MCID: SNS001
MIFTS: 55

Sensorineural Hearing Loss malady

Ear category

Summaries for Sensorineural Hearing Loss

Sources:
64Wikipedia, 47OMIM, 33MalaCards
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Wikipedia:64 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 whole blood, brain and cortex, and related mouse phenotype hearing/vestibular/ear.

Description from OMIM:47 304400

Aliases & Classifications for Sensorineural Hearing Loss

Sources:
8Disease Ontology, 22GTR, 10DISEASES, 61UMLS, 45Novoseek, 57SNOMED-CT, 27ICD9CM, 35MeSH, 47OMIM, 40NCIt
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Classifications:

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


Aliases & Descriptions:

sensorineural hearing loss 8 22 10 61
sensory hearing loss 8 45
hearing loss, high-frequency 61
high frequency hearing loss 8
high-frequency hearing loss 8
hearing loss high-frequency 45
hearing loss sensorineural 45
perceptive hearing loss 8
high frequency deafness 8
sensorineural deafness 8
deafness sensorineural 45
hearing loss, central 61
complete hearing loss 61
central hearing loss 8
perceptive deafness 8
deafness 8


Related Diseases for Sensorineural Hearing Loss

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 1327)
idRelated DiseaseScoreTop Affiliating Genes
1pendred syndrome30.6SLC26A4, GJB2
2enlarged vestibular aqueduct30.6GJB2, SLC26A4
3melas syndrome30.4MT-ND6
4dfnb130.4GJB2
5clouston syndrome30.1GJB2, GJB3
6nonsyndromic hearing loss and deafness, mitochondrial29.9MT-TS1, MT-RNR1
7mitochondrial disorders29.4MT-ND6, MT-TL1
8congenital cytomegalovirus10.7
9cytomegalovirus infection10.6
10siderosis10.6
11renal tubular acidosis10.6
12superficial siderosis10.6
13albinism ocular late onset sensorineural deafness10.5
14hypoparathyroidism10.5
15otosclerosis10.5
16n syndrome10.5
17renal tubular acidosis, distal10.5
18ataxia10.5
19arthrogryposis-like hand anomaly and sensorineural deafness10.5
20lyme disease10.5
21auditory neuropathy10.5
22cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss10.5
23perilymphatic fistula10.5
24renal dysplasia10.4
25bjornstad syndrome10.4
26wolfram syndrome10.4
27pregnancy loss10.4
28histiocytosis with joint contractures and sensorineural deafness10.4
29bart-pumphrey syndrome10.4
30thiamine-responsive megaloblastic anemia syndrome10.4
31keratoderma palmoplantar deafness10.4
32aortitis10.3
33microcephaly10.3
34cleft palate10.3
35acoustic neuroma10.3
36arthritis10.3
37suppurative otitis media10.3
38micro syndrome10.3
39autosomal dominant alport syndrome10.3
40dominant optic atrophy10.3
41pili torti10.3
42ectodermal dysplasia, sensorineural hearing loss, and distinctive facial features10.3
43mondini dysplasia10.3
44nonsyndromic hereditary sensorineural hearing loss10.3
45otospondylomegaepiphyseal dysplasia10.3
46knuckle pads, leuconychia and sensorineural deafness10.3
47sesame syndrome10.3
48familial deafness10.3
49chudley-mccullough syndrome10.3
50ectodermal dysplasia - sensorineural 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

Sources:
47OMIM
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Clinical features from OMIM:

304400

Drugs & Therapeutics for Sensorineural Hearing Loss

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-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

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

Sources:
33MalaCards
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MalaCards organs/tissues related to Sensorineural Hearing Loss:

33
Whole blood, Brain, Cortex, Retina, Kidney, Thyroid, Skin, T cells, Endothelial, Pituitary

Animal Models for Sensorineural Hearing Loss or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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MGI Mouse Phenotypes related to Sensorineural Hearing Loss:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537710.8SLC26A4, GJB2, DLX5, GIPC3, POU3F4, ATOH1

Publications for Sensorineural Hearing Loss

Sources:
51PubMed
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Articles related to Sensorineural Hearing Loss:

(show top 50)    (show all 744)
idTitleAuthorsYear
1
Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss. (24359976)
2014
2
Non-syndromic hereditary sensorineural hearing loss: review of the genes involved. (24423691)
2014
3
Comparison of speech discrimination in noise and directional hearing with 2 different sound processors of a bone-anchored hearing system in adults with unilateral severe or profound sensorineural hearing loss. (23856626)
2013
4
Spectrum and frequency of GJB2 mutations in a cohort of 264 Portuguese nonsyndromic sensorineural hearing loss patients. (23668481)
2013
5
Traumatic labyrinthine concussion in a patient with sensorineural hearing loss. (23859168)
2013
6
Idiopathic pachymeningitis presenting with progressive sensorineural hearing loss, tinnitus and confusion. (23739554)
2013
7
Temporal bone histopathology case of the month: Modiolar dehiscence associated with sensorineural hearing loss. (23235552)
2013
8
Endothelin-1 gene polymorphism in sudden sensorineural hearing loss. (23832707)
2013
9
The Many Faces of Sensorineural Hearing Loss: One Founder and Two Novel Mutations Affecting One Family of Mixed Jewish Ancestry. (24367894)
2013
10
Recurrent Vogt-Koyanagi-Harada disease with sensorineural hearing loss and choroidal thickening. (24022645)
2013
11
Efficacy of intratympanic steroid therapy for idiopathic sudden sensorineural hearing loss: comparison with systemic steroid therapy and combined therapy. (23356871)
2013
12
Efficacy of intratympanic steroid administration on idiopathic sudden sensorineural hearing loss in comparison with hyperbaric oxygen therapy. (22447636)
2012
13
Early combination treatment with intratympanic steroid injection in severe to profound sudden sensorineural hearing loss improves speech discrimination performance. (22160102)
2012
14
Hyperbaric oxygen therapy for idiopathic sudden sensorineural hearing loss. (22670557)
2012
15
Spontaneous intracranial hypotension presenting with severe sensorineural hearing loss and headache. (22722142)
2012
16
Analysis of frequency loss as a prognostic factor in idiopathic sensorineural hearing loss. (22497556)
2012
17
Value of systematic aetiological investigation in children with sensorineural hearing loss. (22633142)
2012
18
Molecular investigation of pediatric portuguese patients with sensorineural hearing loss. (22567359)
2011
19
Late-onset sensorineural hearing loss due to asymptomatic congenital cytomegalovirus infection retrospectively diagnosed by polymerase chain reaction using preserved umbilical cord. (20837611)
2011
20
A case of cochlear nerve deficiency without profound sensorineural hearing loss. (21358452)
2011
21
Therapy of idiopathic sudden sensorineural hearing loss with intratympanic steroid injection. (22295738)
2011
22
AMTAS(Ar): automated method for testing auditory sensitivity: III. sensorineural hearing loss and air-bone gaps. (21668325)
2011
23
Bilateral superior semicircular canal dehiscence in a child with sensorineural hearing loss and without vestibular symptoms. (21524803)
2011
24
Rule 3,000: a more reliable precursor to perceive vestibular schwannoma on MRI in screened asymmetric sensorineural hearing loss. (20835831)
2011
25
Sensorineural hearing loss after concurrent chemoradiotherapy in nasopharyngeal cancer patients. (21333025)
2011
26
Intratympanic methylprednisolone as first-line therapy in sudden sensorineural hearing loss: preliminary results from a case-control series. (21806858)
2011
27
Acute transient sensorineural hearing loss due to Anaplasma phagocytophilum. (22324206)
2011
28
Idiopathic sudden sensorineural hearing loss: evolution in the presence of hypertension, diabetes mellitus and dyslipidemias. (20658017)
