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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 54 38
Acute Sensorineural Hearing Loss by Acute Acoustic Trauma or Sudden Deafness or Surgery Induced Acoustic Trauma 54 60
Sudden Deafness 54

Characteristics:

Orphanet epidemiological data:

60
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: 35


External Ids:

KEGG 38 H01704
ICD10 via Orphanet 35 H83.3
Orphanet 60 ORPHA90059
Sources

Summaries for Sudden Sensorineural Hearing Loss

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NIH Rare Diseases : 54 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 acetate and Dexamethasone 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 230)
# Related Disease Score Top Affiliating Genes
1 antiphospholipid syndrome 30.1 APOH F2 F5 MTHFR
2 endocarditis 30.0 APOH F2 IL6
3 peripheral vascular disease 29.9 F2 IL6
4 polyarteritis nodosa 29.7 APOH IL6
5 sickle cell anemia 29.7 F2 MTHFR
6 temporal arteritis 29.7 APOH IL6
7 patent foramen ovale 29.5 APOH F2 F5 MTHFR
8 cerebrovascular disease 29.4 F2 F5 IL6 MTHFR
9 infective endocarditis 29.1 APOH F2 IL6 ITGB3
10 myocardial infarction 29.0 F2 F5 IL6 ITGB3 MTHFR
11 central retinal vein occlusion 28.6 APOH F2 F5 IL6 MTHFR
12 retinal vein occlusion 28.6 APOH F2 F5 IL6 MTHFR
13 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 11.5
14 sensorineural hearing loss 11.5
15 vertigo, benign recurrent 10.4
16 benign paroxysmal positional nystagmus 10.4
17 acoustic neuroma 10.4
18 lupus erythematosus 10.4
19 multiple sclerosis 10.4
20 systemic lupus erythematosus 10.4
21 hepatitis 10.3
22 herpes simplex 10.3
23 pathologic nystagmus 10.3
24 acute myocardial infarction 10.3
25 neuritis 10.3
26 neurilemmoma 10.3
27 paracetamol poisoning 10.2 F2 F5
28 hemoglobin e disease 10.2 F2 F5
29 sagittal sinus thrombosis 10.2 F2 F5
30 factor viii deficiency 10.2 F2 F5
31 dysfibrinogenemia 10.2 F2 F5
32 may-thurner syndrome 10.2 APOH F5
33 inferior vena cava interruption 10.2 F5 MTHFR
34 eclampsia 10.2 F2 MTHFR
35 acanthamoeba keratitis 10.2 APOH F5
36 acute liver failure 10.2 F2 F5
37 amaurosis fugax 10.2 F5 MTHFR
38 pregnancy loss, recurrent 1 10.2 APOH F5
39 factor x deficiency 10.2 F2 F5
40 afibrinogenemia 10.2 F2 F5
41 carotid artery occlusion 10.2 APOH F5
42 meniere disease 10.2
43 langerhans cell histiocytosis 10.2
44 diabetes mellitus 10.2
45 mumps 10.2
46 chlamydia 10.2
47 lyme disease 10.2
48 impotence 10.2
49 hepatitis c 10.2
50 neuroma 10.2

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:

27
# 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:

47
# 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 acetate Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 1177-87-3
2
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 50-02-2 5743
3
Cisplatin Approved Phase 4 15663-27-1 441203 84093 2767
4 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 2,Not Applicable
5 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Not Applicable
6 HIV Protease Inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
7 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
8
protease inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
9 Autonomic Agents Phase 4,Phase 3,Phase 2,Not Applicable
10 BB 1101 Phase 4,Phase 3,Phase 2,Not Applicable
11 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
12 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
13 Antiemetics Phase 4,Phase 3,Phase 2,Not Applicable
14 Gastrointestinal Agents Phase 4,Phase 3,Phase 2,Not Applicable
15 Dexamethasone 21-phosphate Phase 4,Phase 2,Not Applicable
16 glucocorticoids Phase 4,Phase 2,Phase 3,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
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
21
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
22
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
23
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
24 Prednisolone acetate Phase 3
25 Methylprednisolone Acetate Phase 3
26 Protective Agents Phase 3
27 Neuroprotective Agents Phase 3
28 Anesthetics Phase 3
29 Ancrod Approved, Investigational Phase 1, Phase 2 9046-56-4
30 Fibrinolytic Agents Phase 1, Phase 2
31 Anticoagulants Phase 1, Phase 2
32 Pharmaceutical Solutions Phase 1, Phase 2,Phase 2
33 Antirheumatic Agents Phase 1, Phase 2,Phase 2
34 Interleukin 1 Receptor Antagonist Protein Phase 1, Phase 2,Phase 2
35
Fludrocortisone Approved, Investigational Not Applicable 127-31-1 31378
36 Mineralocorticoids Not Applicable
37 Antiviral Agents
38 interferons
39 Anti-Infective Agents
40 Interferon-alpha
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:

42
Neutrophil, Testes, Endothelial, Bone, Brain, Kidney, Cortex
Sources

Publications for Sudden Sensorineural Hearing Loss

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Articles related to Sudden Sensorineural Hearing Loss:

(show top 50) (show all 883)
# Title Authors Year
1
Metformin decreases the risk of sudden sensorineural hearing loss in patients with diabetes mellitus: A 14-year follow-up study. ( 30712377 )
2019
2
Primary Ewing sarcoma of the squamous temporal bone with intracranial and extracranial extension: A rare cause of sudden sensorineural hearing loss. ( 30584673 )
2019
3
Predictive values of neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), and other prognostic factors in pediatric idiopathic sudden sensorineural hearing loss. ( 30784810 )
2019
4
Sudden sensorineural hearing loss in adults. ( 30787010 )
2019
5
Improvement or Recovery From Sudden Sensorineural Hearing Loss With Steroid Therapy Does Not Preclude the Need for MRI to Rule Out Vestibular Schwannoma. ( 30807520 )
2019
6
Sudden Sensorineural Hearing Loss in Children: Clinical Characteristics, Etiology, Treatment Outcomes, and Prognostic Factors. ( 30870353 )
2019
7
Genetic Polymorphisms and Susceptibility to Sudden Sensorineural Hearing Loss: A Systematic Review. ( 30870848 )
2019
8
The atherogenic index (ATH index) as a potential predictive marker of idiopathic sudden sensorineural hearing loss: a case control study. ( 30876416 )
2019
9
Markers of Oxidant-Antioxidant Equilibrium in Patients with Sudden Sensorineural Hearing Loss Treated with Hyperbaric Oxygen Therapy. ( 30881599 )
2019
10
The Natural History of Labyrinthine Hemorrhage in Patients With Sudden Sensorineural Hearing Loss. ( 30909739 )
2019
11
MR evaluation of encephalic leukoaraiosis in sudden sensorineural hearing loss (SSNHL) patients. ( 30465101 )
2019
12
Time and geographic clustering of adult patients with unilateral idiopathic sudden sensorineural hearing loss. ( 30593408 )
2019
13
Hearing Outcome of Low-tone Compared to High-tone Sudden Sensorineural Hearing Loss. ( 30647786 )
2019
14
Sudden sensorineural hearing loss during pregnancy: clinical characteristics, management and outcome. ( 30664387 )
2019
15
Acupuncture for the treatment of sudden sensorineural hearing loss: A systematic review and meta-analysis: Acupuncture for SSNHL. ( 30670271 )
2019
16
Inflammatory cytokines and mononuclear cells in sudden sensorineural hearing loss. ( 30739608 )
2019
17
Post-contrast 3D-FLAIR in idiopathic sudden sensorineural hearing loss. ( 30747317 )
2019
18
Efficacy of combination therapy in adolescent and adult patients with total-deafness sudden sensorineural hearing loss. ( 30762471 )
2019
19
Intratympanic Steroid Use for Sudden Sensorineural Hearing Loss: Current Otolaryngology Practice. ( 30770021 )
2019
20
Nationwide Survey of Intratympanic Steroids for the Management of Sudden Sensorineural Hearing Loss. ( 30772961 )
2019
21
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
22
Association of Sudden Sensorineural Hearing Loss With Risk of Cardiocerebrovascular Disease: A Study Using Data From the Korea National Health Insurance Service. ( 29270613 )
2018
23
Mobile Hearing Testing Applications and the Diagnosis of Sudden Sensorineural Hearing Loss: A Cautionary Tale. ( 29227445 )
2018
24
Hypertrophic Pachymeningitis of the Internal Auditory Canal: A Rare Case of Unilateral Sudden Sensorineural Hearing Loss. ( 29938521 )
2018
25
Assessment of Spontaneous Recovery Rates in Patients With Idiopathic Sudden Sensorineural Hearing Loss. ( 29931029 )
2018
26
Comparison of intratympanic dexamethasone therapy and hyperbaric oxygen therapy for the salvage treatment of refractory high-frequency sudden sensorineural hearing loss. ( 29891394 )
2018
27
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
28
Oral and intratympanic steroid therapy for idiopathic sudden sensorineural hearing loss. ( 29721537 )
2018
29
The role of explorative tympanotomy in patients with sudden sensorineural hearing loss with and without perilymphatic fistula. ( 29055686 )
2018
30
A protocol for a network meta-analysis of interventions to treat patients with sudden sensorineural hearing loss. ( 29769117 )
2018
31
Delayed Recovery in Pediatric Sudden Sensorineural Hearing Loss Predicted via Magnetic Resonance Imaging. ( 29717656 )
2018
32
Inpatient treatment of patients with idiopathic sudden sensorineural hearing loss: a population-based healthcare research study. ( 29330597 )
2018
33
Association of Vertigo With Hearing Outcomes in Patients With Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-analysis. ( 29931169 )
2018
34
Idiopathic Sudden Sensorineural Hearing Loss Is Not a Sentinel Event for Acute Myocardial Infarction. ( 29912831 )
2018
35
Prognostic role of haematological indices in sudden sensorineural hearing loss: Review and meta-analysis. ( 29684383 )
2018
36
Can A Sudden Sensorineural Hearing Loss Occur Due to Miliary Tuberculosis? ( 29036759 )
2018
37
Comparison of 2 and 4 Intratympanic Steroid Injections in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss. ( 29411622 )
2018
38
Association of Idiopathic Sudden Sensorineural Hearing Loss With Affective Disorders. ( 29852049 )
2018
39
The Clinical Value of Vertigo as a Prognostic Indicator of Outcome in Sudden Sensorineural Hearing Loss. ( 29931206 )
2018
40
Mobile phone usage does not affect sudden sensorineural hearing loss. ( 29179790 )
2018
41
Association of Metabolic Syndrome With Sudden Sensorineural Hearing Loss. ( 29450496 )
2018
42
Coexistence of IL-6 -572C/G and ICAM-1 K469E Polymorphisms among Patients with Sudden Sensorineural Hearing Loss. ( 29695657 )
2018
43
The Characteristic and Short-Term Prognosis of Tinnitus Associated with Sudden Sensorineural Hearing Loss. ( 29861716 )
2018
44
Modified Siegel's criteria for sudden sensorineural hearing loss: Reporting recovery outcomes with matched pretreatment hearing grades. ( 29929833 )
2018
45
Effect of Antioxidant Vitamins as Adjuvant Therapy for Sudden Sensorineural Hearing Loss: Systematic Review Study. ( 29929192 )
2018
46
Sudden Sensorineural Hearing Loss in the Department of Defense. ( 29631507 )
2018
47
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
48
Is nucleate cell count and neutrophil to lymphocyte ratio related to patients with audiographically distinct sudden sensorineural hearing loss? ( 29768325 )
2018
49
Comparison between Bilateral and Unilateral Sudden Sensorineural Hearing Loss. ( 29363646 )
2018
50
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
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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+ 67
Show member pathways
 12.7 APOH F2 F5 ITGB3
2
Microglia Activation During Neuroinflammation: Microglia Polarization 66
11.22 IL6 ITGB3
3
Selenium Micronutrient Network 1
Show member pathways
 11.13 F2 IL6 MTHFR
4
Platelet Aggregation Inhibitor Pathway, Pharmacodynamics 62
10.96 F2 ITGB3
5
Hematopoietic Stem Cell Differentiation 1
10.84 IL6 ITGB3
6
Antifolate resistance 38
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 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
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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