MCID: SNS001
MIFTS: 60

Sensorineural Hearing Loss

Categories: Ear diseases, Rare diseases

Aliases & Classifications for Sensorineural Hearing Loss

MalaCards integrated aliases for Sensorineural Hearing Loss:

Name: Sensorineural Hearing Loss 12 77 30 6 15 17 74
Sensory Hearing Loss 12 56 74
Hearing Loss, High-Frequency 45 74
Hearing Loss, Sensorineural 45 41
Hearing Loss, Central 45 74
Perceptive Hearing Loss or Deafness 12
High Frequency Hearing Loss 12
High-Frequency Hearing Loss 12
Hearing Loss High-Frequency 56
Hearing Loss Sensorineural 56
High Frequency Deafness 12
Perceptive Hearing Loss 12
Sensorineural Deafness 12
Deafness Sensorineural 56
Central Hearing Loss 12
Perceptive Deafness 12

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Ear diseases


Summaries for Sensorineural Hearing Loss

MalaCards based summary : Sensorineural Hearing Loss, also known as sensory hearing loss, is related to deafness, autosomal dominant 22 and deafness, x-linked 2, and has symptoms including tinnitus An important gene associated with Sensorineural Hearing Loss is WFS1 (Wolframin ER Transmembrane Glycoprotein), and among its related pathways/superpathways is Gap junction trafficking. The drugs Cisplatin and Dexamethasone have been mentioned in the context of this disorder. Affiliated tissues include bone, testes and brain, and related phenotypes are Dynamic nuclei (hole, folded or small irregular) and hearing/vestibular/ear

Wikipedia : 77 Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the inner... more...

Related Diseases for Sensorineural Hearing Loss

Diseases in the Sensorineural Hearing Loss family:

Nonsyndromic Hereditary Sensorineural Hearing Loss

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

(show top 50) (show all 1007)
# Related Disease Score Top Affiliating Genes
1 deafness, autosomal dominant 22 33.9 COCH MYH9 MYO7A
2 deafness, x-linked 2 33.9 GJB2 GJB6
3 deafness, autosomal recessive 6 33.2 GJB2 MYO7A USH2A
4 deafness, autosomal recessive 1a 33.2 GJB2 GJB3 GJB6 OTOF
5 deafness, autosomal dominant 6 33.2 COCH GJB2 MITF MYO7A OTOF SLC26A4
6 deafness, autosomal recessive 9 33.2 GJB2 OTOF
7 deafness, autosomal dominant 48 33.1 MYH9 MYO7A
8 deafness, autosomal recessive 16 33.0 COCH GJB2 GJB6 MYO7A OTOF SLC26A4
9 deafness, autosomal recessive 2 33.0 GJB2 MYO7A OTOF
10 deafness, autosomal recessive 23 33.0 GJB2 GJB6 MYO7A SLC26A4
11 auditory neuropathy, autosomal dominant, 1 32.9 GJB2 OTOF
12 knuckle pads, leukonychia, and sensorineural deafness 32.8 GJB2 GJB6
13 deafness, autosomal recessive 4, with enlarged vestibular aqueduct 32.8 ATP6V1B1 COCH GJB2 GJB3 GJB6 MYO7A
14 deafness, autosomal dominant 36 32.8 GJB2 SLC26A4
15 deafness, autosomal dominant 2a 32.8 COCH GJB2 GJB6 KCNQ4 WFS1
16 deafness, autosomal recessive 28 32.8 GJB2 GJB3
17 deafness, autosomal dominant 13 32.7 COCH GJB2 MYO7A OTOF SLC26A4 USH2A
18 deafness, autosomal recessive 3 32.4 GJB2 MYO7A
19 mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratoderma 32.2 GJB2 GJB3 MITF
20 waardenburg syndrome, type 4a 32.0 MITF RET
21 usher syndrome, type id 31.9 MYO7A USH2A
22 autosomal recessive nonsyndromic deafness 31.6 GJB2 OTOF SLC26A4
23 deafness, autosomal dominant 24 31.6 GJB2 GJB6
24 deafness, autosomal recessive 26 31.5 GJB2 OTOF SLC26A4
25 deafness, autosomal recessive 30 31.5 GJB2 MYH9 MYO7A SLC26A4
26 deafness, autosomal recessive 85 31.5 MYO7A OTOF SLC26A4
27 deafness, autosomal recessive 83 31.5 MYO7A OTOF SLC26A4
28 autosomal recessive nonsyndromic deafness 3 31.5 GJB2 MYH9 MYO7A OTOF SLC26A4
29 deafness, autosomal recessive 59 31.5 GJB2 OTOF
30 auditory neuropathy spectrum disorder 31.5 MYO7A OTOF
31 congenital cytomegalovirus 31.5 GJB2 GJB6
32 hypotrichosis-deafness syndrome 31.4 GJB2 GJB3
33 deafness, autosomal recessive 93 31.4 GJB2 GJB3
34 autosomal dominant nonsyndromic deafness 31.4 COCH MYO7A WFS1
35 retinitis pigmentosa-deafness syndrome 31.0 MYO7A USH2A
36 knuckle pads 30.9 GJB2 GJB3 GJB6
37 autosomal recessive non-syndromic sensorineural deafness type dfnb 30.9 GJB2 GJB3 GJB6 KARS MYH9 MYO7A
38 non-syndromic genetic deafness 30.9 COCH GJB2 KARS KCNQ4 MYH9 OTOF
39 dfnb1 30.9 GJB2 GJB6 MYO7A OTOF
40 deafness, autosomal recessive 33 30.8 GJB2 RET
41 mutism 30.7 GJB2 MITF
42 kid syndrome 30.6 GJB2 GJB3 GJB6
43 clouston syndrome 30.5 GJB2 GJB3 GJB6
44 vohwinkel syndrome 30.5 GJB2 GJB6
45 waardenburg's syndrome 30.2 GJB2 MITF RET
46 auditory system disease 30.2 ATP6V1B1 COCH GJB2 GJB3 GJB6 KCNQ4
47 nonsyndromic deafness 29.7 COCH GJB2 GJB3 GJB6 KARS KCNQ4
48 split-hand/foot malformation 1 with sensorineural hearing loss, autosomal recessive 12.8
49 corneal dystrophy and perceptive deafness 12.8
50 cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss 12.7

Comorbidity relations with Sensorineural Hearing Loss via Phenotypic Disease Network (PDN):


Acute Cystitis Deficiency Anemia
Heart Disease Hypertension, Essential
Schizophreniform Disorder

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



Diseases related to Sensorineural Hearing Loss

Symptoms & Phenotypes for Sensorineural Hearing Loss

UMLS symptoms related to Sensorineural Hearing Loss:


tinnitus

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

27
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Dynamic nuclei (hole, folded or small irregular) GR00257-A-1 9.28 COCH GJB3 KCNQ4 LMX1A MYH9 RET

MGI Mouse Phenotypes related to Sensorineural Hearing Loss:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 hearing/vestibular/ear MP:0005377 10.03 ATP6V1B1 COCH GJB2 GJB6 KCNQ4 LMX1A
2 behavior/neurological MP:0005386 10.02 ATP6V1B1 GJB6 LMX1A MITF MYO7A OTOF
3 homeostasis/metabolism MP:0005376 9.93 ATP6V1B1 GJB2 GJB3 GJB6 LMX1A MITF
4 nervous system MP:0003631 9.77 ATP6V1B1 GJB2 GJB6 KCNQ4 LMX1A MITF
5 no phenotypic analysis MP:0003012 9.32 GJB2 GJB3 GJB6 LMX1A MITF MYH9

Drugs & Therapeutics for Sensorineural Hearing Loss

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

(show top 50) (show all 138)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cisplatin Approved Phase 4,Phase 1,Phase 2 15663-27-1 2767 441203 84093
2
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 50-02-2 5743
3
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 1177-87-3
4
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
5
protease inhibitors Phase 4,Phase 2,Phase 3
6 Hormones Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
7 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
8 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
9 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
10 glucocorticoids Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
11 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
12 Hormone Antagonists Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
13 HIV Protease Inhibitors Phase 4,Phase 2,Phase 3
14 Dexamethasone 21-phosphate Phase 4,Phase 2
15 Antiemetics Phase 4,Phase 3,Phase 2,Not Applicable
16 Gastrointestinal Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable
17 Autonomic Agents Phase 4,Phase 3,Phase 2,Not Applicable
18 BB 1101 Phase 4,Phase 3,Phase 2,Not Applicable
19
Prednisone Approved, Vet_approved Phase 2, Phase 3,Phase 3 53-03-2 5865
20
Valganciclovir Approved, Investigational Phase 3,Phase 2 175865-60-8 64147
21
Acyclovir Approved Phase 2, Phase 3 59277-89-3 2022
22
Acetylcysteine Approved, Investigational Phase 2, Phase 3,Phase 1 616-91-1 12035
23
Deferiprone Approved Phase 2, Phase 3 30652-11-0 2972
24
Metformin Approved Phase 2, Phase 3 657-24-9 14219 4091
25
Iron Approved, Experimental Phase 2, Phase 3 7439-89-6, 15438-31-0 27284 23925
26
Magnesium Sulfate Approved, Investigational, Vet_approved Phase 3 7487-88-9 24083
27
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
28
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
29
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
30
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
31
Medroxyprogesterone acetate Approved, Investigational Phase 3 71-58-9
32
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
33
Polyestradiol phosphate Approved Phase 3 28014-46-2
34
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
35
Mannitol Approved, Investigational Phase 3 69-65-8 6251 453
36
Povidone Approved Phase 3 9003-39-8
37
leucovorin Approved Phase 3,Not Applicable 58-05-9 143 6006
38
Methotrexate Approved Phase 3 1959-05-2, 59-05-2 126941
39
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
40
Calcium Approved, Nutraceutical Phase 3 7440-70-2 271
41
Folic Acid Approved, Nutraceutical, Vet_approved Phase 3,Not Applicable 59-30-3 6037
42
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
43 Pharmaceutical Solutions Phase 3,Phase 1,Phase 2,Not Applicable
44 Anti-Infective Agents Phase 3,Phase 2,Phase 1
45 Antiviral Agents Phase 3,Phase 2,Phase 1
46 Protective Agents Phase 2, Phase 3,Phase 3,Phase 1
47 Free Radical Scavengers Phase 2, Phase 3,Phase 1
48 Incretins Phase 2, Phase 3
49 Iron Chelating Agents Phase 2, Phase 3
50 Sitagliptin Phosphate Phase 2, Phase 3

