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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8Disease Ontology, 22GTR, 10DISEASES, 60UMLS, 44Novoseek, 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, T cells, Skin, Thyroid, Kidney, Retina, Cortex, Pituitary

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
Epidemiology of unilateral sensorineural hearing loss with universal newborn hearing screening. (23553416)
2014
2
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report. (23332734)
2013
3
Characteristics of radiation-induced sensorineural hearing loss in head and neck cancer: a systematic review. (23280686)
2013
4
Sensorineural hearing loss in OPA1-linked disorders. (23384603)
2013
5
Absence of SERPINB6A causes sensorineural hearing loss with multiple histopathologies in the mouse inner ear. (23669344)
2013
6
The significance of routine laboratory parameters in patients with sudden sensorineural hearing loss. (24427713)
2013
7
Labyrinthine Artery Detection in Patients with Idiopathic Sudden Sensorineural Hearing Loss by 7-T MRI. (24381016)
2013
8
Long-term functional outcome and satisfaction of patients with an active middle ear implant for sensorineural hearing loss compared to a matched population with conventional hearing aids. (24248731)
2013
9
A comparison of different murine models for cytomegalovirus-induced sensorineural hearing loss. (23616191)
2013
10
Relationship between CT findings and sensorineural hearing loss in chronic otitis media. (24387800)
2013
11
Sudden sensorineural hearing loss: early diagnosis improves outcome. (23972201)
2013
12
Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive andA Sensorineural Hearing Loss and a Group with Vestibular Schawannoma. (24303434)
2013
13
Intratympanic dexamethasone perfusion versus injection for treatment of refractory sudden sensorineural hearing loss. (22669272)
2013
14
A comparison of phonological processing skills of children with mild to moderate sensorineural hearing loss and children with dyslexia. (22978204)
2012
15
Diagnostic and prognostic utility of measuring tumor necrosis factor in the peripheral circulation of patients with immune-mediated sensorineural hearing loss. (23165380)
2012
16
Temporal modulation transfer functions measured from auditory-nerve responses following sensorineural hearing loss. (22366500)
2012
17
Prevalence of risk factors for sensorineural hearing loss in NICU newborns. (22545383)
2012
18
Celiac disease and sensorineural hearing loss in children. (22216922)
2012
19
Bilateral sensorineural hearing loss and cerebellar ataxia in the case of late stage Lyme disease. (23306581)
2012
20
Antivirals for idiopathic sudden sensorineural hearing loss. (22895957)
2012
21
Sudden sensorineural hearing loss: an otologic emergency. (23448732)
2012
22
A novel nonsense CDK5RAP2 mutation in a Somali child with primary microcephaly and sensorineural hearing loss. (22887808)
2012
23
Sensorineural Hearing Loss due to Air Bag Deployment. (22953102)
2012
24
Idiopathic sudden sensorineural hearing loss and comorbid benign paroxysmal positional vertigo: some pathogenic additional considerations. (22525218)
2012
25
Brainstem auditory evoked potentials and electrocochleographic findings in patients with idiopathic sudden sensorineural hearing loss. (21777530)
2011
26
Facial nerve schwannoma involving middle cranial fossa: when the unilateral sensorineural hearing loss guide to the correct diagnosis. (22754837)
2011
27
Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis. (21606048)
2011
28
Low prevalence of DFNB1 (connexin 26) mutations in British Pakistani children with non-syndromic sensorineural hearing loss. (21586435)
2011
29
Hyperbaric oxygen therapy in idiopathic sudden sensorineural hearing loss (ISSNHL) in association with combined treatment. (21510273)
2011
30
Lactic acidosis, hypotension, and sensorineural hearing loss following intentional metformin overdose. (22424544)
2011
31
Progressive sensorineural hearing loss and normal vestibular function in a Dutch DFNB7/11 family with a novel mutation in TMC1. (21252500)
2011
32
Two new cases with microdeletion of 17q23.2 suggest presence of a candidate gene for sensorineural hearing loss within this region. (22052739)
2011
33
Screening for GJB2 and GJB6 gene mutations in patients from Campania region with sensorineural hearing loss. (20233142)
2010
34
Does intracochlear brain-derived nerve growth factor improve auditory brainstem click thresholds in sensorineural hearing loss? (20470666)
2010
35
Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. (20422698)
2010
36
Sensorineural hearing loss in a pediatric population: association of congenital cytomegalovirus infection with intracranial abnormalities. (20956747)
2010
37
Hypertensive retinopathy and sensorineural hearing loss. (23120650)
2009
38
The occurrence of sensorineural hearing loss in a patient with myeloperoxidase-anti-neutrophil cytoplasmic antibody-related microscopic polyangiitis. (16969635)
2007
39
Sensorineural hearing loss in a case of familial hemophagocytic lymphohistiocytosis. (16358309)
2007
40
A case of relapsing polychondritis with bilateral sensorineural hearing loss and perforation of the nasal septum at the onset. (17437171)
2007
41
Etanercept, a tumour necrosis factor alpha receptor antagonist, and methotrexate in acute sensorineural hearing loss. (17040588)
2006
42
Sensorineural hearing loss, striate palmoplantar hyperkeratosis, and knuckle pads in a patient with a novel connexin 26 (GJB2) mutation. (15635064)
2005
43
Mutation in the transcriptional coactivator EYA4 causes dilated cardiomyopathy and sensorineural hearing loss. (15735644)
2005
44
Childhood-onset osteoarthritis, tall stature, and sensorineural hearing loss associated with Arg75-Cys mutation in procollagen type II gene (COL2A1). (15593085)
2004
45
Can low frequency sound stimulation during posturography help diagnosing possible perilymphatic fistula in patients with sensorineural hearing loss and/or vertigo? (12883814)
2004
46
Nerve growth factor and brain-derived neurotrophic factor: a possible etiopathogenic role in sensorineural hearing loss. Preliminary data]. (14587217)
2003
47
Role of transtympanic endoscopy of the middle ear in the diagnosis of perilymphatic fistula in patients with sensorineural hearing loss or vertigo. (12417768)
2002
48
Dilated cardiomyopathy and sensorineural hearing loss: a heritable syndrome that maps to 6q23-24. (10769282)
2000
49
Autosomal-dominant, prelingual, nonprogressive sensorineural hearing loss: localization of the gene (DFNA8) to chromosome 11q by linkage in an Austrian family. (9763681)
1998
50
Congenital cytomegalovirus infection: a cause of sensorineural hearing loss. (2829749)
1987

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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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
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  • 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