2010
29
The effects of glutathione enhancement on sensorineural hearing loss. (20859867)
2010
30
A truncating mutation in SERPINB6 is associated with autosomal- recessive nonsyndromic sensorineural hearing loss. (20451170)
2010
31
GJB2 mutations and genotype-phenotype correlation in 335 patients from germany with nonsyndromic sensorineural hearing loss: evidence for additional recessive mutations not detected by current methods. (20234132)
2010
32
Is computed tomography (CT) or magnetic resonance imaging (MRI) more useful in the evaluation of pediatric sensorineural hearing loss? (21108424)
2010
33
Multivariate analysis of hearing outcomes in patients with idiopathic sudden sensorineural hearing loss. (20879814)
2010
34
Sensorineural hearing loss in patients with mixed connective tissue disease: immunological markers and cytokine levels. (19684145)
2009
35
Mutations in TMC1 contribute significantly to nonsyndromic autosomal recessive sensorineural hearing loss: a report of five novel mutations. (19187973)
2009
36
Sensorineural hearing loss due to secondary syphilis. (18482972)
2008
37
Genetic investigation of autosomal recessive distal renal tubular acidosis: evidence for early sensorineural hearing loss associated with mutations in the ATP6V0A4 gene. (16611712)
2006
38
Bilateral sudden sensorineural hearing loss as a presentation of metastatic adenocarcinoma of unknown primary mimicking cerebellopontine angle tumor on the magnetic resonance image. (16500481)
2006
39
A novel mutation of POU3F4 causes congenital profound sensorineural hearing loss in a large Chinese family. (16735904)
2006
40
Optic atrophy and sensorineural hearing loss in a family caused by an R445H OPA1 mutation. (16158427)
2005
41
Congenital cataract, muscular hypotonia, developmental delay and sensorineural hearing loss associated with a defect in copper metabolism. (15902551)
2005
42
Sensorineural hearing loss in Jewish children born in Jerusalem. (15364494)
2004
43
DFNB40, a recessive form of sensorineural hearing loss, maps to chromosome 22q11.21-12.1. (14512974)
2003
44
Late-onset, progressive sensorineural hearing loss after severe neonatal respiratory failure. (11981395)
2002
45
Sensorineural hearing loss and the incidence of Cx26 mutations in Austria. (11313763)
2001
46
De novo mutation of the connexin 26 gene associated with dominant non-syndromic sensorineural hearing loss. (11354642)
2001
47
Sensorineural hearing loss and Mondini dysplasia caused by a deletion at locus DFN3. (10979118)
2000
48
Amelogenesis imperfecta, sensorineural hearing loss, and Beau's lines, a second case report of Heimler's syndrome. (10636745)
1999
49
Sensorineural hearing loss and nail patella syndrome. (8198793)
1994
50
Progressive optic atrophy and sensorineural hearing loss due to chronic glue sniffing. (6644323)
1983

Genetic Variations for Sensorineural Hearing Loss

Expression for genes affiliated with Sensorineural Hearing Loss

Sources:
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

Sources:
54Reactome, 30KEGG, 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

Sources:
60Tocris Bioscience, 29IUPHAR, 24HMDB, 45Novoseek, 11DrugBank
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Compounds related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
110panx6010.7GJB2, GJB3
2gap 276010.7GJB3, GJB2
3carbenoxolone disodium6010.6GJB3, GJB2
4scrambled 10panx6010.6GJB3, GJB2
5ca2+2910.6GJB2, GJB3
6octanol29 2411.5GJB2, GJB3
7carbenoxolone29 45 1112.3GJB2, GJB3

GO Terms for genes affiliated with Sensorineural Hearing Loss

Sources:
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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  • Proteins
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  • Antibodies

Sources for Sensorineural Hearing Loss

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet