MCID: SNS001
MIFTS: 48

Sensorineural Hearing Loss malady

Ear diseases category

Summaries for Sensorineural Hearing Loss

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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 based summary: Sensorineural Hearing Loss, also known as sensory hearing loss, is related to nonsyndromic hearing loss and deafness, mitochondrial and dfnb1. 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.

Aliases & Classifications for Sensorineural Hearing Loss

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Sources:
9Disease Ontology, 11DISEASES, 43Novoseek, 22GTR, 60UMLS, 55SNOMED-CT, 33MeSH, 38NCIt, 27ICD9CM
See all sources

Sensorineural Hearing Loss, Aliases & Descriptions:

Name: Sensorineural Hearing Loss 9 11 60
Sensory Hearing Loss 9 43 60
Hearing Loss Sensorineural 43 22
Hearing Loss, High-Frequency 60
High-Frequency Hearing Loss 9
Hearing Loss High-Frequency 43
High Frequency Hearing Loss 9
High Frequency Deafness 9
 
Perceptive Hearing Loss 9
Deafness Sensorineural 43
Sensorineural Deafness 9
Hearing Loss, Central 60
Central Hearing Loss 9
Perceptive Deafness 9
Deafness 9


Classifications:

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


Related Diseases for Sensorineural Hearing Loss

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

(show top 50)    (show all 1385)
idRelated DiseaseScoreTop Affiliating Genes
1nonsyndromic hearing loss and deafness, mitochondrial30.5MT-TS1, MT-RNR1
2dfnb130.4GJB6, GJB2
3bart-pumphrey syndrome30.3GJB6, GJB2
4ectodermal dysplasia30.2GJB2
5dfna 3 nonsyndromic hearing loss and deafness30.2GJB6, GJB2
6dfnb 1 nonsyndromic hearing loss and deafness30.1GJB6, GJB2
7mitochondrial disorders30.0MT-TL1
8deafness, autosomal recessive 4, with enlarged vestibular aqueduct29.9GJB3, GJB2, GJB6
9skin disease29.7GJB3, GJB2, GJB6
10erythrokeratodermia variabilis et progressiva29.6GJB3, GJB2
11nonsyndromic deafness28.9POU3F4, STRC, COCH, GJB6, GJB2, GJB3
12congenital cytomegalovirus10.7
13cytomegalovirus infection10.6
14renal tubular acidosis10.6
15siderosis10.6
16otitis media10.6
17superficial siderosis10.6
18maternally inherited diabetes and deafness10.6
19otosclerosis10.5
20meningitis10.5
21neuropathy10.5
22ataxia10.5
23renal tubular acidosis with deafness10.5
24pendred syndrome10.5
25auditory neuropathy10.5
26cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss10.5
27superficial siderosis of the central nervous system10.5
28keratoderma, palmoplantar, with deafness10.5
29keratitis-ichthyosis-deafness syndrome10.5
30bjornstad syndrome10.5
31deafness, x-linked 210.5
32split-hand/foot malformation 1 with sensorineural hearing loss10.5
33lyme disease10.5
34labyrinthitis10.5
35jones syndrome10.5
36perilymphatic fistula10.5
37sensorineural deafness with dilated cardiomyopathy10.5
38mutism10.5
39hypoparathyroidism10.4
40wolfram syndrome10.4
41ectodermal dysplasia, sensorineural hearing loss, and distinctive facial features10.4
42nonsyndromic hereditary sensorineural hearing loss10.4
43x-linked non-syndromic sensorineural deafness type dfn10.4
44autosomal dominant non-syndromic sensorineural deafness type dfna10.4
45dystonia10.4
46deafness, congenital with inner ear agenesis, microtia, and microdontia10.4
47cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia10.4
48alport syndrome, autosomal dominant10.4
49migraine10.4
50cerebellar ataxia10.4

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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Drugs & Therapeutics for Sensorineural Hearing Loss

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Drug clinical trials:

Search ClinicalTrials for Sensorineural Hearing Loss

Search NIH Clinical Center for Sensorineural Hearing Loss

Genetic Tests for Sensorineural Hearing Loss

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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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MalaCards organs/tissues related to Sensorineural Hearing Loss:

31
Brain, Bone, Testes, Endothelial, Neutrophil, Cortex, Retina, Kidney, Thyroid, Skin, Myeloid, T cells, Pituitary

Animal Models for Sensorineural Hearing Loss or affiliated genes

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MGI Mouse Phenotypes related to Sensorineural Hearing Loss:

35
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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Articles related to Sensorineural Hearing Loss:

(show top 50)    (show all 785)
idTitleAuthorsYear
1
Risk of sudden sensorineural hearing loss in patients with common preexisting sensorineural hearing impairment: a population-based study in taiwan. (25815507)
2015
2
Pulse steroid therapy in idiopathic sudden sensorineural hearing loss: A randomized controlled clinical trial. (25782020)
2015
3
Intratympanic steroid injection as a first-line therapy in uremia patients with sudden sensorineural hearing loss. (25783847)
2015
4
Altered autonomic regulation as a cardiovascular risk marker for patients with sudden sensorineural hearing loss. (25299829)
2014
5
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report. (23332734)
2013
6
What is the optimal workup for a child with bilateral sensorineural hearing loss? (23529877)
2013
7
Non-flat audiograms in sensorineural hearing loss and speech perception. (23778471)
2013
8
Autoimmune sensorineural hearing loss: the otology-rheumatology interface. (23436581)
2013
9
Late-onset sensorineural hearing loss due to congenital cytomegalovirus infection: could head injury be a trigger? (23025246)
2013
10
Comparison of intratympanic steroid injection, hyperbaric oxygen and combination therapy in refractory sudden sensorineural hearing loss. (24005170)
2013
11
Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and MAcniA"re's disease. (23560644)
2013
12
ATP6V1B1 mutations in distal renal tubular acidosis and sensorineural hearing loss: clinical and genetic spectrum of five families. (23923981)
2013
13
Characteristics of radiation-induced sensorineural hearing loss in head and neck cancer: a systematic review. (23280686)
2013
14
Quality of life in youth with severe to profound sensorineural hearing loss. (23657441)
2013
15
Systematic review and meta-analysis of the risk factors for sudden sensorineural hearing loss in adults. (22252719)
2012
16
In-flight speech intelligibility evaluation of a service member with sensorineural hearing loss: case report. (23025145)
2012
17
A comparison of phonological processing skills of children with mild to moderate sensorineural hearing loss and children with dyslexia. (22978204)
2012
18
Electrocochleographic findings in recurrent idiopathic sudden sensorineural hearing loss. (22691116)
2012
19
Molecular investigation of pediatric portuguese patients with sensorineural hearing loss. (22567359)
2011
20
Management of acute idiopathic sensorineural hearing loss: a survey of UK ENT consultants. (22058587)
2011
21
Role of H.E.L.P.-apheresis in the treatment of sudden sensorineural hearing loss in a group of 230 patients. (22323851)
2011
22
Autosomal dominant progressive sensorineural hearing loss due to a novel mutation in the KCNQ4 gene. (21242547)
2011
23
Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course. (21389742)
2011
24
The design and screening of drugs to prevent acquired sensorineural hearing loss. (22646075)
2011
25
Brainstem auditory evoked potentials and electrocochleographic findings in patients with idiopathic sudden sensorineural hearing loss. (21777530)
2011
26
Idiopathic sudden sensorineural hearing loss: evolution in the presence of hypertension, diabetes mellitus and dyslipidemias. (20658017)
2010
27
Autosomal dominant optic neuropathy and sensorineural hearing loss associated with a novel mutation of WFS1. (20069065)
2010
28
Methylenetetrahydrofolate reductase C677T gene mutation as risk factor for sudden sensorineural hearing loss: association with plasma homocysteine, folate and cholesterol concentrations. (20492738)
2010
29
In reference to Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. (20927780)
2010
30
Screening for GJB2 and GJB6 gene mutations in patients from Campania region with sensorineural hearing loss. (20233142)
2010
31
Sensorineural hearing loss: an underdiagnosed complication of Kawasaki disease. (20859226)
2010
32
Asymmetric sudden sensorineural hearing loss: is all this testing necessary? (20828509)
2010
33
Cost analysis of asymmetric sensorineural hearing loss investigations. (20661936)
2010
34
Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]. (19705751)
2009
35
Auditory neuropathy: three cases among a group with sensorineural hearing loss. (19787161)
2009
36
A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. (18571542)
2008
37
Optic atrophy and sensorineural hearing loss in a family caused by an R445H OPA1 mutation. (16158427)
2005
38
Sensorineural hearing loss, striate palmoplantar hyperkeratosis, and knuckle pads in a patient with a novel connexin 26 (GJB2) mutation. (15635064)
2005
39
Congenital cataract, muscular hypotonia, developmental delay and sensorineural hearing loss associated with a defect in copper metabolism. (15902551)
2005
40
Sudden sensorineural hearing loss after rapid reduction of blood pressure in malignant hypertension. (14763578)
2004
41
Genetic linkage analysis of a novel syndrome comprising North Carolina-like macular dystrophy and progressive sensorineural hearing loss. (12812894)
2003
42
Fluctuating sensorineural hearing loss associated with enlarged vestibular aqueduct maps to 7q31, the region containing the Pendred gene. (10051166)
1999
43
A Moroccan family with autosomal recessive sensorineural hearing loss caused by a mutation in the gap junction protein gene connexin 26 (GJB2). (9507396)
1998
44
Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. (9638673)
1998
45
Progressive sensorineural hearing loss and a widened vestibular aqueduct in Pendred syndrome. (9604973)
1998
46
A new locus for non-syndromal, autosomal recessive, sensorineural hearing loss (DFNB16) maps to human chromosome 15q21-q22. (9429146)
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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Search GEO for disease gene expression data for Sensorineural Hearing Loss.

Pathways for genes affiliated with Sensorineural Hearing Loss

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Compounds for genes affiliated with Sensorineural Hearing Loss

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

idCompoundScoreTop Affiliating Genes
110panx599.2GJB3, GJB6, GJB2
2gap 27599.2GJB6, GJB2, GJB3
3carbenoxolone disodium599.1GJB6, GJB2, GJB3
4scrambled 10panx599.1GJB6, GJB2, GJB3
5octanol28 2410.0GJB6, GJB2, GJB3
6flufenamic acid28 43 2 1212.0GJB3, GJB2, GJB6
7carbenoxolone43 28 1210.9GJB3, GJB2, GJB6
8ca2+288.7GJB6, GJB2, GJB3

GO Terms for genes affiliated with Sensorineural Hearing Loss

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

idNameGO IDScoreTop Affiliating Genes
1connexon complexGO:00059229.0GJB3, GJB2, GJB6

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

idNameGO IDScoreTop Affiliating Genes
1cell communicationGO:00071549.4GJB3, GJB6
2sensory perception of soundGO:00076057.0GJB3, GJB2, GJB6, COCH, WFS1, POU3F4

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

idNameGO IDScoreTop Affiliating Genes
1gap junction channel activityGO:00052439.5GJB3, GJB2

Products for genes affiliated with Sensorineural Hearing Loss

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Sources for Sensorineural Hearing Loss

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet