MCID: ATM021
MIFTS: 37

Autoimmune Inner Ear Disease

Categories: Rare diseases, Ear diseases

Aliases & Classifications for Autoimmune Inner Ear Disease

MalaCards integrated aliases for Autoimmune Inner Ear Disease:

Name: Autoimmune Inner Ear Disease 53
Ocular Albinism, Type Ii 73
Aied 53

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases


External Ids:

UMLS 73 C0268505

Summaries for Autoimmune Inner Ear Disease

MalaCards based summary : Autoimmune Inner Ear Disease, also known as ocular albinism, type ii, is related to ulcerative colitis and colitis. An important gene associated with Autoimmune Inner Ear Disease is MMP9 (Matrix Metallopeptidase 9), and among its related pathways/superpathways are Immune response IL-23 signaling pathway and Colorectal Cancer Metastasis. The drugs Methotrexate and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include testes and lung, and related phenotypes are cardiovascular system and neoplasm

Wikipedia : 76 Autoimmune inner ear disease (AIED) was first defined by Dr. Brian McCabe in a landmark paper describing... more...

Related Diseases for Autoimmune Inner Ear Disease

Diseases related to Autoimmune Inner Ear Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 115)
# Related Disease Score Top Affiliating Genes
1 ulcerative colitis 29.6 MPO TNF
2 colitis 29.2 MPO TNF
3 inner ear disease 29.1 MMP9 MPO TIMP1 TNF
4 androgen insensitivity syndrome 11.9
5 amelogenesis imperfecta 11.8
6 aland island eye disease 11.7
7 scoliosis, isolated 1 11.4
8 hereditary amyloidosis 11.2
9 aortic valve insufficiency 11.1
10 childhood-onset cerebral x-linked adrenoleukodystrophy 11.1
11 pediatric arterial ischemic stroke 11.0
12 amelogenesis imperfecta, type ib 11.0
13 salt and pepper developmental regression syndrome 10.9
14 jalili syndrome 10.8
15 amelogenesis imperfecta, type ie 10.8
16 amelogenesis imperfecta, type ij 10.8
17 auditory system disease 10.8
18 alzheimer disease 10.7
19 scleritis 10.3 MMP9 TNF
20 neuronitis 10.3
21 diverticulitis 10.3 MMP9 TNF
22 transverse myelitis 10.3 MMP9 TNF
23 tuberculous meningitis 10.3 MMP9 TNF
24 rosacea 10.3 MMP9 TNF
25 cerebritis 10.2
26 cheilitis 10.2 MMP9 TNF
27 amyloidosis 10.2
28 primary progressive multiple sclerosis 10.2 MMP9 TNF
29 chorioamnionitis 10.2 MMP9 TNF
30 spinal cord injury 10.2 MMP9 TNF
31 carotid stenosis 10.2 MMP9 TNF
32 bacterial meningitis 10.2 MMP9 TNF
33 gingivitis 10.2 MMP9 TNF
34 microscopic polyangiitis 10.2 MPO TNF
35 takayasu arteritis 10.2 MMP9 TNF
36 anca-associated vasculitis 10.2 MPO TNF
37 periodontosis 10.1 MMP9 MPO
38 pleural disease 10.1 MPO TNF
39 proctitis 10.1 MPO TNF
40 arteries, anomalies of 10.1 MMP9 TNF
41 churg-strauss syndrome 10.1 MPO TNF
42 ileitis 10.1 MPO TNF
43 pelvic inflammatory disease 10.1 MMP9 MPO
44 suppurative cholangitis 10.1 MPO TNF
45 pleurisy 10.1 MPO TNF
46 meningitis 10.1 MMP9 TNF
47 gastrointestinal system cancer 10.1 MMP9 TNF
48 sclerosing cholangitis 10.1 MPO TNF
49 autoinflammation, lipodystrophy, and dermatosis syndrome 10.0 MPO TNF
50 demyelinating disease 10.0 MMP9 TNF

Graphical network of the top 20 diseases related to Autoimmune Inner Ear Disease:



Diseases related to Autoimmune Inner Ear Disease

Symptoms & Phenotypes for Autoimmune Inner Ear Disease

MGI Mouse Phenotypes related to Autoimmune Inner Ear Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.26 MMP9 MPO TIMP1 TNF
2 neoplasm MP:0002006 8.8 MMP9 TIMP1 TNF

Drugs & Therapeutics for Autoimmune Inner Ear Disease

Drugs for Autoimmune Inner Ear Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 17)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
2
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
3
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
4
leucovorin Approved, Nutraceutical Phase 3 58-05-9 143 6006
5
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
6 Antirheumatic Agents Phase 3,Phase 1,Phase 2
7 Folic Acid Antagonists Phase 3
8 Dermatologic Agents Phase 3
9 Nucleic Acid Synthesis Inhibitors Phase 3
10 Vitamin B Complex Phase 3
11 Immunosuppressive Agents Phase 3
12 Antimetabolites Phase 3
13 Antimetabolites, Antineoplastic Phase 3
14 Folate Nutraceutical Phase 3
15 Vitamin B9 Nutraceutical Phase 3
16 Pharmaceutical Solutions Phase 2
17 Interleukin 1 Receptor Antagonist Protein Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Autoimmunity in Inner Ear Disease Terminated NCT00000361 Phase 3 Corticosteroids;Methotrexate
2 A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease Completed NCT01267994 Phase 1, Phase 2 Anakinra
3 The Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01950312 Phase 2 gevokizumab
4 Intratympanic Injection for Autoimmune Inner Ear Disease Terminated NCT01526174 Phase 1, Phase 2 Golimumab
5 Strength Training on Pancreatic Cancer Withdrawn NCT01395563 Not Applicable

Search NIH Clinical Center for Autoimmune Inner Ear Disease

Genetic Tests for Autoimmune Inner Ear Disease

Anatomical Context for Autoimmune Inner Ear Disease

MalaCards organs/tissues related to Autoimmune Inner Ear Disease:

41
Testes, Lung

Publications for Autoimmune Inner Ear Disease

Articles related to Autoimmune Inner Ear Disease:

(show top 50) (show all 69)
# Title Authors Year
1
Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan's Syndrome. ( 29850616 )
2018
2
The Balance of Tissue Inhibitor of Metalloproteinase-1 and Matrix Metalloproteinase-9 in the Autoimmune Inner Ear Disease Patients. ( 28696822 )
2017
3
Autoimmune inner ear disease in a melanoma patient treated with pembrolizumab. ( 26885370 )
2016
4
Systematic Review of Treatments for Autoimmune Inner Ear Disease. ( 26485595 )
2015
5
Progression of autoimmune inner ear disease to labyrinthitis ossificans: clinical and radiologic correlation. ( 25738715 )
2015
6
Current understanding of the pathogenesis of autoimmune inner ear disease: a review. ( 25847404 )
2015
7
Paraneoplastic syndrome: a masquerade of autoimmune inner ear disease. ( 25427223 )
2015
8
N-Acetylcysteine attenuates tumor necrosis factor alpha levels in autoimmune inner ear disease patients. ( 26392121 )
2015
9
Diffuse systemic sclerosis presenting as Meniere's disease-like symptoms as part of autoimmune inner ear disease. ( 26040060 )
2015
10
Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease. ( 25133431 )
2014
11
IL-1I^ inhibition in autoimmune inner ear disease: can you hear me now? ( 25133418 )
2014
12
Cochlin in autoimmune inner ear disease: Is the search for an inner ear autoantigen over? ( 25199741 )
2014
13
An open label study to evaluate the safety and efficacy of intratympanic golimumab therapy in patients with autoimmune inner ear disease. ( 25203561 )
2014
14
Cogan's syndrome: an autoimmune inner ear disease. ( 22846458 )
2013
15
Innate immune recognition of molds and homology to the inner ear protein, cochlin, in patients with autoimmune inner ear disease. ( 23912888 )
2013
16
Autoimmune inner ear disease preliminary case report: audiometric findings following steroid treatments. ( 23800807 )
2013
17
Infliximab for autoimmune inner ear disease: case report and literature review. ( 24125068 )
2013
18
Autoimmune inner ear disease: a retrospective review of forty-seven patients. ( 23817208 )
2013
19
Autoimmune inner ear disease in children. ( 21178803 )
2011
20
IL-1I^ is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease. ( 21199898 )
2011
21
High dose combination pertussis toxin induces autoimmune inner ear disease in Sprague-Dawley rats. ( 21366505 )
2011
22
Extraintestinal Crohn's disease mimicking autoimmune inner ear disease: a histopathological approach. ( 20523038 )
2011
23
Labyrinthitis ossificans secondary to autoimmune inner ear disease: a previously unreported condition. ( 20416474 )
2010
24
Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls. ( 21082747 )
2010
25
Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease. ( 19395984 )
2009
26
Immunosuppressive therapy for autoimmune inner ear disease. ( 19885385 )
2009
27
Induction of tolerance by oral administration of beta-tubulin in an animal model of autoimmune inner ear disease. ( 19365153 )
2009
28
The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. ( 18777037 )
2009
29
Autoimmune inner ear disease: clinical and laboratory findings and treatment outcome. ( 19128599 )
2008
30
Does the serological study for viral infection in autoimmune inner ear disease make sense? ( 18235201 )
2008
31
Recurrent autoimmune inner ear disease (AIED) and polyarteritis nodosa in a patient with large cell lung carcinoma. ( 16552463 )
2007
32
Etanercept treatment for autoimmune inner ear disease: results of a pilot placebo-controlled study. ( 16151336 )
2005
33
Radiology quiz case 1. Autoimmune inner ear disease (AIED). ( 15967892 )
2005
34
Autoimmune inner ear disease. ( 15377956 )
2004
35
Autoimmune inner ear disease: diagnostic and therapeutic approaches in a multidisciplinary setting. ( 12940706 )
2003
36
Autoimmune inner ear disease: diagnostic and advanced treatments. ( 12528256 )
2002
37
Expression of heat shock protein 70 in the cochlea in experimental autoimmune inner ear disease. ( 11913688 )
2002
38
Relationship between three inner ear antigens with different molecular weights and autoimmune inner ear disease. ( 11876599 )
2002
39
Clinical and experimental studies of autoimmune inner ear disease. ( 12211349 )
2002
40
COCH5B2 is a target antigen of anti-inner ear antibodies in autoimmune inner ear diseases. ( 11568667 )
2001
41
Autoimmune inner ear disease: steroid and cytotoxic drug therapy. ( 11816893 )
2001
42
Identification of beta-actin as a candidate autoantigen in autoimmune inner ear disease. ( 11122295 )
2000
43
Autoimmune inner ear disease. ( 11123055 )
2000
44
[The role of heat shock protein 70 and its autoantibody in experimental autoimmune inner ear disease]. ( 12768679 )
2000
45
Sudden hearing loss and autoimmune inner ear disease. ( 10976497 )
2000
46
Experimental autoimmune inner ear disease: an electrocochleographic and histophysiologic study. ( 10823474 )
2000
47
Utility of laboratory testing in autoimmune inner ear disease. ( 10569401 )
1999
48
Sulfoglucuronosyl glycolipids as putative antigens for autoimmune inner ear disease. ( 9628451 )
1998
49
You're the flight surgeon: autoimmune inner ear disease. ( 9441590 )
1997
50
Plasmapheresis in autoimmune inner ear disease: long-term follow-up. ( 9303152 )
1997

Variations for Autoimmune Inner Ear Disease

Expression for Autoimmune Inner Ear Disease

Search GEO for disease gene expression data for Autoimmune Inner Ear Disease.

Pathways for Autoimmune Inner Ear Disease

Pathways related to Autoimmune Inner Ear Disease according to GeneCards Suite gene sharing:

(show all 16)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.11 MPO TIMP1 TNF
2 11.78 MMP9 TNF
3 11.69 MMP9 TNF
4 11.62 MMP9 TNF
5 11.59 MPO TNF
6 11.54 MMP9 TNF
7 11.49 MMP9 TIMP1 TNF
8 11.44 MMP9 TIMP1
9
Show member pathways
11.33 MMP9 TIMP1 TNF
10
Show member pathways
11.28 MMP9 TNF
11 11.24 MMP9 TIMP1
12 11.12 TIMP1 TNF
13 10.93 MMP9 TIMP1 TNF
14 10.77 MMP9 TIMP1
15 10.53 MPO TNF
16 10.48 MMP9 MPO TNF

GO Terms for Autoimmune Inner Ear Disease

Cellular components related to Autoimmune Inner Ear Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.26 MMP9 MPO TIMP1 TNF
2 extracellular space GO:0005615 8.92 MMP9 MPO TIMP1 TNF

Biological processes related to Autoimmune Inner Ear Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of protein phosphorylation GO:0001934 9.43 MMP9 TNF
2 negative regulation of apoptotic process GO:0043066 9.43 MMP9 MPO TIMP1
3 defense response GO:0006952 9.4 MPO TNF
4 response to organic substance GO:0010033 9.37 TIMP1 TNF
5 extracellular matrix disassembly GO:0022617 9.32 MMP9 TIMP1
6 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.16 MMP9 TNF
7 cytokine-mediated signaling pathway GO:0019221 9.13 MMP9 TIMP1 TNF
8 negative regulation of growth of symbiont in host GO:0044130 8.62 MPO TNF

Molecular functions related to Autoimmune Inner Ear Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 8.62 TIMP1 TNF

Sources for Autoimmune Inner Ear Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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