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Autoimmune Inner Ear Disease

Autoimmune Disease of the Inner Ear


  • A case is presented of a 39-year-old woman with left AIED 22 weeks into her first pregnancy and right AIED 24 weeks into her second pregnancy. A review of the literature examines the safety and efficacy of systemic steroid use in pregnancy.[theajo.com]
  • Tabs Content Clinical Overview Diagnosis Indications for Testing Appropriate clinical presentation Exclusion of other causes of hearing loss, including Ménière disease, other autoimmune diseases (with associated hearing loss ), otosclerosis, retrocochlear[arupconsult.com]
  • AIED presents with rapidly progressive, often fluctuating bilateral sensorineural hearing loss (SNHL). It usually occurs over weeks to months. Patients may also be aware of fullness in the ears and tinnitus. Dizziness occurs in 50% of patients.[lmhofmeyr.co.za]
  • We will describe the clinical aspects of the disease, the histopathology, the immunologic indicators, the types of presentation, both from the audiologic and vestibular point of view, clinical trials for treatment and the follow-up.[mayoclinic.pure.elsevier.com]
  • […] disease in children. ( 21178803 ) Huang N.C....Sataloff R.T. 2011 20 IL-1I is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease. ( 21199898 ) Pathak S....Vambutas A. 2011 21 High dose combination pertussis[malacards.org]
Hearing Impairment
  • Autoimmune Inner Ear Disease (AIED) is rare and probably only 1 per cent of all cases of hearing impairment or dizziness is due to Autoimmune Inner Ear Disease (AIED).[hear-it.org]
  • In cases with a classic rapidly progressive bilateral hearing impairment, a trial of steroids (prednisone or dexamethasone) for four weeks may be tried.[diversifiedhearing.com]
  • “Patients with RA are at higher risk of hearing impairment compared to healthy subjects in their course of the disease. The hearing impairment in RA seems to be a multifactorial condition.[healthline.com]
  • In the multivariable logistic analysis, RA (odds ratios [OR] 1.47, 95% confidence interval [CI] 1.05-2.06, p 0.025) was an independent risk factor of low/mid-frequency hearing impairment... " So in other words, RA is associated with "hearing impairment[dizziness-and-balance.com]
Neurologic Manifestation
  • Manifestations Nervous System Diseases Signs and Symptoms Otitis Interleukin 1 Receptor Antagonist Protein Antirheumatic Agents[clinicaltrials.gov]


  • Workup should also include tests to rule out syphilis.[oatext.com]
  • This change may prompt a workup for AIED.[emedicine.medscape.com]
  • Conclusion A broad and exhaustive workup is crucial in early onset hearing loss cases. Genetic study has a crescent importance in diagnostic search. There are a myriad of proteins evolved in the hearing process.[alliedacademies.org]
  • Some authors suggest that any patient diagnosed with unilateral Menière’s disease who develops symptoms in the contralateral ear should undergo workup for an autoimmune aetiology [ 37 ].[academic.oup.com]
Treponema Pallidum
  • pallidum – fluorescent treponemal antibody (FTA) Borrelia burgdorferi – Lyme serology Diabetes mellitus – hemoglobin A1c HIV – Western blot C-reactive protein (CRP) Preferred test to detect acute phase inflammation ( Choosing Wisely: 15 Things Physicians[arupconsult.com]
Borrelia Burgdorferi
  • burgdorferi – Lyme serology Diabetes mellitus – hemoglobin A1c HIV – Western blot C-reactive protein (CRP) Preferred test to detect acute phase inflammation ( Choosing Wisely: 15 Things Physicians and Patients Should Question , 2016; American Society[arupconsult.com]


  • Because a rheumatologist has extensive training and experience in these treatments, nearly all otolaryngologists and neurotologists have them manage the drug treatment.[vestibular.org]
  • Treatment options are limited, with corticosteroids being the only validated treatment option, and methotrexate offering no significant benefit to patients.[ncbi.nlm.nih.gov]
  • 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.[en.wikipedia.org]


  • This study concluded that early diagnosis and early immunosuppressive therapy on AIED would improve prognosis. 17 メニエール病の重症度分類について 八木 聰明 , 伊藤 壽一 , 久保 武 , 高橋 正紘 , 高橋 光明 , 古屋 信彦 , 山下 敏夫 , 渡辺 行雄 , 工田 昌矢 , 室伏 利久 , 富山 俊一 J-STAGE 医中誌Web 参考文献8件 被引用文献8件 21 ブタ[ci.nii.ac.jp]
  • Differential diagnosis and prognosis of T1-weighted post-gadolinium intralabyrinthine hyperintensities. Eur Radiol 2010;20(11):2628-36. From the Department of Diagnostic Radiology, Changi General Hospital, Singapore. Corresponding author: Dr.[mydigimag.rrd.com]
  • Unfortunately, the prognosis is difficult to gauge except in the setting of profound, sustained SNHL, in which case significant recovery of hearing is unlikely. Figure 68–1. Anatomy of the temporal bone and audiovestibular apparatus.[accessmedicine.mhmedical.com]
  • As such, the longer symptoms persist, the more severe the hearing loss, and the simultaneous occurrence of vertigo all give rise to a poorer prognosis. 3 Unfortunately, delay in diagnosis is common because of an insufficient index of suspicion—often due[hearingreview.com]
  • Kaplan-Meier analysis showed that functional allelic variants on REL, TNFAIP3, REL and TNIP1 genes did not influence the auditory prognosis in MD ( Table 3 ).[journals.plos.org]


  • Screening for viral and rheumatologic etiologies was again negative.[mydigimag.rrd.com]
  • No single laboratory test is recommended for the evaluation of AIED; however, testing may be useful to rule out alternate etiologies associated with hearing loss.[arupconsult.com]
  • Gidley, Sudden hearing loss in a melanoma patient on pembrolizumab: an etiology not to be omitted in the differential diagnosis, Journal for ImmunoTherapy of Cancer, 5, 1, (2017).[doi.org]
  • Laboratory studies should be conducted based on the patient’s complaint, history, and suspected etiologies (Table 1).[hearingreview.com]
  • De Leenheer EMR, Janssens S, Padalko E, Loose D, Leroy BP, et al. (2011) Etiological diagnosis in the hearing impaired newbord: Proposal of a flow chart. Int J Pediatr Otorhinolaryngol 75: 27-32.[alliedacademies.org]


  • A recent retrospective of 47 AIED cases by Matsuoka and Harris provides an excellent summary of the clinical epidemiology ( 4 ). In contrast to many systemic autoimmune diseases, type 1 AIED equally affects males and females.[doi.org]
  • […] syndrome Infectious disease Lyme disease hearing loss Syphilitic hearing loss Viral – HIV Ménière disease Nonsyndromic hearing loss Idiopathic sensorineural hearing loss Systemic disease Diabetes mellitus Systemic lupus erythematosus (SLE) Background Epidemiology[arupconsult.com]
  • In 2012, a study was published in the American Journal of Epidemiology .[healthline.com]
  • Hughes et al found that approximately one half of their patients with AIED have manifestations of autoimmune Ménière syndrome. [7, 8] Epidemiology Frequency United States Because the existence of autoimmune inner ear disease (AIED) has been recognized[emedicine.medscape.com]
Sex distribution
Age distribution


  • The pathophysiology of AIED is still not well understood. Multiple potential mechanisms have been identified that can result in immune-mediated inner ear pathology.[ncbi.nlm.nih.gov]
  • However, the pathophysiology of many common clinical conditions is still not completely understood. There is no FDA-approved pharmacological agent for treatment of sensorineural hearing loss.[grantome.com]
  • […] disease Diabetes mellitus Systemic lupus erythematosus (SLE) Background Epidemiology Prevalence Rare disease, accounting for True prevalence unknown Age Peak onset in 20s-40s Rare in pediatric ages Sex – M:F ratio unknown due to low prevalence of disease Pathophysiology[arupconsult.com]
  • Pathophysiology The term autoimmune inner ear disease (AIED) implies a direct attack of the immune system upon an endogenous inner ear antigen.[emedicine.medscape.com]


  • Lack of a precise serological marker prevents accurate immunological understanding. Definition is, of course, difficult in a disease whose natural history is not well delineated.[ncbi.nlm.nih.gov]
  • Methotrexate, which initially showed promise, has recently been shown to be ineffective in preventing progression of hearing loss.[ncbi.nlm.nih.gov]
  • Methotrexate, which initially showed promise, has recently been shown to be ineffective in preventing progression of hearing loss .[journals.lww.com]
  • Since you may not get a clear answer, your doctor may start you on treatment without a sure diagnosis to prevent damage to your hearing that can't be fixed.[webmd.com]
  • Treatment of antiphospholipid syndrome can be directed toward preventing thromboembolic events by using antithrombotic medications. Only warfarin has been shown to be effective.[ncbi.nlm.nih.gov]

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