Autoimmune inner ear disease

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Autoimmune inner ear disease
Other namesAIED, Ménière's disease [1]
Cochlea-crosssection.svg
Cochlea, which is affected by this condition
Specialty Audiology, immunology

Autoimmune inner ear disease (AIED) was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing. [2] The disease results in progressive sensorineural hearing loss (SNHL) that acts bilaterally and asymmetrically, and sometimes affects an individual's vestibular system. AIED is used to describe any disorder in which the inner ear is damaged as a result of an autoimmune response. [3] Some examples of autoimmune disorders that have presented with AIED are Cogan's syndrome, relapsing polychondritis, systemic lupus erythematosus, granulomatosis with polyangiitis, polyarteritis nodosa, Sjogren's syndrome, and Lyme disease. [4]

Contents

Research has come to the consensus that AIED is the result of antibodies or other immune cells that cause damage to structures of the inner ear such as the cochlea and vestibular system. Of note, AIED is the only known SNHL that responds to medical treatment, but withholding treatment for longer than three months may result in permanent hearing loss and the need for cochlear implant installation. [5]

Although AIED has been studied extensively over the past 25 years, no clear mechanism of pathogenesis has emerged. A recent paper [6] performed a literature review of all relevant articles dating back to 1980, and proposed a mechanism of pathogenesis which includes an inflammatory response and immune cell attack on inner ear structures. This response leads to an over-activation of other immune cells such as T helper cells, resulting in vascular changes and cochlear harm. AIED appears to be a consequence of damaged sensorineural hearing due to electrochemical disturbances, microthrombosis, and immune cell deposition. Additionally, self-reactive antibodies and T-cells contribute to the aforementioned damage. Research has suggested a valuable next step in uncovering AIED pathogenesis is inquiry into the role of interleukin-1β (IL-1β). [7] [8]

Signs and symptoms

Signs and symptoms of AIED are: [4]

Causes

AIED is generally caused by either antibodies or immune cells that cause damage to the inner ear. There are several theories that propose a cause of AIED:

Currently, the cross-reactions theory appears to be the favored mechanism of AIED pathogenesis. [11]

Diagnosis

Since AIED symptoms are fairly common to many hearing loss disorders, it may be difficult to diagnose AIED without performing multiple medical tests. Some examples of these tests include: [11]

There are also blood tests specific to inner ear disorders:

Though it has also been proposed that the use of anti heat shock protein 70 antibodies may be useful in the detection and diagnosis of AIED, there is not enough evidence to confirm the reliability of this method. [13]

Treatment

AIED treatment is a rapidly changing field. Several medical therapies have been proposed in the treatment of AIED, with corticosteroid therapy being the most effective. However, corticosteroid therapy (specifically with prednisone and dexamethasone) has demonstrated limited effectiveness in some patients, suggesting the need for novel treatment methods. The goal of most AIED treatments is to administer corticosteroids over a certain period of time, re-evaluate hearing at each appointment, and eventually taper off corticosteroid administration. Ideally, patients can be tapered off with hearing fully recovered, though this is the least likely outcome. Often, tumor necrosis factor-α (TNF-α) inhibitors must be administered alongside corticosteroids to achieve a favorable outcome and eventual end to corticosteroid treatment. Recent papers have indicated that the TNF-α inhibitor, infliximab, has the potential to allow for sustained patient improvement and alleviation of symptoms. [7] [14] [15]

Cytotoxic agents such as cyclophosphamide and methotrexate have been used in AIED treatment in the past; however, findings have suggested limited symptom alleviation from these drugs. [7] [16]

Related Research Articles

This is a glossary of medical terms related to communication disorders which are psychological or medical conditions that could have the potential to affect the ways in which individuals can hear, listen, understand, speak and respond to others.

<span class="mw-page-title-main">Ménière's disease</span> Disorder of the inner ear

Ménière's disease (MD) is a disease of the inner ear that is characterized by potentially severe and incapacitating episodes of vertigo, tinnitus, hearing loss, and a feeling of fullness in the ear. Typically, only one ear is affected initially, but over time, both ears may become involved. Episodes generally last from 20 minutes to a few hours. The time between episodes varies. The hearing loss and ringing in the ears can become constant over time.

<span class="mw-page-title-main">Autoimmunity</span> Immune response against an organisms own healthy cells

In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". Prominent examples include celiac disease, post-infectious IBS, diabetes mellitus type 1, Henoch–Schönlein purpura (HSP) sarcoidosis, systemic lupus erythematosus (SLE), Sjögren syndrome, eosinophilic granulomatosis with polyangiitis, Hashimoto's thyroiditis, Graves' disease, idiopathic thrombocytopenic purpura, Addison's disease, rheumatoid arthritis (RA), ankylosing spondylitis, polymyositis (PM), dermatomyositis (DM), and multiple sclerosis (MS). Autoimmune diseases are very often treated with steroids.

<span class="mw-page-title-main">Vestibulocochlear nerve</span> Cranial nerve VIII, for hearing and balance

The vestibulocochlear nerve or auditory vestibular nerve, also known as the eighth cranial nerve, cranial nerve VIII, or simply CN VIII, is a cranial nerve that transmits sound and equilibrium (balance) information from the inner ear to the brain. Through olivocochlear fibers, it also transmits motor and modulatory information from the superior olivary complex in the brainstem to the cochlea.

Ototoxicity is the property of being toxic to the ear (oto-), specifically the cochlea or auditory nerve and sometimes the vestibular system, for example, as a side effect of a drug. The effects of ototoxicity can be reversible and temporary, or irreversible and permanent. It has been recognized since the 19th century. There are many well-known ototoxic drugs used in clinical situations, and they are prescribed, despite the risk of hearing disorders, for very serious health conditions. Ototoxic drugs include antibiotics, loop diuretics, and platinum-based chemotherapy agents. A number of nonsteroidal anti-inflammatory drugs (NSAIDS) have also been shown to be ototoxic. This can result in sensorineural hearing loss, dysequilibrium, or both. Some environmental and occupational chemicals have also been shown to affect the auditory system and interact with noise.

<span class="mw-page-title-main">Granulomatosis with polyangiitis</span> Medical condition

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), after the Nazi German physician Friedrich Wegener, is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs and kidneys can be fatal.

<span class="mw-page-title-main">Sensorineural hearing loss</span> Hearing loss caused by an inner ear or vestibulocochlear nerve defect

Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner ear or sensory organ or the vestibulocochlear nerve. SNHL accounts for about 90% of reported hearing loss. SNHL is usually permanent and can be mild, moderate, severe, profound, or total. Various other descriptors can be used depending on the shape of the audiogram, such as high frequency, low frequency, U-shaped, notched, peaked, or flat.

An autoantibody is an antibody produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases are associated with such antibodies.

<span class="mw-page-title-main">Neurofibromatosis type II</span> Type of neurofibromatosis disease

Neurofibromatosis type II is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves. The types of tumors frequently associated with NF2 include vestibular schwannomas, meningiomas, and ependymomas. The main manifestation of the condition is the development of bilateral benign brain tumors in the nerve sheath of the cranial nerve VIII, which is the "auditory-vestibular nerve" that transmits sensory information from the inner ear to the brain. Besides, other benign brain and spinal tumors occur. Symptoms depend on the presence, localisation and growth of the tumor(s), in which multiple cranial nerves can be involved. Many people with this condition also experience vision problems. Neurofibromatosis type II is caused by mutations of the "Merlin" gene, which seems to influence the form and movement of cells. The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. Historically the underlying disorder has not had any therapy due to the cell function caused by the genetic mutation.

Autoimmune hemolytic anemia (AIHA) occurs when antibodies directed against the person's own red blood cells (RBCs) cause them to burst (lyse), leading to an insufficient number of oxygen-carrying red blood cells in the circulation. The lifetime of the RBCs is reduced from the normal 100–120 days to just a few days in serious cases. The intracellular components of the RBCs are released into the circulating blood and into tissues, leading to some of the characteristic symptoms of this condition. The antibodies are usually directed against high-incidence antigens, therefore they also commonly act on allogenic RBCs. AIHA is a relatively rare condition, with an incidence of 5–10 cases per 1 million persons per year in the warm-antibody type and 0.45 to 1.9 cases per 1 million persons per year in the cold antibody type. Autoimmune hemolysis might be a precursor of later onset systemic lupus erythematosus.

Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye and often fever, fatigue, and weight loss, episodes of vertigo (dizziness), tinnitus and hearing loss. It can lead to deafness or blindness if untreated. The classic form of the disease was first described by D. G. Cogan in 1945.

Cold agglutinin disease (CAD) is a rare autoimmune disease characterized by the presence of high concentrations of circulating cold sensitive antibodies, usually IgM and autoantibodies that are also active at temperatures below 30 °C (86 °F), directed against red blood cells, causing them to agglutinate and undergo lysis. It is a form of autoimmune hemolytic anemia, specifically one in which antibodies bind red blood cells only at low body temperatures, typically 28–31 °C.

<span class="mw-page-title-main">Cochlin</span> Protein highly abundant in the cochlea and vestibule of the inner ear

Cochlin is a protein that in humans is encoded by the COCH gene. It is an extracellular matrix (ECM) protein highly abundant in the cochlea and vestibule of the inner ear, constituting the major non-collagen component of the ECM of the inner ear. The protein is highly conserved in human, mouse, and chicken, showing 94% and 79% amino acid identity of human to mouse and chicken sequences, respectively.

<span class="mw-page-title-main">Autoimmune disease</span> Abnormal immune response to a normal body part

An autoimmune disease is a condition that results from an anomalous response of the immune system, wherein it mistakenly targets and attacks healthy, functioning parts of the body as if they were foreign organisms. It is estimated that there are more than 80 recognized autoimmune diseases, with recent scientific evidence suggesting the existence of potentially more than 100 distinct conditions. Nearly any body part can be involved.

<span class="mw-page-title-main">Enlarged vestibular aqueduct</span> Human disease

Large vestibular aqueduct is a structural deformity of the inner ear. Enlargement of this duct is one of the most common inner ear deformities and is commonly associated with hearing loss during childhood. The term was first discovered in 1791 by Mondini when he was completing a temporal bone dissection. It was then defined by Valvassori and Clemis as a vestibular aqueduct that is greater than or equal to 2.0 mm at the operculum and/or greater than or equal to 1.0 mm at the midpoint. Some use the term enlarged vestibular aqueduct syndrome, but this is felt by others to be erroneous as it is a clinical finding which can occur in several syndromes.

<span class="mw-page-title-main">Anti-SSA/Ro autoantibodies</span> Type of anti-nuclear autoantibodies

Anti-SSA autoantibodies are a type of anti-nuclear autoantibodies that are associated with many autoimmune diseases, such as systemic lupus erythematosus (SLE), SS/SLE overlap syndrome, subacute cutaneous lupus erythematosus (SCLE), neonatal lupus and primary biliary cirrhosis. They are often present in Sjögren's syndrome (SS). Additionally, Anti-Ro/SSA can be found in other autoimmune diseases such as systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), rheumatoid arthritis (RA), and mixed connective tissue disease (MCTD), and are also associated with heart arrhythmia.

Autoimmune retinopathy (AIR) is a rare disease in which the patient's immune system attacks proteins in the retina, leading to loss of eyesight. The disease is poorly understood, but may be the result of cancer or cancer chemotherapy. The disease is an autoimmune condition characterized by vision loss, blind spots, and visual field abnormalities. It can be divided into cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR). The condition is associated with retinal degeneration caused by autoimmune antibodies recognizing retinal proteins as antigens and targeting them. AIR's prevalence is extremely rare, with CAR being more common than MAR. It is more commonly diagnosed in females in the age range of 50–60.

<span class="mw-page-title-main">Vogt–Koyanagi–Harada disease</span> Medical condition

Vogt–Koyanagi–Harada disease (VKH) is a multisystem disease of presumed autoimmune cause that affects melanin-pigmented tissues. The most significant manifestation is bilateral, diffuse uveitis, which affects the eyes. VKH may variably also involve the inner ear, with effects on hearing, the skin, and the meninges of the central nervous system.

Peripheral Ulcerative Keratitis (PUK) is a group of destructive inflammatory diseases involving the peripheral cornea in human eyes. The symptoms of PUK include pain, redness of the eyeball, photophobia, and decreased vision accompanied by distinctive signs of crescent-shaped damage of the cornea. The causes of this disease are broad, ranging from injuries, contamination of contact lenses, to association with other systemic conditions. PUK is associated with different ocular and systemic diseases. Mooren's ulcer is a common form of PUK. The majority of PUK is mediated by local or systemic immunological processes, which can lead to inflammation and eventually tissue damage. Standard PUK diagnostic test involves reviewing the medical history and a completing physical examinations. Two major treatments are the use of medications such as corticosteroids or other immunosuppressive agents and surgical resection of the conjunctiva. The prognosis of PUK is unclear with one study providing potential complications. PUK is a rare condition with an estimated incidence of 3 per million annually.

Cochlear hydrops is a condition of the inner ear involving a pathological increase of fluid affecting the cochlea. This results in swelling that can lead to hearing loss or changes in hearing perception. It is a form of endolymphatic hydrops and related to Ménière's disease. Cochlear hydrops refers to a case of inner-ear hydrops that only involves auditory symptoms and does not cause vestibular issues.

References

  1. 1 2 "Autoimmune Inner Ear Disease". Autoimmune Registry Inc. Retrieved 14 June 2022.
  2. ↑ McCabe, Brian (September 1979). "Autoimmune sensorineural hearing loss". Annals of Otology, Rhinology, and Laryngology. 88 (5 Pt 1): 585–9. doi:10.1177/000348947908800501. PMID   496191. S2CID   21133298.
  3. ↑ "Autoimmune Inner Ear Disease | Vestibular Disorders Association". vestibular.org. 2012-03-07. Retrieved 2016-02-11.
  4. 1 2 "Autoimmune Inner Ear Disease". www.asha.org. Archived from the original on 2016-02-16. Retrieved 2016-02-11.
  5. ↑ "Autoimmune Inner Ear Disease (AIED) | American Hearing Research Foundation". american-hearing.org. Archived from the original on 2016-02-08. Retrieved 2016-02-11.
  6. ↑ Goodall, A. F.; Siddiq, M. A. (2015-10-01). "Current understanding of the pathogenesis of autoimmune inner ear disease: a review". Clinical Otolaryngology. 40 (5): 412–419. doi:10.1111/coa.12432. ISSN   1749-4486. PMID   25847404. S2CID   41616626.
  7. 1 2 3 Rauch, Steven D. (2014-09-01). "IL-1β inhibition in autoimmune inner ear disease: can you hear me now?". The Journal of Clinical Investigation. 124 (9): 3685–3687. doi:10.1172/JCI77197. ISSN   1558-8238. PMC   4151210 . PMID   25133418.
  8. ↑ Zhao, Ruijuan; Zhou, Hongyan; Su, Shao Bo (2013-11-01). "A critical role for interleukin-1β in the progression of autoimmune diseases". International Immunopharmacology. 17 (3): 658–669. doi:10.1016/j.intimp.2013.08.012. ISSN   1878-1705. PMID   24012439.
  9. ↑ Greco, A.; Gallo, A.; Fusconi, M.; Marinelli, C.; Macri, G.F.; Vincentiis, M. de (2012). "Meniere's disease might be an autoimmune condition?". Autoimmunity Reviews. 11 (10): 731–738. doi: 10.1016/j.autrev.2012.01.004 . PMID   22306860.
  10. 1 2 3 Kommareddi, Pavan K.; Nair, Thankam S.; Vallurupalli, Mounica; Telian, Steven A.; Arts, H. Alexander; El-Kashlan, Hussam K.; Sataloff, Robert T.; Carey, Thomas E. (2009-05-01). "Autoantibodies to recombinant human CTL2 in autoimmune hearing loss". The Laryngoscope. 119 (5): 924–932. doi:10.1002/lary.20136. ISSN   1531-4995. PMC   4128554 . PMID   19319905.
  11. 1 2 "Autoimmune Inner Ear Disease (AIED) | American Hearing Research Foundation". american-hearing.org. Archived from the original on 2016-02-08. Retrieved 2016-02-11.
  12. ↑ Pichler, Werner J. (2014-01-01). "Lymphocyte Transformation Test". In Vohr, Hans-Werner (ed.). Encyclopedia of Immunotoxicology. Springer Berlin Heidelberg. pp. 1–5. doi:10.1007/978-3-642-27786-3_924-5. ISBN   9783642277863.
  13. ↑ Ianuale, Carolina; Cadoni, Gabriella; De Feo, Emma; Liberati, Luca; Simo, Rachel Kamgaing; Paludetti, Gaetano; Ricciardi, Walter; Boccia, Stefania (2013-02-01). "A systematic review and meta-analysis of the diagnostic accuracy of anti-heat shock protein 70 antibodies for the detection of autoimmune hearing loss". Otology & Neurotology. 34 (2): 214–219. doi:10.1097/MAO.0b013e31827d0b8b. ISSN   1537-4505. PMID   23295728. S2CID   1580427.
  14. ↑ Heywood, R. L.; Hadavi, S.; Donnelly, S.; Patel, N. (2013-11-01). "Infliximab for autoimmune inner ear disease: case report and literature review". The Journal of Laryngology and Otology. 127 (11): 1145–1147. doi:10.1017/S002221511300217X. ISSN   1748-5460. PMID   24125068. S2CID   7912213.
  15. ↑ Gazeau, Pierre; Saraux, Alain; Devauchelle-Pensec, Valérie; Cornec, Divi (2014-09-01). "Long-term efficacy of infliximab in autoimmune sensorineural hearing loss associated with rheumatoid arthritis". Rheumatology. 53 (9): 1715–1716. doi: 10.1093/rheumatology/keu025 . ISSN   1462-0332. PMID   24625506.
  16. ↑ Broughton, Shelley S.; Meyerhoff, William E.; Cohen, Stanley B. (2004-10-01). "Immune-mediated inner ear disease: 10-year experience". Seminars in Arthritis and Rheumatism. 34 (2): 544–548. doi:10.1016/j.semarthrit.2004.07.001. ISSN   0049-0172. PMID   15505770.
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