From Lyme disease to Sjogren’s, treatment is the best way to manage symptoms and decrease flare ups.
Treatments For Autoimmune Inner Ear Disease
Like most diseases and disorders of the immune system, the treatments for autoimmune inner ear disease focus on multi-pronged management, rather than a cure. Management interventions include the following:
- Steroid therapy
- Immunosuppressive therapy
- Corticosteroid treatment
- Hearing aids
- Cochlear Implants
None of these will eliminate hearing loss entirely. Instead, each of these treatments will minimize the symptoms of autoimmune inner ear disease, and make hearing and participating in daily life that much easier.
How Are Autoimmune Diseases Linked To Hearing Loss And Tinnitus?
When the immune system attacks healthy cells within the body (and specifically the inner ear), this can cause irreversible damage to the cells within the hearing and balance organs leading to changes in hearing and balance function overall. These changes are typically considered permanent and lead to sensorineural hearing loss. Vestibular symptoms often accompany an autoimmune inner ear disease, including dizziness and balance dysfunction.
Hearing loss is currently the leading cause of tinnitus, and this is thought to be the result of increased hearing sensitivity as a result of damaged hair cells within the inner ear that relay sound to the brain. When cells are damaged, the body works to compensate, which can create phantom sounds or increased sensitivity to sound (called hyperacusis). Tinnitus often sounds like ringing, buzzing, hissing, or clicking, can be intermittent or constant, and can affect one or both ears at a given moment.
Treatment Options For Hearing Loss And Tinnitus
While systemic autoimmune diseases are typically treated or addressed with medication and lifestyle interventions, tinnitus may not be directly caused by an overactive immune system. Instead, it can be associated with sensorineural hearing loss, exposure to loud noises, and complications of systemic autoimmune disease, including those impacting blood flow. Treatment for ear disorders like tinnitus often includes the following:
- Hearing aids. Hearing aids are commonly used to treat hearing loss. The additional benefit of hearing aids is that their use can alleviate tinnitus. In addition to allowing the user to hear more sounds which help to cover up tinnitus, hearing aids can also provide targeted sound therapy via white noise and other preprogrammed sounds.
- Tinnitus Retraining Therapy (TRT). TRT is used to treat tinnitus through a combination of education, counseling and sound therapy.
- Cognitive Behavioral Therapy (CBT). CBT is a therapy method commonly used by mental health professionals. It focuses on addressing an individual’s responses to circumstances and modifying his or her emotions, behaviors and thoughts to deliver better outcomes. CBT can be used to combat the depression and anxiety that frequently accompany ear disorders and their complications, including tinnitus and hearing loss.
- Lifestyle changes. Because tinnitus can be caused by factors such as exposure to loud noise, there are steps tinnitus patients can take to limit flare ups of symptoms or manage their symptoms. These include wearing ear protection, eating a healthy diet, and doing things that you enjoy.
Currently, there is no cure for tinnitus; however, the symptoms of tinnitus can be managed and therefore improved upon. From a systemic autoimmune disorder as a cause to sudden sensorineural hearing loss, audiologists and other hearing professionals can help develop a treatment plan to help manage symptoms and improve the quality of life.
Autoimmune diseases are extremely challenging, both in terms of the symptoms they present, and the difficult road to correct diagnosis. The many changes in the body that come as a result of autoimmunity can take a tall on anyone living with an autoimmune diseases. Learning to manage at least one symptom can provide a great deal of relief.
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