词条 | Autoimmune inner ear disease |
释义 |
| name = Autoimmune inner ear disease | synonyms = AIED | image = File:Cochlea-crosssection.svg | caption = Cochlea, which is affected by this condition | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Autoimmune inner ear disease was first defined by Dr. Brian McCabe in a landmark paper describing an autoimmune loss of hearing.[1] 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.[2] 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.[3] 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.[4] Although AIED has been studied extensively over the past 25 years, no clear mechanism of pathogenesis has emerged. A recent paper[5] 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).[6][7] Signs and symptomsSigns and symptoms of AIED are:[3]
CausesAIED 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.[13] DiagnosisSince 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:[10]
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.[12] TreatmentAIED 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.[6][13][14] 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.[6][15] References1. ^{{Cite journal|title = Autoimmune sensorineural hearing loss|last = McCabe|first = Brian|date = September 1979|journal = Annals of Otology, Rhinology, and Laryngology|doi = 10.1177/000348947908800501|pmid = 496191|volume=88|issue = 5 Pt 1|pages=585–9}} 2. ^{{Cite web|title = Autoimmune Inner Ear Disease {{!}} Vestibular Disorders Association|url = http://vestibular.org/autoimmune-inner-ear-disease-aied|website = vestibular.org|access-date = 2016-02-11|date = 2012-03-07}} 3. ^1 {{Cite web|title = Autoimmune Inner Ear Disease|url = http://www.asha.org/aud/articles/innereardisease/|website = www.asha.org|access-date = 2016-02-11}} 4. ^{{Cite web|title = Autoimmune Inner Ear Disease (AIED) {{!}} American Hearing Research Foundation|url = http://american-hearing.org/disorders/autoimmune-inner-ear-disease-aied/|website = american-hearing.org|access-date = 2016-02-11}} 5. ^{{Cite journal|title = Current understanding of the pathogenesis of autoimmune inner ear disease: a review|journal = Clinical Otolaryngology|date = 2015-10-01|issn = 1749-4486|pmid = 25847404|pages = 412–419|volume = 40|issue = 5|doi = 10.1111/coa.12432|first = A. F.|last = Goodall|first2 = M. A.|last2 = Siddiq}} 6. ^1 2 {{Cite journal|title = IL-1β inhibition in autoimmune inner ear disease: can you hear me now?|journal = The Journal of Clinical Investigation|date = 2014-09-01|issn = 1558-8238|pmc = 4151210|pmid = 25133418|pages = 3685–3687|volume = 124|issue = 9|doi = 10.1172/JCI77197|first = Steven D.|last = Rauch}} 7. ^{{Cite journal|title = A critical role for interleukin-1β in the progression of autoimmune diseases|journal = International Immunopharmacology|date = 2013-11-01|issn = 1878-1705|pmid = 24012439|pages = 658–669|volume = 17|issue = 3|doi = 10.1016/j.intimp.2013.08.012|first = Ruijuan|last = Zhao|first2 = Hongyan|last2 = Zhou|first3 = Shao Bo|last3 = Su}} 8. ^{{Cite journal|title = Meniere's disease might be an autoimmune condition?|journal = Autoimmunity Reviews|pages = 731–738|volume = 11|issue = 10|doi = 10.1016/j.autrev.2012.01.004|pmid = 22306860|first = A.|last = Greco|first2 = A.|last2 = Gallo|first3 = M.|last3 = Fusconi|first4 = C.|last4 = Marinelli|first5 = G.F.|last5 = Macri|first6 = M. de|last6 = Vincentiis|year = 2012}} 9. ^1 2 {{Cite journal|title = Autoantibodies to recombinant human CTL2 in autoimmune hearing loss|journal = The Laryngoscope|date = 2009-05-01|issn = 1531-4995|pmc = 4128554|pmid = 19319905|pages = 924–932|volume = 119|issue = 5|doi = 10.1002/lary.20136|first = Pavan K.|last = Kommareddi|first2 = Thankam S.|last2 = Nair|first3 = Mounica|last3 = Vallurupalli|first4 = Steven A.|last4 = Telian|first5 = H. Alexander|last5 = Arts|first6 = Hussam K.|last6 = El-Kashlan|first7 = Robert T.|last7 = Sataloff|first8 = Thomas E.|last8 = Carey}} 10. ^1 {{Cite web|title = Autoimmune Inner Ear Disease (AIED) {{!}} American Hearing Research Foundation|url = http://american-hearing.org/disorders/autoimmune-inner-ear-disease-aied/#diagnosed|website = american-hearing.org|access-date = 2016-02-11}} 11. ^{{Cite book|publisher = Springer Berlin Heidelberg|date = 2014-01-01|isbn = 9783642277863|pages = 1–5|doi = 10.1007/978-3-642-27786-3_924-5|language = en|first = Werner J.|last = Pichler|editor-first = Hans-Werner|editor-last = Vohr|title = Encyclopedia of Immunotoxicology|chapter = Lymphocyte Transformation Test}} 12. ^{{Cite journal|title = A systematic review and meta-analysis of the diagnostic accuracy of anti-heat shock protein 70 antibodies for the detection of autoimmune hearing loss|journal = Otology & Neurotology |date = 2013-02-01|issn = 1537-4505|pmid = 23295728|pages = 214–219|volume = 34|issue = 2|doi = 10.1097/MAO.0b013e31827d0b8b|first = Carolina|last = Ianuale|first2 = Gabriella|last2 = Cadoni|first3 = Emma|last3 = De Feo|first4 = Luca|last4 = Liberati|first5 = Rachel Kamgaing|last5 = Simo|first6 = Gaetano|last6 = Paludetti|first7 = Walter|last7 = Ricciardi|first8 = Stefania|last8 = Boccia}} 13. ^{{Cite journal|title = Infliximab for autoimmune inner ear disease: case report and literature review|journal = The Journal of Laryngology and Otology|date = 2013-11-01|issn = 1748-5460|pmid = 24125068|pages = 1145–1147|volume = 127|issue = 11|doi = 10.1017/S002221511300217X|first = R. L.|last = Heywood|first2 = S.|last2 = Hadavi|first3 = S.|last3 = Donnelly|first4 = N.|last4 = Patel}} 14. ^{{Cite journal|title = Long-term efficacy of infliximab in autoimmune sensorineural hearing loss associated with rheumatoid arthritis|journal = Rheumatology (Oxford, England)|date = 2014-09-01|issn = 1462-0332|pmid = 24625506|pages = 1715–1716|volume = 53|issue = 9|doi = 10.1093/rheumatology/keu025|first = Pierre|last = Gazeau|first2 = Alain|last2 = Saraux|first3 = Valérie|last3 = Devauchelle-Pensec|first4 = Divi|last4 = Cornec}} 15. ^{{Cite journal|title = Immune-mediated inner ear disease: 10-year experience|journal = Seminars in Arthritis and Rheumatism|date = 2004-10-01|issn = 0049-0172|pmid = 15505770|pages = 544–548|volume = 34|issue = 2|first = Shelley S.|last = Broughton|first2 = William E.|last2 = Meyerhoff|first3 = Stanley B.|last3 = Cohen|doi=10.1016/j.semarthrit.2004.07.001}} External links{{Medical resources| DiseasesDB = | ICD10 = | ICD9 = {{ICD9|388.8}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = article | eMedicineTopic = 857511 | MeshID = }} 2 : Autoimmune diseases|Diseases of the ear and mastoid process |
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