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MCID: SDD008
MIFTS: 46

Sudden Sensorineural Hearing Loss

Categories: Ear diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Sudden Sensorineural Hearing Loss

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MalaCards integrated aliases for Sudden Sensorineural Hearing Loss:

Name: Sudden Sensorineural Hearing Loss 53 37
Acute Sensorineural Hearing Loss by Acute Acoustic Trauma or Sudden Deafness or Surgery Induced Acoustic Trauma 53 59
Sudden Deafness 53

Characteristics:

Orphanet epidemiological data:

59
acute sensorineural hearing loss by acute acoustic trauma or sudden deafness or surgery induced acoustic trauma
Prevalence: 1-5/10000 (Europe);

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases
See all MalaCards categories (disease lists)
ICD10: 34


External Ids:

Orphanet 59 ORPHA90059
ICD10 via Orphanet 34 H83.3
KEGG 37 H01704
Sources

Summaries for Sudden Sensorineural Hearing Loss

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NIH Rare Diseases : 53 Sudden sensorineural deafness is a condition that is characterized by rapid, unexplained hearing loss. More specifically, affected people experience a reduction in hearing of greater than 30 decibels, which may occur all at once or over several days. In most cases, only one ear is affected. People with sudden sensorineural deafness often become dizzy, have ringing in their ears (tinnitus), or both (40% of the cases). The condition has a variety of causes, including infection, inflammation, tumors, trauma, exposure to toxins and conditions that affect the inner ear such as Ménière's disease. About half of people with sudden sensorineural deafness will recover some or all of their hearing spontaneously and about 85% of those who receive treatment will recover some of their hearing.

MalaCards based summary : Sudden Sensorineural Hearing Loss, also known as acute sensorineural hearing loss by acute acoustic trauma or sudden deafness or surgery induced acoustic trauma, is related to antiphospholipid syndrome and endocarditis. An important gene associated with Sudden Sensorineural Hearing Loss is F5 (Coagulation Factor V), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Microglia Activation During Neuroinflammation: Microglia Polarization. The drugs Dexamethasone and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, testes and endothelial, and related phenotypes are Increased shRNA abundance and homeostasis/metabolism

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Related Diseases for Sudden Sensorineural Hearing Loss

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Diseases related to Sudden Sensorineural Hearing Loss via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 222)
# Related Disease Score Top Affiliating Genes
1 antiphospholipid syndrome 30.3 APOH F2 F5 MTHFR
2 endocarditis 30.1 APOH F2 IL6
3 patent foramen ovale 29.7 APOH F2 F5 MTHFR
4 temporal arteritis 29.7 APOH IL6
5 cerebrovascular disease 29.6 F2 F5 IL6 MTHFR
6 myocardial infarction 29.5 F2 F5 IL6 ITGB3 MTHFR
7 infective endocarditis 29.4 APOH F2 IL6 ITGB3
8 central retinal vein occlusion 29.0 APOH F2 F5 IL6 MTHFR
9 retinal vein occlusion 29.0 APOH F2 F5 IL6 MTHFR
10 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 11.5
11 sensorineural hearing loss 11.5
12 vertigo, benign recurrent 10.4
13 benign paroxysmal positional nystagmus 10.4
14 lupus erythematosus 10.4
15 multiple sclerosis 10.4
16 systemic lupus erythematosus 10.4
17 viral infectious disease 10.4
18 hepatitis 10.3
19 acoustic neuroma 10.3
20 herpes simplex 10.3
21 pathologic nystagmus 10.3
22 acute myocardial infarction 10.3
23 neuritis 10.3
24 langerhans cell histiocytosis 10.2
25 mumps 10.2
26 chlamydia 10.2
27 lyme disease 10.2
28 impotence 10.2
29 hepatitis c 10.2
30 neuroma 10.2
31 iron metabolism disease 10.2
32 ischemia 10.2
33 histiocytosis 10.2
34 hyperglycemia 10.2
35 sickle cell disease 10.2
36 paracetamol poisoning 10.1 F2 F5
37 hemoglobin e disease 10.1 F2 F5
38 sagittal sinus thrombosis 10.1 F2 F5
39 factor viii deficiency 10.1 F2 F5
40 dysfibrinogenemia 10.1 F2 F5
41 may-thurner syndrome 10.1 APOH F5
42 inferior vena cava interruption 10.1 F5 MTHFR
43 acute liver failure 10.1 F2 F5
44 acanthamoeba keratitis 10.1 APOH F5
45 amaurosis fugax 10.1 F5 MTHFR
46 eclampsia 10.1 F2 MTHFR
47 pregnancy loss, recurrent 1 10.1 APOH F5
48 factor x deficiency 10.1 F2 F5
49 afibrinogenemia 10.1 F2 F5
50 carotid artery occlusion 10.1 APOH F5

Graphical network of the top 20 diseases related to Sudden Sensorineural Hearing Loss:



Diseases related to Sudden Sensorineural Hearing Loss
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Symptoms & Phenotypes for Sudden Sensorineural Hearing Loss

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GenomeRNAi Phenotypes related to Sudden Sensorineural Hearing Loss according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance GR00327-A 8.8 F5 ITGB3 MTHFR

MGI Mouse Phenotypes related to Sudden Sensorineural Hearing Loss:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.73 APOH F2 F5 IL6 ITGB3 MTHFR
2 integument MP:0010771 9.55 F2 F5 IL6 ITGB3 MTHFR
3 liver/biliary system MP:0005370 9.26 F5 IL6 ITGB3 MTHFR
4 mortality/aging MP:0010768 9.1 APOH F2 F5 IL6 ITGB3 MTHFR
Sources

Drugs & Therapeutics for Sudden Sensorineural Hearing Loss

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Drugs for Sudden Sensorineural Hearing Loss (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 41)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 50-02-2 5743
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 1177-87-3
3
Cisplatin Approved Phase 4 15663-27-1 2767 441203 84093
4 BB 1101 Phase 4,Phase 3,Phase 2,Not Applicable
5 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
6
protease inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
7 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Not Applicable
8 Dexamethasone 21-phosphate Phase 4,Phase 2,Not Applicable
9 HIV Protease Inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
10 Antiemetics Phase 4,Phase 3,Phase 2,Not Applicable
11 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
12 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
13 Autonomic Agents Phase 4,Phase 3,Phase 2,Not Applicable
14 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
15 Gastrointestinal Agents Phase 4,Phase 3,Phase 2,Not Applicable
16 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Not Applicable
17 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
18
Prednisone Approved, Vet_approved Phase 2, Phase 3,Phase 3 53-03-2 5865
19
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
20
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
21
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
22
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
23
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
24 Neuroprotective Agents Phase 3
25 Prednisolone acetate Phase 3
26 Protective Agents Phase 3
27 Methylprednisolone acetate Phase 3
28 Anesthetics Phase 3
29 Ancrod Approved, Investigational Phase 1, Phase 2 9046-56-4
30 Anticoagulants Phase 1, Phase 2
31 Pharmaceutical Solutions Phase 1, Phase 2,Phase 2
32 Fibrinolytic Agents Phase 1, Phase 2
33 Interleukin 1 Receptor Antagonist Protein Phase 1, Phase 2,Phase 2
34 Antirheumatic Agents Phase 1, Phase 2,Phase 2
35
Fludrocortisone Approved, Investigational Not Applicable 127-31-1 31378
36 Mineralocorticoids Not Applicable
37 Interferon-alpha
38 Anti-Infective Agents
39 Antiviral Agents
40 interferons
41 Immunologic Factors

Interventional clinical trials:

(show all 21)
# Name Status NCT ID Phase Drugs
1 Prevention of Cisplatin-Induced Hearing Loss by Intratympanic Dexamethasone Treatment Completed NCT01372904 Phase 4 Dexamethasone Phosphate
2 Bed Rest for Idiopathic Sudden Sensorineural Hearing Loss Unknown status NCT00416143 Phase 2, Phase 3 prednisone - oral corticosteroid 1mg/kg/D for 1 week
3 Sudden Deafness Treatment Trial Completed NCT00097448 Phase 3 prednisone;methylprednisolone sodium succinate
4 Safety and Efficacy of Intratympanic Application of Dexamethasone Via Catheter in Patients With Sudden Hearing Loss Completed NCT00335920 Phase 3 Dexamethasone-dihydrogenphosphate (4mg/ml)
5 AM-111 in the Treatment of Acute Inner Ear Hearing Loss Completed NCT02561091 Phase 3 AM-111 0.4 mg/ml;AM-111 0.8 mg/ml
6 Efficacy of SENS 401 in Subjects With Severe or Profound Sudden Sensorineural Hearing Loss Not yet recruiting NCT03603314 Phase 2, Phase 3 SENS-401;SENS-401
7 Safety and Efficacy of STR001-IT and STR001-ER in Patients With SSHL Not yet recruiting NCT03331627 Phase 3 STR001-IT and STR001-ER
8 Efficacy and Safety of AM-111 as Acute Sudden Sensorineural Hearing Loss Treatment Terminated NCT02809118 Phase 3 AM-111 0.4 mg/ml;AM-111 0.8 mg/ml
9 Efficacy, Safety, and Tolerability of Ancrod in Patients With Sudden Hearing Loss Completed NCT01621256 Phase 1, Phase 2 Ancrod;Saline solution
10 The Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01950312 Phase 2 gevokizumab
11 Efficacy of AM-111 in Patients With Acute Sensorineural Hearing Loss Completed NCT00802425 Phase 2 AM-111;placebo
12 A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease Completed NCT01267994 Phase 1, Phase 2 Anakinra
13 High Dose Oral Steroids in Sudden Sensorineural Hearing Loss Recruiting NCT03255473 Phase 2 Dexamethasone;Prednisone
14 Study of the Effects of Anakinra in Corticosteroid-resistant Subjects With Sudden Sensorineural Hearing Loss Terminated NCT02414152 Phase 1, Phase 2 anakinra
15 Fludrocortisone for Sudden Hearing Loss Unknown status NCT01186185 Not Applicable Fludrocortisone
16 Sudden Hearing Loss Multi-center Clinical Trial Unknown status NCT02026479 Not Applicable Dexamethasone Phosphate;Dexamethasone Phosphate;Ginaton
17 The Effect of Antiviral Therapy With Pegylated Interferon-alpha on Auditory Disability Completed NCT01265693
18 Effects of Repetitive Hyperbaric Oxygen Therapy in Patients With Acute Ischaemic Stroke Recruiting NCT03431402 Not Applicable Hyperbaric oxygen
19 OSA and Sudden Sensorineural Hearing Loss Enrolling by invitation NCT03747874 Not Applicable
20 Hyperbaric Oxygen Therapy Registry Enrolling by invitation NCT02483650
21 Ischemia- Modified Albumin Levels in the Sudden Hearing Loss Not yet recruiting NCT03706183

Search NIH Clinical Center for Sudden Sensorineural Hearing Loss

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Genetic Tests for Sudden Sensorineural Hearing Loss

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Anatomical Context for Sudden Sensorineural Hearing Loss

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MalaCards organs/tissues related to Sudden Sensorineural Hearing Loss:

41
Neutrophil, Testes, Endothelial, Bone, Brain, Kidney, Heart
Sources

Publications for Sudden Sensorineural Hearing Loss

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Articles related to Sudden Sensorineural Hearing Loss:

(show top 50) (show all 860)
# Title Authors Year
1
Prognostic factors regarding the hearing outcome in severe to profound sudden sensorineural hearing loss treated by tympanotomy and sealing of labyrinthine windows after ineffective systemic corticosteroid application. ( 29855690 )
2018
2
Association of Sudden Sensorineural Hearing Loss With Risk of Cardiocerebrovascular Disease: A Study Using Data From the Korea National Health Insurance Service. ( 29270613 )
2018
3
Mobile Hearing Testing Applications and the Diagnosis of Sudden Sensorineural Hearing Loss: A Cautionary Tale. ( 29227445 )
2018
4
Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss. ( 29938521 )
2018
5
Assessment of Spontaneous Recovery Rates in Patients With Idiopathic Sudden Sensorineural Hearing Loss. ( 29931029 )
2018
6
Comparison of intratympanic dexamethasone therapy and hyperbaric oxygen therapy for the salvage treatment of refractory high-frequency sudden sensorineural hearing loss. ( 29891394 )
2018
7
Efficacy of systemic and intratympanic corticosteroid combination therapy versus intratympanic or systemic therapy in patients with idiopathic sudden sensorineural hearing loss: a randomized controlled trial. ( 29149379 )
2018
8
Oral and intratympanic steroid therapy for idiopathic sudden sensorineural hearing loss. ( 29721537 )
2018
9
The role of explorative tympanotomy in patients with sudden sensorineural hearing loss with and without perilymphatic fistula. ( 29055686 )
2018
10
A protocol for a network meta-analysis of interventions to treat patients with sudden sensorineural hearing loss. ( 29769117 )
2018
11
Delayed Recovery in Pediatric Sudden Sensorineural Hearing Loss Predicted via Magnetic Resonance Imaging. ( 29717656 )
2018
12
Inpatient treatment of patients with idiopathic sudden sensorineural hearing loss: a population-based healthcare research study. ( 29330597 )
2018
13
Association of Vertigo With Hearing Outcomes in Patients With Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis. ( 29931169 )
2018
14
Idiopathic Sudden Sensorineural Hearing Loss Is Not a Sentinel Event for Acute Myocardial Infarction. ( 29912831 )
2018
15
Prognostic role of haematological indices in sudden sensorineural hearing loss: Review and meta-analysis. ( 29684383 )
2018
16
Can A Sudden Sensorineural Hearing Loss Occur Due to Miliary Tuberculosis? ( 29036759 )
2018
17
Comparison of 2 and 4 Intratympanic Steroid Injections in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss. ( 29411622 )
2018
18
Association of Idiopathic Sudden Sensorineural Hearing Loss With Affective Disorders. ( 29852049 )
2018
19
The Clinical Value of Vertigo as a Prognostic Indicator of Outcome in Sudden Sensorineural Hearing Loss. ( 29931206 )
2018
20
Mobile phone usage does not affect sudden sensorineural hearing loss. ( 29179790 )
2018
21
Association of Metabolic Syndrome With Sudden Sensorineural Hearing Loss. ( 29450496 )
2018
22
Coexistence of IL-6 -572C/G and ICAM-1 K469E Polymorphisms among Patients with Sudden Sensorineural Hearing Loss. ( 29695657 )
2018
23
The Characteristic and Short-Term Prognosis of Tinnitus Associated with Sudden Sensorineural Hearing Loss. ( 29861716 )
2018
24
Modified Siegel's criteria for sudden sensorineural hearing loss: Reporting recovery outcomes with matched pretreatment hearing grades. ( 29929833 )
2018
25
Effect of Antioxidant Vitamins as Adjuvant Therapy for Sudden Sensorineural Hearing Loss: Systematic Review Study. ( 29929192 )
2018
26
Sudden Sensorineural Hearing Loss in the Department of Defense. ( 29631507 )
2018
27
Acupuncture as an early treatment for idiopathic sudden sensorineural hearing loss (ISSNHL) patients with flat or high-frequency drop audiograms: study protocol for a randomized controlled trial. ( 29973265 )
2018
28
Is nucleate cell count and neutrophil to lymphocyte ratio related to patients with audiographically distinct sudden sensorineural hearing loss? ( 29768325 )
2018
29
Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss. ( 29363646 )
2018
30
Letter to the Editor Regarding Comparison of 2 and 4 Intratympanic Steroid Injections in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss by Suzuki et al. ( 29911911 )
2018
31
Does the addition of hyperbaric oxygen therapy to conventional treatment modalities influence the outcome of soldiers with idiopathic sudden sensorineural hearing loss? ( 29431146 )
2018
32
International consensus (ICON) on treatment of sudden sensorineural hearing loss. ( 29396226 )
2018
33
Predicting the hearing outcome in sudden sensorineural hearing loss via machine learning models. ( 29356346 )
2018
34
Trough-Shaped Audiograms Are Common in Patients with Acoustic Neuroma and Sudden Sensorineural Hearing Loss. ( 30021194 )
2018
35
Bilateral sudden sensorineural hearing loss as a first symptom of infective endocarditis: two case reports. ( 29463328 )
2018
36
Risk of developing sudden sensorineural hearing loss in patients with hepatitis B virus infection: A population-based study. ( 30481851 )
2018
37
Video head impulse test results suggest that different pathomechanisms underlie sudden sensorineural hearing loss with vertigo and vestibular neuritis: Our experience in fifty-two patients. ( 30027648 )
2018
38
Migraine increases the proportion of sudden sensorineural hearing loss: A longitudinal follow-up study. ( 30389313 )
2018
39
Patterns of nystagmus conversion in sudden sensorineural hearing loss with vertigo. ( 30412127 )
2018
40
Intratympanic Methylprednisolone versus Dexamethasone for the Primary Treatment of Idiopathic Sudden Sensorineural Hearing Loss. ( 30431012 )
2018
41
MR evaluation of encephalic leukoaraiosis in sudden sensorineural hearing loss (SSNHL) patients. ( 30465101 )
2018
42
Intratympanic methylprednisolone administration promotes the recovery of idiopathic sudden sensorineural hearing loss: a retrospective case-control study. ( 30465620 )
2018
43
Audiological outcomes in sudden sensorineural hearing loss with presumed inner ear hemorrhage. ( 30473168 )
2018
44
MTHFR AND ApoE genetic variants association with sudden sensorineural hearing loss. ( 30477909 )
2018
45
Intratympanic Dexamethasone via Saturated Gelfoam for Idiopathic Sudden Sensorineural Hearing Loss. ( 30511891 )
2018
46
Pretreatment Hearing Level-Another Prognostic Factor in Sudden Sensorineural Hearing Loss. ( 30520942 )
2018
47
Pretreatment Hearing Level-Another Prognostic Factor in Sudden Sensorineural Hearing Loss-Reply. ( 30520981 )
2018
48
The Efficacy of Concurrent or Sequential Intravenous and Intratympanic Steroid for Idiopathic Sudden Sensorineural Hearing Loss. ( 30537751 )
2018
49
Circulating Immune Complexes and Heat Shock Protein 70 in the Sera of Patients with Sudden Sensorineural Hearing Loss. ( 30541735 )
2018
50
Vestibular Dysfunctions in Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis. ( 29459846 )
2018
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Variations for Sudden Sensorineural Hearing Loss

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Expression for Sudden Sensorineural Hearing Loss

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Search GEO for disease gene expression data for Sudden Sensorineural Hearing Loss.
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Pathways for Sudden Sensorineural Hearing Loss

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Pathways related to Sudden Sensorineural Hearing Loss according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Response to elevated platelet cytosolic Ca2+ 66
Show member pathways
 12.7 APOH F2 F5 ITGB3
2
Microglia Activation During Neuroinflammation: Microglia Polarization 65
11.22 IL6 ITGB3
3
Selenium Micronutrient Network 1
Show member pathways
 11.13 F2 IL6 MTHFR
4
Platelet Aggregation Inhibitor Pathway, Pharmacodynamics 61
10.96 F2 ITGB3
5
Hematopoietic Stem Cell Differentiation 1
10.84 IL6 ITGB3
6
Antifolate resistance 37
10.45 IL6 MTHFR
Sources

GO Terms for Sudden Sensorineural Hearing Loss

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Cellular components related to Sudden Sensorineural Hearing Loss according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum lumen GO:0005788 8.8 F2 F5 IL6

Biological processes related to Sudden Sensorineural Hearing Loss according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.63 F2 F5 IL6
2 blood coagulation GO:0007596 9.61 F2 F5 ITGB3
3 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.54 IL6 ITGB3
4 hemostasis GO:0007599 9.52 F2 F5
5 blood circulation GO:0008015 9.51 F5 MTHFR
6 acute-phase response GO:0006953 9.49 F2 IL6
7 positive regulation of JAK-STAT cascade GO:0046427 9.48 F2 IL6
8 blood coagulation, intrinsic pathway GO:0007597 9.4 APOH F2
9 positive regulation of blood coagulation GO:0030194 9.37 APOH F2
10 platelet degranulation GO:0002576 9.33 APOH F5 ITGB3
11 regulation of blood coagulation GO:0030193 9.32 APOH F2
12 negative regulation of fibrinolysis GO:0051918 9.26 APOH F2
13 negative regulation of lipid storage GO:0010888 8.96 IL6 ITGB3
14 platelet activation GO:0030168 8.8 F2 IL6 ITGB3
Sources

Sources for Sudden Sensorineural Hearing Loss

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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
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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