MCID: SNS001
MIFTS: 56

Sensorineural Hearing Loss malady

Ear diseases category

Summaries for Sensorineural Hearing Loss

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63Wikipedia, 46OMIM, 32MalaCards
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Wikipedia:63 Sensorineural hearing loss (SNHL) is a type of hearing loss in which the root cause lies in the... more...

MalaCards: Sensorineural Hearing Loss, also known as sensory hearing loss, is related to pendred syndrome and enlarged vestibular aqueduct. An important gene associated with Sensorineural Hearing Loss is DLX5 (distal-less homeobox 5), and among its related pathways are tRNA Aminoacylation and Transcription factors in neurogenesis. The compounds 10panx and gap 27 have been mentioned in the context of this disorder. Affiliated tissues include brain, bone and testes, and related mouse phenotype hearing/vestibular/ear.

Description from OMIM:46 304400

Aliases & Classifications for Sensorineural Hearing Loss

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Sources:
8Disease Ontology, 22GTR, 10DISEASES, 44Novoseek, 60UMLS, 56SNOMED-CT, 27ICD9CM, 34MeSH, 46OMIM, 39NCIt
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Ear diseases


Aliases & Descriptions:

sensorineural hearing loss 8 22 10 60
sensory hearing loss 8 44
hearing loss, high-frequency 60
high frequency hearing loss 8
high-frequency hearing loss 8
hearing loss high-frequency 44
hearing loss sensorineural 44
perceptive hearing loss 8
high frequency deafness 8
sensorineural deafness 8
deafness sensorineural 44
hearing loss, central 60
complete hearing loss 60
central hearing loss 8
perceptive deafness 8
deafness 8


Related Diseases for Sensorineural Hearing Loss

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to Sensorineural Hearing Loss via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 1326)
idRelated DiseaseScoreTop Affiliating Genes
1pendred syndrome30.6SLC26A4, GJB2
2enlarged vestibular aqueduct30.6GJB2, SLC26A4
3melas syndrome30.3MT-ND6
4nonsyndromic deafness30.1COCH, POU3F4, GIPC3, GJB3, GJB2, SLC26A4
5clouston syndrome30.1GJB2, GJB3
6nonsyndromic hearing loss and deafness, mitochondrial29.9MT-TS1, MT-RNR1
7dfnb129.8GJB2
8mitochondrial disorders29.6MT-ND6, MT-TL1
9siderosis10.5
10renal tubular acidosis10.5
11otitis media10.5
12hypoparathyroidism10.5
13otosclerosis10.5
14meningitis10.5
15neuropathy10.5
16optic atrophy10.5
17arthrogryposis-like hand anomaly and sensorineural deafness10.5
18bjornstad syndrome10.4
19lyme disease10.4
20cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss10.4
21cardiomyopathy, dilated, 1j10.4
22wolfram syndrome10.4
23hypertension10.4
24labyrinthitis10.4
25maternally inherited diabetes and deafness10.4
26deafness, x-linked 210.4
27deafness, autosomal dominant 4b10.4
28deafness, x-linked 110.4
29histiocytosis with joint contractures and sensorineural deafness10.4
30bart-pumphrey syndrome10.3
31aortitis10.3
32microcephaly10.3
33cleft palate10.3
34acoustic neuroma10.3
35arthritis10.3
36dilated cardiomyopathy10.3
37middle ear disease10.3
38migraine10.3
39neuroma10.3
40suppurative otitis media10.3
41optic atrophy type 110.3
42autosomal dominant alport syndrome10.3
43ectodermal dysplasia, sensorineural hearing loss, and distinctive facial features10.3
44jones syndrome10.3
45nonsyndromic hereditary sensorineural hearing loss10.3
46otospondylomegaepiphyseal dysplasia10.3
47thiamine-responsive megaloblastic anemia syndrome10.3
48knuckle pads, leuconychia and sensorineural deafness10.3
49renal tubular acidosis with deafness10.3
50autosomal recessive nonsyndromic deafness10.3

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



Diseases related to sensorineural hearing loss

Clinical Features for Sensorineural Hearing Loss

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Sources:
46OMIM
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Clinical features from OMIM:

304400

Drugs & Therapeutics for Sensorineural Hearing Loss

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Sensorineural Hearing Loss

Drug clinical trials:

Search ClinicalTrials for Sensorineural Hearing Loss

Search NIH Clinical Center for Sensorineural Hearing Loss

Search CenterWatch for Sensorineural Hearing Loss

Genetic Tests for Sensorineural Hearing Loss

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Sources:
22GTR
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Genetic tests related to Sensorineural Hearing Loss:

id Genetic test Affiliating Genes
1 Sensorineural Hearing Loss22

Anatomical Context for Sensorineural Hearing Loss

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32MalaCards
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MalaCards organs/tissues related to Sensorineural Hearing Loss:

32
Brain, Bone, Testes, Endothelial, Retina, Pituitary, T cells, Thyroid, Kidney, Skin, Cortex

Animal Models for Sensorineural Hearing Loss or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Sensorineural Hearing Loss:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537710.8SLC26A4, GJB2, DLX5, GIPC3, POU3F4, ATOH1

Publications for Sensorineural Hearing Loss

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50PubMed
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Articles related to Sensorineural Hearing Loss:

(show top 50)    (show all 743)
idTitleAuthorsYear
1
Non-flat audiograms in sensorineural hearing loss and speech perception. (23778471)
2013
2
Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and MAcniA"re's disease. (23560644)
2013
3
Migraine is a risk factor for sudden sensorineural hearing loss: a nationwide population-based study. (23197354)
2013
4
Routine testing for Borrelia serology in sudden sensorineural hearing loss. (23243138)
2013
5
Sensorineural hearing loss incidence among U.S. military aviators between 1997 and 2011. (24024310)
2013
6
Sensorineural hearing loss amplifies neural coding of envelope information in the central auditory system of chinchillas. (24315815)
2013
7
Reply to 'migraine, sudden sensorineural hearing loss and autoimmune ear disease'. (23615492)
2013
8
PECONPI: a novel software for uncovering pathogenic copy number variations in non-syndromic sensorineural hearing loss and other genetically heterogeneous disorders. (23897863)
2013
9
Steroids for idiopathic sudden sensorineural hearing loss. (23818120)
2013
10
Homozygous and compound heterozygous mutations in the ATP6V1B1 gene in patients with renal tubular acidosis and sensorineural hearing loss. (22509993)
2013
11
Developmental venous anomaly of the internal auditory canal in a child with unilateral sensorineural hearing loss--a rare association. (22402831)
2012
12
Sensorineural hearing loss in acromegalic patients under treatment. (22936144)
2012
13
Comparing treatments for idiopathic sudden sensorineural hearing loss. (24237291)
2012
14
Heat shock protein 70 gene polymorphisms in sudden sensorineural hearing loss. (22922572)
2012
15
Molecular and hereditary mechanisms of sensorineural hearing loss with focus on selected endocrinopathies. (22808909)
2012
16
Hyperbaric oxygen for idiopathic sudden sensorineural hearing loss and tinnitus. (23076907)
2012
17
Benign paroxysmal positional vertigo accompanied by sudden sensorineural hearing loss: a comparative study with idiopathic benign paroxysmal positional vertigo. (22915291)
2012
18
Variability in voice fundamental frequency of sustained vowels in speakers with sensorineural hearing loss. (21227645)
2012
19
Contribution of complement factor H Y402H polymorphism to sudden sensorineural hearing loss risk and possible interaction with diabetes. (22426290)
2012
20
The design and screening of drugs to prevent acquired sensorineural hearing loss. (22646075)
2011
21
Daily short-term intratympanic dexamethasone treatment alone as an initial or salvage treatment for idiopathic sudden sensorineural hearing loss. (20962524)
2011
22
Efficacy comparison of oral steroid, intratympanic steroid, hyperbaric oxygen and oral steroidA +A hyperbaric oxygen treatments in idiopathic sudden sensorineural hearing loss cases. (21431435)
2011
23
Intratympanic dexamethasone as initial therapy for idiopathic sudden sensorineural hearing loss: Clinical evaluation and laboratory investigation. (20817429)
2011
24
Are systemic oral steroids effective for sensorineural hearing loss in bullous myringitis? Considerations from a preliminary study. (21838095)
2011
25
Intratympanic steroid injections as a salvage treatment for sudden sensorineural hearing loss: a randomized, double-blind, placebo-controlled study. (21646929)
2011
26
Newly recognized recessive syndrome characterized by dysmorphic features, hypogonadotropic hypogonadism, severe microcephaly, and sensorineural hearing loss maps to 3p21.3. (22002932)
2011
27
Congenital cytomegalovirus infection as a cause of sensorineural hearing loss in a highly immune population. (21814153)
2011
28
Sensorineural hearing loss in pediatric celiac patients. (21067821)
2011
29
The importance of an extended preoperative trial of BAHA in unilateral sensorineural hearing loss: a prospective cohort study. (21883961)
2011
30
Gipc3 mutations associated with audiogenic seizures and sensorineural hearing loss in mouse and human. (21326233)
2011
31
Autosomal dominant optic neuropathy and sensorineural hearing loss associated with a novel mutation of WFS1. (20069065)
2010
32
Prevalence of acoustic neuroma associated with each configuration of pure tone audiogram in patients with asymmetric sensorineural hearing loss. (21033029)
2010
33
Acupuncture for refractory cases of sudden sensorineural hearing loss. (20704516)
2010
34
Intratympanic prednisolone therapy for sudden sensorineural hearing loss: A new protocol. (20735183)
2010
35
Intratympanic steroids in severe to profound sudden sensorineural hearing loss as salvage treatment. (20978628)
2010
36
Improvements in speech understanding with wireless binaural broadband digital hearing instruments in adults with sensorineural hearing loss. (20457725)
2010
37
Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]. (19705751)
2009
38
De novo 9 Mb deletion of 6q23.2q24.1 disrupting the gene EYA4 in a patient with sensorineural hearing loss, cardiac malformation, and mental retardation. (19576303)
2009
39
A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. (18571542)
2008
40
Connexin 26 mutation in keratitis-ichthyosis-deafness (KID) syndrome in mother and daughter with combined conductive and sensorineural hearing loss. (18412859)
2008
41
Impact of methionine synthase gene and methylenetetrahydrofolate reductase gene polymorphisms on the risk of sudden sensorineural hearing loss. (16778415)
2006
42
A diagnostic paradigm for childhood idiopathic sensorineural hearing loss. (15577772)
2004
43
Progression of low-frequency sensorineural hearing loss (DFNA6/14-WFS1). (12707188)
2003
44
Three members of a family with pili torti and sensorineural hearing loss: the Bjornstad syndrome. (11807445)
2002
45
Congenital corneal dystrophy and progressive sensorineural hearing loss (Harboyan syndrome) (9805138)
1998
46
Late-onset sensorineural hearing loss in Coffin-Lowry syndrome. (9646311)
1998
47
Evidence linking the 68 kilodalton antigen identified in progressive sensorineural hearing loss patient sera with heat shock protein 70. (7872600)
1995
48
Pleiotropy in Coffin-Lowry syndrome: sensorineural hearing deficit and premature tooth loss as early manifestations. (7681250)
1993
49
Sensorineural hearing loss associated to gonadal dysgenesis in sisters: Perrault's syndrome. (1598993)
1992
50
Steroid-responsive sensorineural hearing loss associated with aortitis syndrome. (1970149)
1990

Genetic Variations for Sensorineural Hearing Loss

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Expression for genes affiliated with Sensorineural Hearing Loss

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Sensorineural Hearing Loss

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

Pathways for genes affiliated with Sensorineural Hearing Loss

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Sources:
53Reactome, 29KEGG, 12EMD Millipore
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Pathways related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.6MT-TL1, MT-TS1, HARS2
210.6DLX5, POU3F4, ATOH1

Compounds for genes affiliated with Sensorineural Hearing Loss

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Sources:
59Tocris Bioscience, 28IUPHAR, 24HMDB, 44Novoseek, 11DrugBank
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Compounds related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
110panx5910.7GJB2, GJB3
2gap 275910.7GJB3, GJB2
3carbenoxolone disodium5910.6GJB3, GJB2
4scrambled 10panx5910.6GJB3, GJB2
5ca2+2810.6GJB2, GJB3
6octanol28 2411.5GJB2, GJB3
7carbenoxolone28 44 1112.3GJB2, GJB3

GO Terms for genes affiliated with Sensorineural Hearing Loss

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16Gene Ontology
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Cellular components related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1connexon complexGO:00592210.6GJB2, GJB3

Biological processes related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1sensory perception of soundGO:00760510.8SLC26A4, GJB2, GJB3, POU3F4, WFS1, COCH

Molecular functions related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1gap junction channel activityGO:00524310.6GJB2, GJB3

Products for genes affiliated with Sensorineural Hearing Loss

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Sensorineural Hearing Loss

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet