MCID: SNS001
MIFTS: 57

Sensorineural Hearing Loss malady

Ear diseases category

Summaries for Sensorineural Hearing Loss

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66Wikipedia, 48OMIM, 34MalaCards
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Wikipedia:66 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 bart-pumphrey 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 Cell adhesion Gap junctions and tRNA Aminoacylation. 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 phenotypes are no phenotypic analysis and integument.

Description from OMIM:48 304400

Aliases & Classifications for Sensorineural Hearing Loss

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Sources:
9Disease Ontology, 23GTR, 11DISEASES, 46Novoseek, 63UMLS, 59SNOMED-CT, 28ICD9CM, 36MeSH, 41NCIt, 48OMIM
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Classifications:

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


Aliases & Descriptions:

sensorineural hearing loss 9 23 11 63
sensory hearing loss 9 46
hearing loss, high-frequency 63
high frequency hearing loss 9
high-frequency hearing loss 9
hearing loss high-frequency 46
hearing loss sensorineural 46
high frequency deafness 9
perceptive hearing loss 9
deafness sensorineural 46
sensorineural deafness 9
hearing loss, central 63
complete hearing loss 63
central hearing loss 9
perceptive deafness 9
deafness 9


Related Diseases for Sensorineural Hearing Loss

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Sources:
18GeneCards, 19GeneDecks
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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 1430)
idRelated DiseaseScoreTop Affiliating Genes
1bart-pumphrey syndrome30.6GJB6, GJB2
2enlarged vestibular aqueduct30.6GJB3, GJB2, GJB6
3dfnb130.2GJB6, GJB2
4clouston syndrome30.2GJB6, GJB2, GJB3
5nonsyndromic hearing loss and deafness, mitochondrial30.1MT-TS1, MT-RNR1
6skin disease30.1GJB3, GJB2, GJB6
7ectodermal dysplasia29.9GJB2
8nonsyndromic deafness29.9POU3F4, STRC, COCH, GJB6, GJB2, GJB3
9dfna 3 nonsyndromic hearing loss and deafness29.9GJB6, GJB2
10dfnb 1 nonsyndromic hearing loss and deafness29.8GJB6, GJB2
11mitochondrial disorders29.6MT-TL1
12congenital cytomegalovirus10.7
13cytomegalovirus infection10.6
14siderosis10.6
15renal tubular acidosis10.6
16superficial siderosis10.6
17otosclerosis10.5
18meningitis10.5
19otitis media10.5
20hypoparathyroidism10.5
21neuropathy10.5
22optic atrophy10.5
23superficial siderosis of the central nervous system10.5
24ataxia10.5
25arthrogryposis-like hand anomaly and sensorineural deafness10.5
26lyme disease10.5
27bjornstad syndrome10.5
28pendred syndrome10.5
29auditory neuropathy10.5
30cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss10.5
31perilymphatic fistula10.5
32cardiomyopathy, dilated, 1j10.5
33renal tubular acidosis with progressive nerve deafness10.4
34renal dysplasia10.4
35wolfram syndrome10.4
36hypertension10.4
37labyrinthitis10.4
38deafness, x-linked 210.4
39jones syndrome10.4
40maternally inherited diabetes and deafness10.4
41deafness, autosomal dominant 4b10.4
42deafness, x-linked 110.4
43deafness-infertility syndrome10.4
44histiocytosis with joint contractures and sensorineural deafness10.4
45dystonia10.4
46microcephaly10.4
47aortitis10.4
48acoustic neuroma10.4
49arthritis10.4
50dilated cardiomyopathy10.4

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



Diseases related to sensorineural hearing loss

Symptoms for Sensorineural Hearing Loss

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48OMIM
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Clinical features from OMIM:

304400

Drugs & Therapeutics for Sensorineural Hearing Loss

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-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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23GTR
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Genetic tests related to Sensorineural Hearing Loss:

id Genetic test Affiliating Genes
1 Sensorineural Hearing Loss23

Anatomical Context for Sensorineural Hearing Loss

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34MalaCards
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MalaCards organs/tissues related to Sensorineural Hearing Loss:

34
Brain, Bone, Testes, Endothelial, Skin, Pituitary, Retina, T cells, Thyroid, Kidney, Cortex

Animal Models for Sensorineural Hearing Loss or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Sensorineural Hearing Loss:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00030128.7GJB3, GJB2, GJB6, SERPINB6
2MP:00107717.9POU3F4, WFS1, DLX5, GJB2, GJB3
3MP:00053777.4GJB2, GJB6, DLX5, COCH, STRC, POU3F4

Publications for Sensorineural Hearing Loss

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53PubMed
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Articles related to Sensorineural Hearing Loss:

(show top 50)    (show all 731)
idTitleAuthorsYear
1
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report. (23332734)
2013
2
What is the optimal workup for a child with bilateral sensorineural hearing loss? (23529877)
2013
3
Non-flat audiograms in sensorineural hearing loss and speech perception. (23778471)
2013
4
Autoimmune sensorineural hearing loss: the otology-rheumatology interface. (23436581)
2013
5
Late-onset sensorineural hearing loss due to congenital cytomegalovirus infection: could head injury be a trigger? (23025246)
2013
6
Comparison of intratympanic steroid injection, hyperbaric oxygen and combination therapy in refractory sudden sensorineural hearing loss. (24005170)
2013
7
Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and MAcniA"re's disease. (23560644)
2013
8
ATP6V1B1 mutations in distal renal tubular acidosis and sensorineural hearing loss: clinical and genetic spectrum of five families. (23923981)
2013
9
Characteristics of radiation-induced sensorineural hearing loss in head and neck cancer: a systematic review. (23280686)
2013
10
Quality of life in youth with severe to profound sensorineural hearing loss. (23657441)
2013
11
Risk analysis of unilateral severe-to-profound sensorineural hearing loss in children. (23701899)
2013
12
Systematic review and meta-analysis of the risk factors for sudden sensorineural hearing loss in adults. (22252719)
2012
13
In-flight speech intelligibility evaluation of a service member with sensorineural hearing loss: case report. (23025145)
2012
14
A comparison of phonological processing skills of children with mild to moderate sensorineural hearing loss and children with dyslexia. (22978204)
2012
15
Drug delivery to the inner ear: strategies and their therapeutic implications for sensorineural hearing loss. (22283653)
2012
16
Molecular investigation of pediatric portuguese patients with sensorineural hearing loss. (22567359)
2011
17
Management of acute idiopathic sensorineural hearing loss: a survey of UK ENT consultants. (22058587)
2011
18
Role of H.E.L.P.-apheresis in the treatment of sudden sensorineural hearing loss in a group of 230 patients. (22323851)
2011
19
Autosomal dominant progressive sensorineural hearing loss due to a novel mutation in the KCNQ4 gene. (21242547)
2011
20
Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course. (21389742)
2011
21
The design and screening of drugs to prevent acquired sensorineural hearing loss. (22646075)
2011
22
Brainstem auditory evoked potentials and electrocochleographic findings in patients with idiopathic sudden sensorineural hearing loss. (21777530)
2011
23
Late-onset sensorineural hearing loss due to asymptomatic congenital cytomegalovirus infection retrospectively diagnosed by polymerase chain reaction using preserved umbilical cord. (20837611)
2011
24
Idiopathic sudden sensorineural hearing loss: evolution in the presence of hypertension, diabetes mellitus and dyslipidemias. (20658017)
2010
25
Autosomal dominant optic neuropathy and sensorineural hearing loss associated with a novel mutation of WFS1. (20069065)
2010
26
Methylenetetrahydrofolate reductase C677T gene mutation as risk factor for sudden sensorineural hearing loss: association with plasma homocysteine, folate and cholesterol concentrations. (20492738)
2010
27
In reference to Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. (20927780)
2010
28
Screening for GJB2 and GJB6 gene mutations in patients from Campania region with sensorineural hearing loss. (20233142)
2010
29
Sensorineural hearing loss: an underdiagnosed complication of Kawasaki disease. (20859226)
2010
30
Asymmetric sudden sensorineural hearing loss: is all this testing necessary? (20828509)
2010
31
Cost analysis of asymmetric sensorineural hearing loss investigations. (20661936)
2010
32
Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]. (19705751)
2009
33
Auditory neuropathy: three cases among a group with sensorineural hearing loss. (19787161)
2009
34
A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. (18571542)
2008
35
Optic atrophy and sensorineural hearing loss in a family caused by an R445H OPA1 mutation. (16158427)
2005
36
Sensorineural hearing loss, striate palmoplantar hyperkeratosis, and knuckle pads in a patient with a novel connexin 26 (GJB2) mutation. (15635064)
2005
37
Congenital cataract, muscular hypotonia, developmental delay and sensorineural hearing loss associated with a defect in copper metabolism. (15902551)
2005
38
Sudden sensorineural hearing loss after rapid reduction of blood pressure in malignant hypertension. (14763578)
2004
39
Genetic linkage analysis of a novel syndrome comprising North Carolina-like macular dystrophy and progressive sensorineural hearing loss. (12812894)
2003
40
A child with Saethre-Chotzen syndrome, sensorineural hearing loss, and a TWIST mutation. (11772178)
2002
41
Fluctuating sensorineural hearing loss associated with enlarged vestibular aqueduct maps to 7q31, the region containing the Pendred gene. (10051166)
1999
42
A Moroccan family with autosomal recessive sensorineural hearing loss caused by a mutation in the gap junction protein gene connexin 26 (GJB2). (9507396)
1998
43
Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. (9638673)
1998
44
Progressive sensorineural hearing loss and a widened vestibular aqueduct in Pendred syndrome. (9604973)
1998
45
A new locus for non-syndromal, autosomal recessive, sensorineural hearing loss (DFNB16) maps to human chromosome 15q21-q22. (9429146)
1997
46
Sensorineural hearing loss and cerebellopontine angle lesions. Not always an acoustic neuroma--a pictorial essay. (9156313)
1997
47
An autosomal recessive nonsyndromic form of sensorineural hearing loss maps to 3p-DFNB6. (8593615)
1995
48
Sensorineural hearing loss and nail patella syndrome. (8198793)
1994
49
Middle ear disease in young children with sensorineural hearing loss. (8459744)
1993
50
Progressive optic atrophy and sensorineural hearing loss due to chronic glue sniffing. (6644323)
1983

Variations for Sensorineural Hearing Loss

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Expression for genes affiliated with Sensorineural Hearing Loss

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2BioGPS, 16Gene 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:
51PathCards, 61Thomson Reuters, 56Reactome, 31KEGG, 13EMD Millipore, 39NCBI BioSystems Database, 54QIAGEN
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Compounds for genes affiliated with Sensorineural Hearing Loss

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62Tocris Bioscience, 30IUPHAR, 25HMDB, 46Novoseek, 3BitterDB, 12DrugBank
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Compounds related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
110panx629.2GJB3, GJB6, GJB2
2gap 27629.2GJB6, GJB2, GJB3
3carbenoxolone disodium629.1GJB6, GJB2, GJB3
4scrambled 10panx629.1GJB6, GJB2, GJB3
5octanol30 2510.0GJB6, GJB2, GJB3
6flufenamic acid30 46 3 1212.0GJB3, GJB2, GJB6
7carbenoxolone46 30 1210.9GJB3, GJB2, GJB6
8ca2+308.7GJB6, GJB2, GJB3

GO Terms for genes affiliated with Sensorineural Hearing Loss

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17Gene Ontology
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Cellular components related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1connexon complexGO:0059229.0GJB3, GJB2, GJB6

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

idNameGO IDScoreTop Affiliating Genes
1cell communicationGO:0071549.4GJB3, GJB6
2sensory perception of soundGO:0076057.0GJB3, GJB2, GJB6, COCH, WFS1, POU3F4

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

idNameGO IDScoreTop Affiliating Genes
1gap junction channel activityGO:0052439.5GJB3, GJB2

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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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet