MCID: SNS001
MIFTS: 57

Sensorineural Hearing Loss malady

Ear diseases category
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Summaries for Sensorineural Hearing Loss

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Wikipedia:65 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 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:46 304400

Aliases & Classifications for Sensorineural Hearing Loss

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Sources:
8Disease Ontology, 22GTR, 10DISEASES, 44Novoseek, 62UMLS, 39NCIt, 57SNOMED-CT, 27ICD9CM, 34MeSH, 46OMIM
See all sources

Sensorineural Hearing Loss, Aliases & Descriptions:

Name: Sensorineural Hearing Loss 8 22 10 62
Sensory Hearing Loss 8 44
Hearing Loss, High-Frequency 62
High-Frequency Hearing Loss 8
Hearing Loss High-Frequency 44
High Frequency Hearing Loss 8
Hearing Loss Sensorineural 44
Perceptive Hearing Loss 8
 
High Frequency Deafness 8
Deafness Sensorineural 44
Sensorineural Deafness 8
Hearing Loss, Central 62
Complete Hearing Loss 62
Central Hearing Loss 8
Perceptive Deafness 8
Deafness 8


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/GeneDecks gene sharing:

(show top 50)    (show all 1425)
idRelated DiseaseScoreTop Affiliating Genes
1bart-pumphrey syndrome30.9GJB2, GJB6
2enlarged vestibular aqueduct30.7GJB6, GJB2, GJB3
3dfnb130.5GJB2, GJB6
4nonsyndromic hearing loss and deafness, mitochondrial30.4MT-RNR1, MT-TS1
5ectodermal dysplasia30.2GJB2
6clouston syndrome30.1GJB6, GJB2, GJB3
7dfna 3 nonsyndromic hearing loss and deafness30.1GJB6, GJB2
8mitochondrial disorders30.0MT-TL1
9dfnb 1 nonsyndromic hearing loss and deafness30.0GJB6, GJB2
10skin disease29.8GJB3, GJB6, GJB2
11nonsyndromic deafness29.0GJB3, GJB2, POU3F4, STRC, COCH, GJB6
12congenital cytomegalovirus10.7
13cytomegalovirus infection10.6
14renal tubular acidosis10.6
15siderosis10.6
16otitis media10.6
17superficial siderosis10.6
18hypoparathyroidism10.6
19albinism ocular late onset sensorineural deafness10.6
20otosclerosis10.6
21bjornstad syndrome10.5
22meningitis10.5
23optic atrophy10.5
24jones syndrome10.5
25superficial siderosis of the central nervous system10.5
26ataxia10.5
27arthrogryposis-like hand anomaly and sensorineural deafness10.5
28renal tubular acidosis with progressive nerve deafness10.5
29lyme disease10.5
30labyrinthitis10.5
31neuropathy10.5
32pendred syndrome10.5
33cleft palate, midfacial hypoplasia, triangular facies, and sensorineural hearing loss10.5
34perilymphatic fistula10.5
35cardiomyopathy, dilated, 1j10.5
36renal dysplasia10.5
37hypertension10.4
38wolfram syndrome10.4
39auditory neuropathy10.4
40deafness, x-linked 210.4
41deafness, autosomal dominant 3b10.4
42deafness, x-linked 410.4
43histiocytosis with joint contractures and sensorineural deafness10.4
44deafness dystonia syndrome10.4
45dystonia10.4
46otospondylomegaepiphyseal dysplasia10.4
47arthritis10.4
48dilated cardiomyopathy10.4
49aortitis10.4
50acoustic neuroma10.4

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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Clinical features from OMIM:

304400

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:

32
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:

36
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 761)
idTitleAuthorsYear
1
Altered autonomic regulation as a cardiovascular risk marker for patients with sudden sensorineural hearing loss. (25299829)
2014
2
Prelingual sensorineural hearing loss and infants at risk: Western Sicily report. (23332734)
2013
3
What is the optimal workup for a child with bilateral sensorineural hearing loss? (23529877)
2013
4
Non-flat audiograms in sensorineural hearing loss and speech perception. (23778471)
2013
5
Autoimmune sensorineural hearing loss: the otology-rheumatology interface. (23436581)
2013
6
Late-onset sensorineural hearing loss due to congenital cytomegalovirus infection: could head injury be a trigger? (23025246)
2013
7
Comparison of intratympanic steroid injection, hyperbaric oxygen and combination therapy in refractory sudden sensorineural hearing loss. (24005170)
2013
8
Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and MAcniA"re's disease. (23560644)
2013
9
ATP6V1B1 mutations in distal renal tubular acidosis and sensorineural hearing loss: clinical and genetic spectrum of five families. (23923981)
2013
10
Characteristics of radiation-induced sensorineural hearing loss in head and neck cancer: a systematic review. (23280686)
2013
11
Quality of life in youth with severe to profound sensorineural hearing loss. (23657441)
2013
12
Risk analysis of unilateral severe-to-profound sensorineural hearing loss in children. (23701899)
2013
13
Systematic review and meta-analysis of the risk factors for sudden sensorineural hearing loss in adults. (22252719)
2012
14
In-flight speech intelligibility evaluation of a service member with sensorineural hearing loss: case report. (23025145)
2012
15
A comparison of phonological processing skills of children with mild to moderate sensorineural hearing loss and children with dyslexia. (22978204)
2012
16
Electrocochleographic findings in recurrent idiopathic sudden sensorineural hearing loss. (22691116)
2012
17
Drug delivery to the inner ear: strategies and their therapeutic implications for sensorineural hearing loss. (22283653)
2012
18
Molecular investigation of pediatric portuguese patients with sensorineural hearing loss. (22567359)
2011
19
Management of acute idiopathic sensorineural hearing loss: a survey of UK ENT consultants. (22058587)
2011
20
Role of H.E.L.P.-apheresis in the treatment of sudden sensorineural hearing loss in a group of 230 patients. (22323851)
2011
21
Autosomal dominant progressive sensorineural hearing loss due to a novel mutation in the KCNQ4 gene. (21242547)
2011
22
Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course. (21389742)
2011
23
The design and screening of drugs to prevent acquired sensorineural hearing loss. (22646075)
2011
24
Brainstem auditory evoked potentials and electrocochleographic findings in patients with idiopathic sudden sensorineural hearing loss. (21777530)
2011
25
Idiopathic sudden sensorineural hearing loss: evolution in the presence of hypertension, diabetes mellitus and dyslipidemias. (20658017)
2010
26
Autosomal dominant optic neuropathy and sensorineural hearing loss associated with a novel mutation of WFS1. (20069065)
2010
27
Methylenetetrahydrofolate reductase C677T gene mutation as risk factor for sudden sensorineural hearing loss: association with plasma homocysteine, folate and cholesterol concentrations. (20492738)
2010
28
In reference to Systematic review of the evidence for the etiology of adult sudden sensorineural hearing loss. (20927780)
2010
29
Screening for GJB2 and GJB6 gene mutations in patients from Campania region with sensorineural hearing loss. (20233142)
2010
30
Sensorineural hearing loss: an underdiagnosed complication of Kawasaki disease. (20859226)
2010
31
Asymmetric sudden sensorineural hearing loss: is all this testing necessary? (20828509)
2010
32
Cost analysis of asymmetric sensorineural hearing loss investigations. (20661936)
2010
33
Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia]. (19705751)
2009
34
Auditory neuropathy: three cases among a group with sensorineural hearing loss. (19787161)
2009
35
A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. (18571542)
2008
36
Optic atrophy and sensorineural hearing loss in a family caused by an R445H OPA1 mutation. (16158427)
2005
37
Sensorineural hearing loss, striate palmoplantar hyperkeratosis, and knuckle pads in a patient with a novel connexin 26 (GJB2) mutation. (15635064)
2005
38
Congenital cataract, muscular hypotonia, developmental delay and sensorineural hearing loss associated with a defect in copper metabolism. (15902551)
2005
39
Sudden sensorineural hearing loss after rapid reduction of blood pressure in malignant hypertension. (14763578)
2004
40
Genetic linkage analysis of a novel syndrome comprising North Carolina-like macular dystrophy and progressive sensorineural hearing loss. (12812894)
2003
41
A child with Saethre-Chotzen syndrome, sensorineural hearing loss, and a TWIST mutation. (11772178)
2002
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
Sensorineural hearing loss and cerebellopontine angle lesions. Not always an acoustic neuroma--a pictorial essay. (9156313)
1997
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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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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Compounds for genes affiliated with Sensorineural Hearing Loss

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Compounds related to Sensorineural Hearing Loss according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
110panx619.2GJB3, GJB6, GJB2
2gap 27619.2GJB6, GJB2, GJB3
3carbenoxolone disodium619.1GJB6, GJB2, GJB3
4scrambled 10panx619.1GJB6, GJB2, GJB3
5octanol28 2410.0GJB6, GJB2, GJB3
6flufenamic acid28 44 2 1112.0GJB3, GJB2, GJB6
7carbenoxolone44 28 1110.9GJB3, 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/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
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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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet