MCID: SDD008
MIFTS: 41

Sudden Sensorineural Hearing Loss

Categories: Ear diseases, Rare diseases

Aliases & Classifications for Sudden Sensorineural Hearing Loss

MalaCards integrated aliases for Sudden Sensorineural Hearing Loss:

Name: Sudden Sensorineural Hearing Loss 20 36
Acute Sensorineural Hearing Loss by Acute Acoustic Trauma or Sudden Deafness or Surgery Induced Acoustic Trauma 20 58
Sudden Deafness 20

Characteristics:


Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases
Orphanet: 58  
Rare otorhinolaryngological diseases


External Ids:

KEGG 36 H01704
ICD10 via Orphanet 33 H83.3
Orphanet 58 ORPHA90059

Summaries for Sudden Sensorineural Hearing Loss

KEGG : 36 Sudden sensorineural hearing loss (SNHL) is commonly defined as hearing loss of more than 30 dB, affecting three or more frequencies, arising over less than 3 days. The predominant form of sudden SNHL is unilateral in location, and the main aetiology is idiopathic. As the majority of patients (30-65 %) with unilateral sudden SNHL report spontaneous recovery within 2 weeks, unilateral disease is mostly regarded as a benign condition. Prognosis for recovery is dependent on a number of factors, including patient age, presence of vertigo at onset, degree of hearing loss, and time between onset of hearing loss and treatment. The pathology of sudden SNHL remains unclear. However, a series of factors, including viral infections, microcirculatory disorders, autoimmune disorders, and labyrinthine hemorrhage, have been proposed as causative factors. Different treatment regimens like antioxidants, corticosteroids, vasodilatators, hyperbaric oxygen (HBO), or carbogen therapy have been described. Among these approaches, steroid treatment is the most common.

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 sensorineural hearing loss and antiphospholipid syndrome. An important gene associated with Sudden Sensorineural Hearing Loss is F5 (Coagulation Factor V), and among its related pathways/superpathways are Folate Metabolism and Platelet Aggregation Inhibitor Pathway, Pharmacodynamics. The drugs Ginkgo and Anesthetics have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, endothelial and cortex, and related phenotypes are Increased shRNA abundance and homeostasis/metabolism

GARD : 20 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 Meniere'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.

Related Diseases for Sudden Sensorineural Hearing Loss

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

(show top 50) (show all 426)
# Related Disease Score Top Affiliating Genes
1 sensorineural hearing loss 31.3 MTR MTHFR IL6 GJB2 F2
2 antiphospholipid syndrome 30.9 MTHFR F5 F2
3 migraine with or without aura 1 30.5 MTHFR IL6 F2
4 patent foramen ovale 30.4 MTHFR F5 F2
5 mastoiditis 30.3 MTHFR F2
6 venous insufficiency 30.3 MTHFR F5 F2
7 homocysteinemia 30.3 MTR MTHFR F5 F2
8 thrombophilia due to activated protein c resistance 30.1 MTHFR F5 F2
9 protein s deficiency 30.1 MTHFR F5 F2
10 pericardial effusion 30.0 IL6 F2
11 exanthem 30.0 IL6 F2
12 osteonecrosis 30.0 MTHFR F2
13 stroke, ischemic 30.0 MTHFR IL6 F5 F2
14 homocystinuria 30.0 MTR MTHFR F5
15 pre-eclampsia 29.8 MTHFR IL6 F5 F2
16 thrombophilia 29.8 MTHFR ITGB3 F5 F2
17 thrombosis 29.6 MTHFR ITGB3 F5 F2
18 central retinal vein occlusion 29.6 MTHFR IL6 F5 F2
19 retinal vein occlusion 29.6 MTHFR IL6 F5 F2
20 infective endocarditis 29.5 ITGB3 IL6 F2
21 disseminated intravascular coagulation 29.5 IL6 F5 F2
22 vascular disease 29.3 MTR MTHFR ITGB3 IL6 F5 F2
23 sickle cell anemia 29.2 MTHFR ITGB3 IL6 F2
24 thrombocytopenia 29.1 MTHFR ITGB3 IL6 F5 F2
25 cerebrovascular disease 28.9 MTR MTHFR ITGB3 IL6 F5 F2
26 myocardial infarction 28.9 MTR MTHFR ITGB3 IL6 F5 F2
27 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 11.6
28 branchiootic syndrome 1 11.0
29 acoustic neuroma 10.7
30 neurilemmoma 10.7
31 benign paroxysmal positional nystagmus 10.6
32 pathologic nystagmus 10.6
33 lipid metabolism disorder 10.6
34 labyrinthitis 10.6
35 neuroma 10.6
36 herpes simplex 10.6
37 autoimmune disease 10.5
38 otitis media 10.5
39 hypercholesterolemia, familial, 1 10.5
40 severe acute respiratory syndrome 10.5
41 meniere disease 10.5
42 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.5
43 neuritis 10.5
44 inner ear disease 10.5
45 hyperglycemia 10.5
46 systemic lupus erythematosus 10.4
47 coronary heart disease 1 10.4
48 covid-19 10.4
49 vestibular neuronitis 10.4
50 lupus erythematosus 10.4

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



Diseases related to Sudden Sensorineural Hearing Loss

Symptoms & Phenotypes for Sudden Sensorineural Hearing Loss

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 8.92 F5 ITGB3 MTHFR MTR

MGI Mouse Phenotypes related to Sudden Sensorineural Hearing Loss:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.8 F2 F5 GJB2 IL6 ITGB3 MTHFR
2 integument MP:0010771 9.63 F2 F5 GJB2 IL6 ITGB3 MTHFR
3 mortality/aging MP:0010768 9.5 F2 F5 GJB2 IL6 ITGB3 MTHFR
4 nervous system MP:0003631 9.1 F2 F5 GJB2 IL6 ITGB3 MTHFR

Drugs & Therapeutics for Sudden Sensorineural Hearing Loss

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

(show all 31)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Ginkgo Phase 4
2 Anesthetics Phase 3
3 Ancrod Approved, Investigational Phase 1, Phase 2 9046-56-4
4
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
5
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
6 Fibrinolytic Agents Phase 1, Phase 2
7 Anticoagulants Phase 1, Phase 2
8 Pharmaceutical Solutions Phase 1, Phase 2
9 BB 1101 Phase 2
10 HIV Protease Inhibitors Phase 2
11
protease inhibitors Phase 2
12 Dexamethasone 21-phosphate Phase 2
13
Prednisolone Approved, Vet_approved 50-24-8 5755
14 Prednisolone acetate Approved, Vet_approved 52-21-1
15
Prednisone Approved, Vet_approved 53-03-2 5865
16
Prednisolone phosphate Approved, Vet_approved 302-25-0
17
Methylprednisolone Approved, Vet_approved 83-43-2 6741
18
Methylprednisolone hemisuccinate Approved 2921-57-5
19
Fludrocortisone Approved, Investigational Early Phase 1 127-31-1 31378
20
Prednisolone hemisuccinate Experimental 2920-86-7
21 Anti-Inflammatory Agents
22 Hormones
23 Protective Agents
24 Hormone Antagonists
25 glucocorticoids
26 Methylprednisolone Acetate
27 Neuroprotective Agents
28 Antineoplastic Agents, Hormonal
29 Antiemetics
30 Gastrointestinal Agents
31 Mineralocorticoids Early Phase 1

Interventional clinical trials:

(show all 16)
# Name Status NCT ID Phase Drugs
1 The Effect of Positive Airway Pressure on Idiopathic Sudden Sensorineural Hearing Loss Comorbided With Obstructive Sleep Apnea: A Clinical Randomized Controlled Study Recruiting NCT04192656 Phase 4 Methylprednisolone Hemisuccinate;Ginaton
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 Hearing Loss Multicenter Treatment Trial Completed NCT00097448 Phase 3 prednisone;methylprednisolone sodium succinate
4 A Prospective, Randomized, Double Blind, Placebo Controlled, Multicenter Study on the Safety and Efficacy of Continuous Infusion of Corticosteroid Delivered Via Catheter in Patients With Idiopathic Sudden Sensorineural Hearing Loss Completed NCT00335920 Phase 3 Dexamethasone-dihydrogenphosphate (4mg/ml)
5 A Phase III Multicenter, Double-blind, Placebo-controlled, Study Evaluating the Safety, and Efficacy of STR001 Treatment in Adults With Sudden Sensorineural Hearing Loss Completed NCT03331627 Phase 3 STR001-IT and STR001-ER
6 A Two-part, Randomized, Double-blind, Placebo-controlled, Parallel-group, Efficacy and Safety Study of SENS-401 in Subjects With Severe or Profound Sudden Sensorineural Hearing Loss Recruiting NCT03603314 Phase 2, Phase 3 SENS-401;SENS-401
7 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
8 Double-blind, Randomized, Placebo-controlled Study on Efficacy, Safety and Tolerability of Ancrod in Patients With Sudden Sensorineural Hearing Loss (SSHL) Completed NCT01621256 Phase 1, Phase 2 Ancrod;Saline solution
9 High Dose Oral Steroids in Sudden Sensorineural Hearing Loss Recruiting NCT03255473 Phase 2 Dexamethasone;Prednisone
10 The Evaluation of the Ischemia-modified Albumin Levels in the Patients With Sudden Sensorineural Hearing Loss Unknown status NCT03706183
11 Prevalence of Obstructive Sleep Apnea Syndrome in Patients With a History of Sudden Sensorineural Hearing Loss Completed NCT03747874
12 Prognosis of Vestibular Dysfunction in Patients With Idiopathic Sudden Sensorineural Hearing Loss Recruiting NCT03951584
13 Video HIT Results in Sudden Sensorineural Hearing Loss Patients Not yet recruiting NCT04224909
14 Vestibular Prognosis Assessment of the Idiopathic Sudden Sensorineural Hearing Loss With Vestibular Dysfunction Treated With Oral or Intratympanic Glucocorticoids Not yet recruiting NCT03974867 Prednisone 5Mg Tab;Methylprednisolone 40 mg
15 Prospective Study of Sudden Deafness Not yet recruiting NCT04129983
16 Mineralocorticoid Treatment for Sudden Sensorineural Hearing Loss Terminated NCT01186185 Early Phase 1 Fludrocortisone

Search NIH Clinical Center for Sudden Sensorineural Hearing Loss

Genetic Tests for Sudden Sensorineural Hearing Loss

Anatomical Context for Sudden Sensorineural Hearing Loss

MalaCards organs/tissues related to Sudden Sensorineural Hearing Loss:

40
Neutrophil, Endothelial, Cortex, Myeloid

Publications for Sudden Sensorineural Hearing Loss

Articles related to Sudden Sensorineural Hearing Loss:

(show top 50) (show all 1689)
# Title Authors PMID Year
1
Clinical Feature and Prognosis of Sudden Sensorineural Hearing Loss With Rheumatoid Arthritis. 61
33021510 2021
2
Radiologically-proven New Development of Superior Semicircular Canal Dehiscence Associated With Development of Superior Semicircular Canal Dehiscence Syndrome. 61
33273305 2021
3
Investigating Facial Nerve Stimulation After Cochlear Implantation in Adult and Pediatric Recipients. 61
32222081 2021
4
Vestibular Neuritis as Clinical Presentation of COVID-19. 61
33570425 2021
5
The audiovestibular manifestations as early symptoms of multiple sclerosis: a scoping review of the literature. 61
33544333 2021
6
Weber test accuracy in sudden sensorineural hearing loss: which frequency is best? 61
33586603 2021
7
Comparison of two different intratympanic corticosteroid injection protocols as salvage treatments for idiopathic sudden sensorineural hearing loss. 61
33591388 2021
8
T2 relaxation time shortening in the cochlea of patients with sudden sensory neuronal hearing loss: a retrospective study using quantitative synthetic magnetic resonance imaging. 61
33609144 2021
9
Hypocomplementemic Urticarial Vasculitis Syndrome revealed by Bilateral Sudden Sensorineural Hearing Loss. 61
33576777 2021
10
Evaluation and comparison of the efficacy of long-acting betamethasone and dexamethasone as injections in the treatment of idiopathic sudden hearing loss. 61
33588138 2021
11
Clinical characteristics and correlation between hearing outcomes after different episodes of recurrent idiopathic sudden sensorineural hearing loss. 61
33549393 2021
12
Clinical Profile of Patients With Unilateral Sudden Sensorineural Hearing Loss: Correlation With Hearing Prognosis. 61
33557702 2021
13
Sudden Sensorineural Hearing Loss in Children-Management and Outcomes: A Meta-analysis. 61
32673420 2021
14
Therapeutic efficacy of super-high-dose steroid therapy in patients with profound sudden sensorineural hearing loss: a comparison with conventional steroid therapy. 61
33176548 2021
15
Isolated Saccular Hydrops on High-resolution MRI Is Associated With Full Spectrum Menière's Disease. 61
33481544 2021
16
Sudden Sensorineural Hearing Loss: A Diagnostic and Therapeutic Emergency. 61
33452100 2021
17
Association between routine hematological parameters and sudden sensorineural hearing loss: A meta-analysis. 61
33505450 2021
18
Physician decision support system for idiopathic sudden sensorineural hearing loss patients. 61
33177402 2021
19
Letter to the Editor regarding "Dexamethasone eardrop with grommet placement vs intratympanic steroid injection for sudden sensorineural hearing loss: A randomized prospective clinical trial". 61
33152575 2021
20
Usefulness of vertebrobasilar artery radiological finding as a predictive and prognostic factor for sudden sensorineural hearing loss. 61
33451886 2021
21
Lesion-specific prognosis by magnetic resonance imaging in sudden sensorineural hearing loss. 61
33043763 2021
22
Early hearing improvement predicts the prognosis of idiopathic sudden sensorineural hearing loss. 61
33389010 2021
23
Bilateral sequential sudden sensorineural hearing loss in protein S deficiency. 61
33417006 2021
24
Predicting hearing recovery following treatment of idiopathic sudden sensorineural hearing loss with machine learning models. 61
33445040 2021
25
Analysis of thyroid dysfunction in patients with sudden sensorineural hearing loss. 61
33474537 2021
26
Efficacy of Various Corticosteroid Treatment Modalities for the Initial Treatment of Idiopathic Sudden Hearing Loss: A Prospective Randomized Controlled Trial. 61
32668428 2021
27
Comparison of Therapeutic Results with/without Additional Hyperbaric Oxygen Therapy in Idiopathic Sudden Sensorineural Hearing Loss: A Randomized Prospective Study. 61
32535600 2021
28
Imaging Parameters of the Ipsilateral Medial Geniculate Body May Predict Prognosis of Patients with Idiopathic Unilateral Sudden Sensorineural Hearing Loss on the Basis of Diffusion Spectrum Imaging. 61
33214182 2021
29
Bilateral Sudden Sensorineural Hearing Loss and Intralabyrinthine Hemorrhage in a Patient With COVID-19. 61
33301283 2021
30
Paediatric Sudden Sensorineural Hearing Loss: Pooled Analysis and Systematic Review. 61
33605224 2021
31
Effectiveness of Ginkgo biloba diterpene lactone in the treatment of sudden sensorineural hearing loss. 61
33454456 2021
32
Idiopathic sudden sensorineural hearing loss: Effectiveness of salvage treatment with low-dose intratympanic dexamethasone. 61
33505443 2021
33
Does Treatment of Sudden Sensorineural Hearing Loss in Patients With COVID-19 Require Anticoagulants? 61
33494644 2021
34
Sudden sensorineural hearing loss in patients with vestibular schwannoma. 61
33479297 2021
35
Clinical Features and Hearing Outcomes of Sudden Sensorineural Hearing Loss in Diabetic Patients. 61
33181868 2021
36
High level of IgE in acute low-tone sensorineural hearing loss: A predictor for recurrence and Meniere Disease transformation. 61
33429184 2021
37
Adjuvant Migraine Medications in the Treatment of Sudden Sensorineural Hearing Loss. 61
32243585 2021
38
Clinical course of a critically ill patient with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). 61
32556649 2020
39
The Clinical Spectrum of Dizziness in Sleep Apnea. 61
32740553 2020
40
Migraine and risk of sudden sensorineural hearing loss: A systematic review and meta-analysis. 61
33364398 2020
41
[Analysis of gadolinium magnetic resonance in inner ear by tympanic membrane puncture in 1 126 cases]. 61
33342145 2020
42
Autoimmune Inner Ear Disease among Patients with Selective IgA Deficiency. 61
33311024 2020
43
Cochlear Implantation in the Active Duty Military Population: A Survey Assessing Military Readiness and Satisfaction. 61
33351567 2020
44
Glucocorticoid and Breviscapine Combination Therapy Versus Glucocorticoid Alone on Sudden Sensorineural Hearing Loss in Patients with Different Audiometric Curves. 61
33034871 2020
45
Sudden sensorineural hearing loss and COVID-19: (commentary on "Could sudden sensorineural hearing loss be the sole manifestation of COVID-19? An investigation into SARS-COV-2 in the etiology of sudden sensorineural hearing loss" by Osman Kilic, Mahmut Tayyar Kalcioglu, Yasemin Cag, Ozan Tuysuz, Emel Pektas, Hulya Caskurlu, and Ferihan Cetın). 61
33010462 2020
46
Differences in hearing recovery following intratympanic alone or intravenous dexamethasone with rescue intratympanic steroids for sudden sensorineural hearing loss: A randomised trial. 61
33369870 2020
47
Psoriasis Increases the Risk of Sudden Sensorineural Hearing Loss: A Longitudinal Follow Up Study Using a National Sample Cohort. 61
33322823 2020
48
What did you say? A review of the management of sudden sensorineural hearing loss. 61
33497084 2020
49
Missing perilymph but leaking blood-endolymph and vestibulocochlear nerve barriers in idiopathic sudden sensorineural hearing loss: A case study. 61
33293920 2020
50
Prediction of hearing outcomes by auditory steady-state response in patients with sudden sensorineural hearing loss. 61
33305656 2020

Variations for Sudden Sensorineural Hearing Loss

Expression for Sudden Sensorineural Hearing Loss

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

Pathways for Sudden Sensorineural Hearing Loss

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.26 MTR MTHFR IL6 F2
2 10.96 ITGB3 F2
3 10.84 ITGB3 IL6
4 10.45 MTHFR IL6

GO Terms for Sudden Sensorineural Hearing Loss

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 IL6 F5 F2

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

# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.5 IL6 F5 F2
2 hemostasis GO:0007599 9.43 F5 F2
3 blood coagulation GO:0007596 9.43 ITGB3 F5 F2
4 acute-phase response GO:0006953 9.4 IL6 F2
5 positive regulation of JAK-STAT cascade GO:0046427 9.37 IL6 F2
6 methionine biosynthetic process GO:0009086 9.16 MTR MTHFR
7 negative regulation of lipid storage GO:0010888 8.96 ITGB3 IL6
8 platelet activation GO:0030168 8.8 ITGB3 IL6 F2

Sources for Sudden Sensorineural Hearing Loss

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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