Interventional clinical trials:

(show top 50) (show all 150)
# Name Status NCT ID Phase Drugs
1 Prevention of Cisplatin-Induced Hearing Loss by Intratympanic Dexamethasone Treatment Completed NCT01372904 Phase 4 Dexamethasone Phosphate
2 Diaphragmatic Movement Before and After Stellate Ganglion Block : A Ultrasonographic Study Completed NCT01054378 Phase 4
3 Bed Rest for Idiopathic Sudden Sensorineural Hearing Loss Unknown status NCT00416143 Phase 2, Phase 3 prednisone - oral corticosteroid 1mg/kg/D for 1 week
4 Congenital Cytomegalovirus: Efficacy of Antiviral Treatment Unknown status NCT02005822 Phase 3 Valganciclovir
5 Efficacy of Antiviral Medications in Controlling Vertigo Attacks of Patients With Meniere's Disease Unknown status NCT01729767 Phase 2, Phase 3 Acyclovir;Placebo
6 Intensity-Modulated Radiation Therapy or 3-Dimensional Conformal Radiation Therapy in Decreasing Hearing Loss in Patients Who Have Undergone Surgery for Parotid Tumors Unknown status NCT01216800 Phase 3
7 Treatment of Wolfram Syndrome Type 2 With the Chelator Deferiprone and Incretin Based Therapy Unknown status NCT02882477 Phase 2, Phase 3 Deferiprone;Acetylcysteine;Sitagliptin and Metformin
8 Efficacy Study of Hypothermia Plus Magnesium Sulphate(MgSO4) in the Management of Term and Near Term Babies With Hypoxic Ischemic Encephalopathy Unknown status NCT01646619 Phase 3 Magnesium Sulphate;Placebo
9 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
10 Safety and Efficacy of Intratympanic Application of Dexamethasone Via Catheter in Patients With Sudden Hearing Loss Completed NCT00335920 Phase 3 Dexamethasone-dihydrogenphosphate (4mg/ml)
11 Evaluation of a Binaural Spatialization Method for Hearing Aids Completed NCT02693704 Phase 2, Phase 3
12 Sudden Deafness Treatment Trial Completed NCT00097448 Phase 3 prednisone;methylprednisolone sodium succinate
13 Somatropin Treatment to Final Height in Turner Syndrome Completed NCT00191113 Phase 3 Somatropin;Ethinyl estradiol;Medroxyprogesterone acetate
14 Safety and Efficacy of STR001-IT and STR001-ER in Patients With SSHL Recruiting NCT03331627 Phase 3 STR001-IT and STR001-ER
15 Cochlear Electrical Impedance and the Effect of Topical Dexamethasone on Cochlear Implant Surgery Recruiting NCT03374514 Phase 3 Dexamethasone;Sterile isotonic saline solution
16 Evaluation of the Benefit of Antiviral Treatment With Valganciclovir on Congenital CMV Infection-related Deafness on Hearing and Balance Recruiting NCT02606266 Phase 2, Phase 3 Valganciclovir
17 A Randomized Trial to Prevent Congenital Cytomegalovirus (CMV) Active, not recruiting NCT01376778 Phase 3 CMV hyperimmune globulin
18 Randomized Controlled Trial of Valganciclovir for Cytomegalovirus Infected Hearing Impaired Infants Enrolling by invitation NCT03107871 Phase 3 Valganciclovir;Simple Syrup
19 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
20 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
21 Congenital Cytomegalovirus: Efficacy of Antiviral Treatment in a Randomized Controlled Trial Terminated NCT01655212 Phase 3 Valganciclovir
22 Autoimmunity in Inner Ear Disease Terminated NCT00000361 Phase 3 Corticosteroids;Methotrexate
23 Effectiveness of P02 Digital Hearing Aids Unknown status NCT01902914 Phase 1, Phase 2
24 Transtympanic Ringer's Lactate for the Prevention of Cisplatin Ototoxicity Unknown status NCT01108601 Phase 1, Phase 2 Ringer's Lactate (0.03% Ciprofloxacin)
25 FX-322 in Sensorineural Hearing Loss Completed NCT03616223 Phase 1, Phase 2 FX-322;Placebo
26 Efficacy of AM-111 in Patients With Acute Sensorineural Hearing Loss Completed NCT00802425 Phase 2 AM-111;placebo
27 Efficacy, Safety, and Tolerability of Ancrod in Patients With Sudden Hearing Loss Completed NCT01621256 Phase 1, Phase 2 Ancrod;Saline solution
28 Safety of Autologous Stem Cell Infusion for Children With Acquired Hearing Loss Completed NCT02038972 Phase 1, Phase 2
29 A Clinical Trial of Anakinra for Steroid-Resistant Autoimmune Inner Ear Disease Completed NCT01267994 Phase 1, Phase 2 Anakinra
30 Study to Evaluate SPI-1005 in Adults With Meniere's Disease Completed NCT02603081 Phase 1, Phase 2 SPI-1005
31 Efficacy of AM 101 in Patients With Acute Inner Ear Tinnitus Completed NCT00860808 Phase 2 AM-101
32 Efficacy of Eltrombopag to Improve Thrombocytopenia of MYH9-related Disease Completed NCT01133860 Phase 2 eltrombopag
33 The Effects of Gevokizumab in Corticosteroid-resistant Subjects With Autoimmune Inner Ear Disease Completed NCT01950312 Phase 2 gevokizumab
34 High Dose Oral Steroids in Sudden Sensorineural Hearing Loss Recruiting NCT03255473 Phase 2 Dexamethasone;Prednisone
35 Asymptomatic Congenital CMV Treatment Recruiting NCT03301415 Phase 2 Valganciclovir
36 Effectiveness of Therapy Via Telemedicine Following Cochlear Implants Recruiting NCT02497690 Phase 2
37 SPI-1005 for the Treatment of Patients With Meniere's Disease Active, not recruiting NCT03325790 Phase 2 200mg SPI-1005 BID;400mg SPI-1005 BID
38 SPI-1005 for Prevention and Treatment of Tobramycin Induced Ototoxicity Enrolling by invitation NCT02819856 Phase 2 Placebo;SPI-1005 Ebselen 200mg Capsule x1;SPI-1005 Ebselen 200mg Capsule x2;SPI-1005 Ebselen 200mg Capsule x3
39 Autologous Bone Marrow Harvest and Transplant for Sensorineural Hearing Loss Suspended NCT02616172 Phase 1, Phase 2
40 Study of the Effects of Anakinra in Corticosteroid-resistant Subjects With Sudden Sensorineural Hearing Loss Terminated NCT02414152 Phase 1, Phase 2 anakinra
41 A Study Of The Effects Of PF-04958242 In Subjects With Age-Related Hearing Loss Completed NCT01518920 Phase 1 PF-04958242;PF-04958242;Placebo
42 Wide-Bandwidth Open Canal Hearing Aid For Better Multitalker Speech Understanding Completed NCT00582946 Phase 1
43 Study to Determine the Safety, Tolerability, and Pharmacokinetic Profile of HPN-07 and HPN-07 Plus NAC Completed NCT02259595 Phase 1 HPN-07;NAC;Placebo
44 Triamcinolone Levels in Cochlear Perilymph Recruiting NCT03248856 Phase 1 Triamcinolone Acetonide
45 Middle-Ear Implant With MET V Transducer (Aka MET V System) Unknown status NCT01529333 Not Applicable
46 Clinical Applications of Round Window Imaging Anatomy in Cochlear Implant Surgery Unknown status NCT02093806
47 Fludrocortisone for Sudden Hearing Loss Unknown status NCT01186185 Not Applicable Fludrocortisone
48 Evaluation of the High Frequency Digit Triplet Test in Cystic Fibrosis Unknown status NCT02252601 Not Applicable
49 Cochlear Implant and Vestibular Function. Unknown status NCT02584361 Not Applicable
50 Balance Control and Maturation in Normal and Deaf Children Aged From 6 to 14 Years Old Unknown status NCT00140699

Search NIH Clinical Center for Sensorineural Hearing Loss

Cochrane evidence based reviews: hearing loss, central

Genetic Tests for Sensorineural Hearing Loss

Genetic tests related to Sensorineural Hearing Loss:

# Genetic test Affiliating Genes
1 Sensorineural Hearing Loss 30

Anatomical Context for Sensorineural Hearing Loss

MalaCards organs/tissues related to Sensorineural Hearing Loss:

42
Bone, Testes, Brain, Endothelial, Heart, Kidney, Neutrophil

Publications for Sensorineural Hearing Loss

Articles related to Sensorineural Hearing Loss:

(show top 50) (show all 3026)
# Title Authors Year
1
Cerebral blood flow in a migraine patient with fluctuated sensorineural hearing loss. ( 30322732 )
2019
2
Neutrophil-to-Lymphocyte Ratio and Platelet-to-Lymphocyte Ratio in Patients with Sudden Sensorineural Hearing Loss. ( 30326473 )
2019
3
Asymmetric sensorineural hearing loss is a risk factor for late-onset hearing loss in pediatric cancer survivors following cisplatin treatment. ( 30334605 )
2019
4
Migraine increases the proportion of sudden sensorineural hearing loss: A longitudinal follow-up study. ( 30389313 )
2019
5
What defines asymmetric sensorineural hearing loss? ( 30408187 )
2019
6
Radiographic findings in young adults with asymmetric sensorineural hearing loss. ( 30472122 )
2019
7
Audiological outcomes in sudden sensorineural hearing loss with presumed inner ear hemorrhage. ( 30473168 )
2019
8
MTHFR AND ApoE genetic variants association with sudden sensorineural hearing loss. ( 30477909 )
2019
9
In Response to To Image or Not to Image? A Cost-effectiveness Analysis of MRI for Patients With Asymmetric Sensorineural Hearing Loss. ( 30485449 )
2019
10
Intratympanic Dexamethasone via Saturated Gelfoam for Idiopathic Sudden Sensorineural Hearing Loss. ( 30511891 )
2019
11
Assessment of balance skills and falling risk in children with congenital bilateral profound sensorineural hearing loss. ( 30554713 )
2019
12
Time and geographic clustering of adult patients with unilateral idiopathic sudden sensorineural hearing loss. ( 30593408 )
2019
13
Dysfunction of GRAP, encoding the GRB2-related adaptor protein, is linked to sensorineural hearing loss. ( 30610177 )
2019
14
The effect of Tai Chi on balance and functional mobility in children with congenital sensorineural hearing loss. ( 30623683 )
2019
15
Clinical update on sensorineural hearing loss in Turner syndrome and the X-chromosome. ( 30632288 )
2019
16
The Relationship between the Etiology of Profound Prelingual Sensorineural Hearing Loss and the Results of Vestibular-Evoked Myogenic Potentials. ( 30647776 )
2019
17
Hearing Outcome of Low-tone Compared to High-tone Sudden Sensorineural Hearing Loss. ( 30647786 )
2019
18
Sudden sensorineural hearing loss during pregnancy: clinical characteristics, management and outcome. ( 30664387 )
2019
19
Is Sensorineural Hearing Loss Related to Chronic Rhinosinusitis Caused by Outer Hair Cell Injury? ( 30666045 )
2019
20
Acupuncture for the treatment of sudden sensorineural hearing loss: A systematic review and meta-analysis: Acupuncture for SSNHL. ( 30670271 )
2019
21
Sensorineural hearing loss and cognitive impairments: Contributions of thalamus using multiparametric MRI. ( 30694013 )
2019
22
Association of Glutathione s-transferase M1 and T1 gene polymorphisms with the susceptibility to acquired sensorineural hearing loss: a systematic review and meta-analysis. ( 30696891 )
2019
23
Determining the energetic and informational components of speech-on-speech masking in listeners with sensorineural hearing loss. ( 30710924 )
2019
24
Oxidative stress in prelingual sensorineural hearing loss and the effects of cochlear implant application on serum oxidative stress levels. ( 30738337 )
2019
25
Inflammatory cytokines and mononuclear cells in sudden sensorineural hearing loss. ( 30739608 )
2019
26
Sequential versus Combination Treatment Using Steroids and Diuretics for Acute Low-Frequency Sensorineural Hearing Loss: A Noninferiority Trial. ( 30741894 )
2019
27
Post-contrast 3D-FLAIR in idiopathic sudden sensorineural hearing loss. ( 30747317 )
2019
28
Efficacy of combination therapy in adolescent and adult patients with total-deafness sudden sensorineural hearing loss. ( 30762471 )
2019
29
Acute sensorineural hearing loss in patients with vestibular schwannoma early after cyberknife radiosurgery. ( 30769220 )
2019
30
Intratympanic Steroid Use for Sudden Sensorineural Hearing Loss: Current Otolaryngology Practice. ( 30770021 )
2019
31
Nationwide Survey of Intratympanic Steroids for the Management of Sudden Sensorineural Hearing Loss. ( 30772961 )
2019
32
Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI. ( 30773403 )
2019
33
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
34
Sudden sensorineural hearing loss in adults. ( 30787010 )
2019
35
Contralateral sensorineural hearing loss after vestibular schwannoma surgery. ( 29929725 )
2019
36
The prevalence of vestibular schwannoma among patients treated as sudden sensorineural hearing loss. ( 30042019 )
2019
37
Prevalence and etiology of sensorineural hearing loss in children with down syndrome: A cross-sectional study. ( 30554691 )
2019
38
Severe cryopyrin-associated periodic syndrome first characterized by early childhood-onset sensorineural hearing loss - Case report and literature review. ( 30772614 )
2019
39
Patient education in sudden sensorineural hearing loss: Knowledge, attitude/belief, and practice findings among otolaryngologists and otologists in China. ( 30146406 )
2019
40
Impact of metabolic syndrome on recovery of idiopathic sudden sensorineural hearing loss. ( 31109803 )
2019
41
Right-to-left shunt and idiopathic sudden sensorineural hearing loss. ( 31097828 )
2019
42
OTOF mutation analysis with massively parallel DNA sequencing in 2,265 Japanese sensorineural hearing loss patients. ( 31095577 )
2019
43
Recovery From Sudden Sensorineural Hearing Loss May Be Linked to Chronic Stress Levels and Steroid Treatment Resistance. ( 31084569 )
2019
44
Improvement or Recovery From Sudden Sensorineural Hearing Loss With Steroid Therapy Does Not Preclude the Need for MRI to Rule Out Vestibular Schwannoma. ( 31083098 )
2019
45
Characteristics of Mid-Frequency Sensorineural Hearing Loss Progression. ( 31083079 )
2019
46
An unusual case of sudden sensorineural hearing loss after cycling class. ( 31079847 )
2019
47
Magnetic resonance imaging of the endolymphatic space in patients with acute low-tone sensorineural hearing loss. ( 31076273 )
2019
48
White matter lesions and sudden sensorineural hearing loss. ( 31072738 )
2019
49
Idiopathic acute high-tone sensorineural hearing loss accompanied by vertigo: vestibulo-cochlear artery syndrome? Consideration based on VEMP and vHIT. ( 31065783 )
2019
50
Long-term Audiometric Outcomes in Unilateral Sudden Sensorineural Hearing Loss without Recurrence. ( 31058596 )
2019

Variations for Sensorineural Hearing Loss

ClinVar genetic disease variations for Sensorineural Hearing Loss:

6 (show all 44)
# Gene Variation Type Significance SNP ID Assembly Location
1 GABRA1 NM_001127645.1(GABRA1): c.232G> A (p.Gly78Arg) single nucleotide variant Uncertain significance GRCh37 Chromosome 5, 161292771: 161292771
2 GABRA1 NM_001127645.1(GABRA1): c.232G> A (p.Gly78Arg) single nucleotide variant Uncertain significance GRCh38 Chromosome 5, 161865765: 161865765
3 GIPC3 NM_133261.2(GIPC3): c.400G> A (p.Ala134Thr) single nucleotide variant Likely pathogenic GRCh37 Chromosome 19, 3586667: 3586667
4 GIPC3 NM_133261.2(GIPC3): c.400G> A (p.Ala134Thr) single nucleotide variant Likely pathogenic GRCh38 Chromosome 19, 3586669: 3586669
5 CHN1 NM_001822.5(CHN1): c.667G> A (p.Ala223Thr) single nucleotide variant Likely pathogenic GRCh38 Chromosome 2, 174824479: 174824479
6 CHN1 NM_001822.5(CHN1): c.667G> A (p.Ala223Thr) single nucleotide variant Likely pathogenic GRCh37 Chromosome 2, 175689207: 175689207
7 TMCO1 NM_019026.4(TMCO1): c.616C> T (p.Arg206Ter) single nucleotide variant Pathogenic GRCh38 Chromosome 1, 165743172: 165743172
8 TMCO1 NM_019026.4(TMCO1): c.616C> T (p.Arg206Ter) single nucleotide variant Pathogenic GRCh37 Chromosome 1, 165712409: 165712409
9 RET NM_020975.5(RET): c.1280_1281delTG (p.Val427Glyfs) deletion Pathogenic GRCh38 Chromosome 10, 43111223: 43111224
10 RET NM_020975.5(RET): c.1280_1281delTG (p.Val427Glyfs) deletion Pathogenic GRCh37 Chromosome 10, 43606671: 43606672
11 GJB2 NM_004004.5(GJB2): c.196G> T (p.Asp66Tyr) single nucleotide variant Likely pathogenic GRCh38 Chromosome 13, 20189386: 20189386
12 GJB2 NM_004004.5(GJB2): c.196G> T (p.Asp66Tyr) single nucleotide variant Likely pathogenic GRCh37 Chromosome 13, 20763525: 20763525
13 KARS NM_001130089.1(KARS): c.1438del (p.Leu480Trpfs) deletion Pathogenic GRCh38 Chromosome 16, 75630493: 75630493
14 KARS NM_001130089.1(KARS): c.1438del (p.Leu480Trpfs) deletion Pathogenic GRCh37 Chromosome 16, 75664391: 75664391
15 GSDME NM_001127453.1(GSDME): c.456T> G (p.Asn152Lys) single nucleotide variant Uncertain significance rs148449230 GRCh37 Chromosome 7, 24758786: 24758786
16 GSDME NM_001127453.1(GSDME): c.456T> G (p.Asn152Lys) single nucleotide variant Uncertain significance rs148449230 GRCh38 Chromosome 7, 24719167: 24719167
17 ESPN NM_031475.2(ESPN): c.2150A> G (p.Asn717Ser) single nucleotide variant Uncertain significance rs921702586 GRCh38 Chromosome 1, 6451921: 6451921
18 ESPN NM_031475.2(ESPN): c.2150A> G (p.Asn717Ser) single nucleotide variant Uncertain significance rs921702586 GRCh37 Chromosome 1, 6511981: 6511981
19 POGZ NM_015100.3(POGZ): c.2771delC (p.Pro924Argfs) deletion Pathogenic rs1553212868 GRCh38 Chromosome 1, 151406264: 151406264
20 POGZ NM_015100.3(POGZ): c.2771delC (p.Pro924Argfs) deletion Pathogenic rs1553212868 GRCh37 Chromosome 1, 151378740: 151378740
21 LMX1A NM_001174069.1(LMX1A): c.1106T> C (p.Ile369Thr) single nucleotide variant Pathogenic GRCh38 Chromosome 1, 165203923: 165203923
22 LMX1A NM_001174069.1(LMX1A): c.1106T> C (p.Ile369Thr) single nucleotide variant Pathogenic GRCh37 Chromosome 1, 165173160: 165173160
23 TECTA NM_005422.2(TECTA): c.3107G> A (p.Cys1036Tyr) single nucleotide variant Likely pathogenic GRCh37 Chromosome 11, 121008295: 121008295
24 TECTA NM_005422.2(TECTA): c.3107G> A (p.Cys1036Tyr) single nucleotide variant Likely pathogenic GRCh38 Chromosome 11, 121137586: 121137586
25 AIFM1 NM_004208.3(AIFM1): c.1019T> C (p.Met340Thr) single nucleotide variant Likely pathogenic rs1057518895 GRCh37 Chromosome X, 129271109: 129271109
26 AIFM1 NM_004208.3(AIFM1): c.1019T> C (p.Met340Thr) single nucleotide variant Likely pathogenic rs1057518895 GRCh38 Chromosome X, 130137134: 130137134
27 MYH14 NM_001145809.1(MYH14): c.1945+6G> A single nucleotide variant Uncertain significance rs1057518869 GRCh38 Chromosome 19, 50252759: 50252759
28 MYH14 NM_001145809.1(MYH14): c.1945+6G> A single nucleotide variant Uncertain significance rs1057518869 GRCh37 Chromosome 19, 50756016: 50756016
29 COL11A2 NM_080680.2(COL11A2): c.4430G> T (p.Gly1477Val) single nucleotide variant Likely pathogenic rs745434198 GRCh38 Chromosome 6, 33165983: 33165983
30 COL11A2 NM_080680.2(COL11A2): c.4430G> T (p.Gly1477Val) single nucleotide variant Likely pathogenic rs745434198 GRCh37 Chromosome 6, 33133760: 33133760
31 MT-CYB NC_012920.1: m.15127C> T single nucleotide variant Uncertain significance rs1057516074 GRCh38 Chromosome MT, 15127: 15127
32 MT-CYB NC_012920.1: m.15127C> T single nucleotide variant Uncertain significance rs1057516074 GRCh37 Chromosome MT, 15127: 15127
33 46;XY;t(6;11)(p12.3;p14.2)dn Translocation Pathogenic
34 46;XX;t(1;13)(q11.2;p11.2)dn Translocation Uncertain significance
35 46;XY;t(5;7)(q14.3;q21.3)dn Translocation Likely pathogenic
36 46;XY;t(8;9)(q13;p22)dn Translocation Pathogenic
37 MT-TL1 NC_012920.1: m.3243A> G single nucleotide variant Pathogenic rs199474657 GRCh38 Chromosome MT, 3243: 3243
38 MT-TL1 NC_012920.1: m.3243A> G single nucleotide variant Pathogenic rs199474657 GRCh37 Chromosome MT, 3243: 3243
39 WFS1 NM_006005.3(WFS1): c.1999C> T (p.Gln667Ter) single nucleotide variant Pathogenic GRCh38 Chromosome 4, 6301794: 6301794
40 WFS1 NM_006005.3(WFS1): c.1999C> T (p.Gln667Ter) single nucleotide variant Pathogenic GRCh37 Chromosome 4, 6303521: 6303521
41 KIF1A NM_001244008.1(KIF1A): c.3052C> T (p.His1018Tyr) single nucleotide variant Uncertain significance GRCh38 Chromosome 2, 240747247: 240747247
42 KIF1A NM_001244008.1(KIF1A): c.3052C> T (p.His1018Tyr) single nucleotide variant Uncertain significance GRCh37 Chromosome 2, 241686664: 241686664
43 SOX3 NM_005634.2(SOX3): c.735_737dupCGC (p.Ala248_Ser249insAla) duplication Uncertain significance rs398124211 GRCh38 Chromosome X, 140504324: 140504326
44 SOX3 NM_005634.2(SOX3): c.735_737dupCGC (p.Ala248_Ser249insAla) duplication Uncertain significance rs398124211 GRCh37 Chromosome X, 139586489: 139586491

Copy number variations for Sensorineural Hearing Loss from CNVD:

7 (show all 13)
# CNVD ID Chromosom Start End Type Gene Symbol CNVD Disease
1 115552 17 58172730 60315273 Deletion Sensorineural hearing loss
2 115543 17 58121080 60118579 Deletion USP32 sensorineural hearing loss
3 115539 17 58121080 60118579 Deletion TBX4 sensorineural hearing loss
4 115535 17 58121080 60118579 Deletion TBX2 sensorineural hearing loss
5 115531 17 58121080 60118579 Deletion PPM1D sensorineural hearing loss
6 115527 17 58121080 60118579 Deletion NACA2 sensorineural hearing loss
7 115523 17 58121080 60118579 Deletion MED13 sensorineural hearing loss
8 115519 17 58121080 60118579 Deletion INTS2 sensorineural hearing loss
9 115515 17 58121080 60118579 Deletion CA4 sensorineural hearing loss
10 115511 17 58121080 60118579 Deletion BRIP1 sensorineural hearing loss
11 115507 17 58121080 60118579 Deletion BCAS3 sensorineural hearing loss
12 115503 17 58121080 60118579 Deletion APPBP2 sensorineural hearing loss
13 115500 17 58121080 60118579 Deletion Sensorineural hearing loss

Expression for Sensorineural Hearing Loss

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

Pathways for Sensorineural Hearing Loss

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.79 GJB2 GJB3 GJB6

GO Terms for Sensorineural Hearing Loss

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

# Name GO ID Score Top Affiliating Genes
1 apical plasma membrane GO:0016324 9.55 ATP6V1B1 GJB6 MYO7A SLC26A4 USH2A
2 gap junction GO:0005921 9.13 GJB2 GJB3 GJB6
3 connexin complex GO:0005922 8.8 GJB2 GJB3 GJB6

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 inner ear morphogenesis GO:0042472 9.54 ATP6V1B1 KCNQ4 MYO7A
2 actin filament-based movement GO:0030048 9.49 MYH9 MYO7A
3 regulation of pH GO:0006885 9.48 ATP6V1B1 SLC26A4
4 endoplasmic reticulum calcium ion homeostasis GO:0032469 9.46 TMCO1 WFS1
5 sensory perception of light stimulus GO:0050953 9.43 MYO7A USH2A
6 ER overload response GO:0006983 9.4 TMCO1 WFS1
7 sensory perception of sound GO:0007605 9.36 ATP6V1B1 COCH GJB2 GJB6 KCNQ4 MYO7A
8 inner ear development GO:0048839 9.33 GJB2 GJB6 MYO7A
9 inner ear receptor cell differentiation GO:0060113 9.32 MYO7A USH2A
10 olfactory behavior GO:0042048 9.26 LMX1A WFS1
11 cell communication GO:0007154 9.13 GJB2 GJB3 GJB6

Molecular functions related to Sensorineural Hearing Loss according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 microfilament motor activity GO:0000146 9.26 MYH9 MYO7A
2 gap junction channel activity GO:0005243 9.16 GJB2 GJB3
3 actin-dependent ATPase activity GO:0030898 8.96 MYH9 MYO7A
4 calmodulin binding GO:0005516 8.92 KCNQ4 MYH9 MYO7A WFS1

Sources for Sensorineural Hearing Loss

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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