AIED
MCID: ATM021
MIFTS: 40

Autoimmune Inner Ear Disease (AIED)

Categories: Ear diseases, Rare diseases

Aliases & Classifications for Autoimmune Inner Ear Disease

MalaCards integrated aliases for Autoimmune Inner Ear Disease:

Name: Autoimmune Inner Ear Disease 54
Ocular Albinism, Type Ii 74
Aied 54

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases


External Ids:

UMLS 74 C0268505

Summaries for Autoimmune Inner Ear Disease

MalaCards based summary : Autoimmune Inner Ear Disease, also known as ocular albinism, type ii, is related to inner ear disease and arteries, anomalies of. 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 Prednisone have been mentioned in the context of this disorder. Affiliated tissues include lung, bone and testes, and related phenotypes are cardiovascular system and neoplasm

Wikipedia : 77 Autoimmune inner ear disease was first defined by Dr. Brian McCabe in a landmark paper describing an... more...

Related Diseases for Autoimmune Inner Ear Disease

Diseases in the Inner Ear Disease family:

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 270)
# Related Disease Score Top Affiliating Genes
1 inner ear disease 30.3 MMP9 MPO TIMP1 TNF
2 arteries, anomalies of 30.1 MMP9 TNF
3 colitis 29.8 MPO TNF
4 ulcerative colitis 29.5 MPO TNF
5 spinal cord injury 29.4 MMP9 TNF
6 pneumonia 29.3 MPO TNF
7 cerebrovascular disease 29.1 MMP9 TNF
8 vasculitis 29.1 MPO TNF
9 myocardial infarction 28.9 MMP9 MPO TIMP1 TNF
10 androgen insensitivity syndrome 12.0
11 amelogenesis imperfecta 12.0
12 aland island eye disease 11.9
13 scoliosis, isolated 1 11.7
14 hereditary amyloidosis 11.3
15 aortic valve insufficiency 11.3
16 childhood-onset cerebral x-linked adrenoleukodystrophy 11.3
17 pediatric arterial ischemic stroke 11.2
18 amelogenesis imperfecta, type ib 11.2
19 salt and pepper developmental regression syndrome 11.0
20 jalili syndrome 11.0
21 amelogenesis imperfecta, type ie 11.0
22 amelogenesis imperfecta, type ij 11.0
23 alzheimer disease 10.8
24 scoliosis 10.6
25 idiopathic scoliosis 10.6
26 amyloidosis 10.4
27 auditory system disease 10.3
28 adenocarcinoma 10.3
29 dementia 10.2
30 adenocarcinoma in situ 10.2
31 cerebral amyloid angiopathy, cst3-related 10.2
32 coronary artery anomaly 10.2
33 labyrinthitis 10.1
34 pulmonary fibrosis 10.1 MMP9 TIMP1
35 diabetes mellitus 10.1
36 fungal keratitis 10.1 MMP9 TIMP1
37 cryptogenic organizing pneumonia 10.1 MMP9 TIMP1
38 conjunctival nevus 10.1 MMP9 TIMP1
39 nasal cavity squamous cell carcinoma 10.1 MMP9 TIMP1
40 chronic venous insufficiency 10.1 MMP9 TIMP1
41 venous insufficiency 10.1 MMP9 TIMP1
42 central nervous system tuberculosis 10.1 MMP9 TIMP1
43 aortic aneurysm, familial abdominal, 1 10.1 MMP9 TIMP1
44 pulmonary emphysema 10.1 MMP9 TIMP1
45 carotid artery disease 10.1 MMP9 TIMP1
46 neuroblastoma 10.1
47 avian influenza 10.1
48 influenza 10.1
49 depression 10.1
50 varicose veins 10.1 MMP9 TIMP1

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:

47
# 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 31)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
2
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
3
Cyclophosphamide Approved, Investigational Phase 3 6055-19-2, 50-18-0 2907
4
leucovorin Approved Phase 3 58-05-9 6006 143
5
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
6 Antirheumatic Agents Phase 3,Phase 1,Phase 2
7 Antimetabolites Phase 3,Not Applicable
8 Vitamin B Complex Phase 3
9 Immunosuppressive Agents Phase 3
10 Antimetabolites, Antineoplastic Phase 3
11 Immunologic Factors Phase 3
12 Nucleic Acid Synthesis Inhibitors Phase 3
13 Vitamin B9 Phase 3
14 Folate Phase 3
15 Dermatologic Agents Phase 3
16 Folic Acid Antagonists Phase 3
17 Pharmaceutical Solutions Phase 2
18 Interleukin 1 Receptor Antagonist Protein Phase 1, Phase 2,Phase 2
19
Carbon monoxide Approved, Investigational Not Applicable 630-08-0 281
20
Nitric Oxide Approved Not Applicable 10102-43-9 145068
21 Protective Agents Not Applicable
22 Respiratory System Agents Not Applicable
23 Bronchodilator Agents Not Applicable
24 Endothelium-Dependent Relaxing Factors Not Applicable
25 Vasodilator Agents Not Applicable
26 Antioxidants Not Applicable
27 Peripheral Nervous System Agents Not Applicable
28 Neurotransmitter Agents Not Applicable
29 Free Radical Scavengers Not Applicable
30 Anti-Asthmatic Agents Not Applicable
31 Autonomic Agents Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Autoimmunity in Inner Ear Disease Terminated NCT00000361 Phase 3 Corticosteroids;Methotrexate
2 The Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01950312 Phase 2 gevokizumab
3 A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease Completed NCT01267994 Phase 1, Phase 2 Anakinra
4 Effects of Anakinra in Subjects With Autoimmune Inner Ear Disease Recruiting NCT03587701 Phase 2 Anakinra 100Mg/0.67Ml Inj Syringe (Period 1);Placebo injection (Period 1);Anakinra 100Mg/0.67Ml Inj Syringe (Period 2);Placebo injection (Period 2)
5 Intratympanic Injection for Autoimmune Inner Ear Disease Terminated NCT01526174 Phase 1, Phase 2 Golimumab
6 Exposure of Taxi Drivers to Ultrafine Particles and Black Carbon Within Their Vehicles Recruiting NCT03839537 Not Applicable
7 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:

42
Lung, Bone, Testes, Thyroid

Publications for Autoimmune Inner Ear Disease

Articles related to Autoimmune Inner Ear Disease:

(show top 50) (show all 72)
# Title Authors Year
1
Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan's Syndrome. ( 29850616 )
2018
2
Imaging in autoimmune inner-ear disease and endolymphatic hydrops, bone cement for improving hearing outcomes in stapes surgery, and the reporting of results. External ear canal cholesteatoma: a hypothesis. ( 30019670 )
2018
3
Autoimmune inner ear disease secondary to Hashimoto's thyroiditis: a case report. ( 30181832 )
2018
4
Autoimmune inner ear disease (AIED): A diagnostic challenge. ( 30376736 )
2018
5
The Balance of Tissue Inhibitor of Metalloproteinase-1 and Matrix Metalloproteinase-9 in the Autoimmune Inner Ear Disease Patients. ( 28696822 )
2017
6
Autoimmune inner ear disease in a melanoma patient treated with pembrolizumab. ( 26885370 )
2016
7
Systematic Review of Treatments for Autoimmune Inner Ear Disease. ( 26485595 )
2015
8
Progression of autoimmune inner ear disease to labyrinthitis ossificans: clinical and radiologic correlation. ( 25738715 )
2015
9
Current understanding of the pathogenesis of autoimmune inner ear disease: a review. ( 25847404 )
2015
10
Paraneoplastic syndrome: a masquerade of autoimmune inner ear disease. ( 25427223 )
2015
11
N-Acetylcysteine attenuates tumor necrosis factor alpha levels in autoimmune inner ear disease patients. ( 26392121 )
2015
12
Diffuse systemic sclerosis presenting as Meniere's disease-like symptoms as part of autoimmune inner ear disease. ( 26040060 )
2015
13
Early efficacy trial of anakinra in corticosteroid-resistant autoimmune inner ear disease. ( 25133431 )
2014
14
IL-1I^ inhibition in autoimmune inner ear disease: can you hear me now? ( 25133418 )
2014
15
Cochlin in autoimmune inner ear disease: Is the search for an inner ear autoantigen over? ( 25199741 )
2014
16
An open label study to evaluate the safety and efficacy of intratympanic golimumab therapy in patients with autoimmune inner ear disease. ( 25203561 )
2014
17
Cogan's syndrome: an autoimmune inner ear disease. ( 22846458 )
2013
18
Innate immune recognition of molds and homology to the inner ear protein, cochlin, in patients with autoimmune inner ear disease. ( 23912888 )
2013
19
Autoimmune inner ear disease preliminary case report: audiometric findings following steroid treatments. ( 23800807 )
2013
20
Infliximab for autoimmune inner ear disease: case report and literature review. ( 24125068 )
2013
21
Autoimmune inner ear disease: a retrospective review of forty-seven patients. ( 23817208 )
2013
22
Autoimmune inner ear disease in children. ( 21178803 )
2011
23
IL-1I^ is overexpressed and aberrantly regulated in corticosteroid nonresponders with autoimmune inner ear disease. ( 21199898 )
2011
24
High dose combination pertussis toxin induces autoimmune inner ear disease in Sprague-Dawley rats. ( 21366505 )
2011
25
Extraintestinal Crohn's disease mimicking autoimmune inner ear disease: a histopathological approach. ( 20523038 )
2011
26
Labyrinthitis ossificans secondary to autoimmune inner ear disease: a previously unreported condition. ( 20416474 )
2010
27
Cochlear implantation in patients with autoimmune inner ear disease including cogan syndrome: a comparison with age- and sex-matched controls. ( 21082747 )
2010
28
Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease. ( 19395984 )
2009
29
Immunosuppressive therapy for autoimmune inner ear disease. ( 19885385 )
2009
30
Induction of tolerance by oral administration of beta-tubulin in an animal model of autoimmune inner ear disease. ( 19365153 )
2009
31
The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. ( 18777037 )
2009
32
Autoimmune inner ear disease: clinical and laboratory findings and treatment outcome. ( 19128599 )
2008
33
Does the serological study for viral infection in autoimmune inner ear disease make sense? ( 18235201 )
2008
34
Recurrent autoimmune inner ear disease (AIED) and polyarteritis nodosa in a patient with large cell lung carcinoma. ( 16552463 )
2007
35
Etanercept treatment for autoimmune inner ear disease: results of a pilot placebo-controlled study. ( 16151336 )
2005
36
Radiology quiz case 1. Autoimmune inner ear disease (AIED). ( 15967892 )
2005
37
Autoimmune inner ear disease. ( 15377956 )
2004
38
Autoimmune inner ear disease: diagnostic and therapeutic approaches in a multidisciplinary setting. ( 12940706 )
2003
39
Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. ( 14532316 )
2003
40
Autoimmune inner ear disease: diagnostic and advanced treatments. ( 12528256 )
2002
41
Expression of heat shock protein 70 in the cochlea in experimental autoimmune inner ear disease. ( 11913688 )
2002
42
Relationship between three inner ear antigens with different molecular weights and autoimmune inner ear disease. ( 11876599 )
2002
43
Clinical and experimental studies of autoimmune inner ear disease. ( 12211349 )
2002
44
Autoimmune inner ear disease: steroid and cytotoxic drug therapy. ( 11816893 )
2001
45
Identification of beta-actin as a candidate autoantigen in autoimmune inner ear disease. ( 11122295 )
2000
46
Autoimmune inner ear disease. ( 11123055 )
2000
47
[The role of heat shock protein 70 and its autoantibody in experimental autoimmune inner ear disease]. ( 12768679 )
2000
48
Sudden hearing loss and autoimmune inner ear disease. ( 10976497 )
2000
49
Experimental autoimmune inner ear disease: an electrocochleographic and histophysiologic study. ( 10823474 )
2000
50
Utility of laboratory testing in autoimmune inner ear disease. ( 10569401 )
1999

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 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.11 MPO TIMP1 TNF
2 11.79 MMP9 TNF
3 11.71 MMP9 TNF
4
Show member pathways
11.66 MMP9 TNF
5 11.63 MMP9 TNF
6 11.6 MPO TNF
7 11.57 MMP9 TNF
8 11.49 MMP9 TIMP1 TNF
9 11.46 MMP9 TIMP1
10 11.35 MMP9 TNF
11
Show member pathways
11.33 MMP9 TIMP1 TNF
12
Show member pathways
11.28 MMP9 TNF
13 11.24 MMP9 TIMP1
14 11.12 TIMP1 TNF
15 10.93 MMP9 TIMP1 TNF
16 10.77 MMP9 TIMP1
17 10.53 MPO TNF
18 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 negative regulation of apoptotic process GO:0043066 9.5 MMP9 MPO TIMP1
2 defense response GO:0006952 9.4 MPO TNF
3 response to organic substance GO:0010033 9.37 TIMP1 TNF
4 extracellular matrix disassembly GO:0022617 9.26 MMP9 TIMP1
5 positive regulation of vascular smooth muscle cell proliferation GO:1904707 9.16 MMP9 TNF
6 cytokine-mediated signaling pathway GO:0019221 9.13 MMP9 TIMP1 TNF
7 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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