MCID: ADT004
MIFTS: 36

Auditory Neuropathy malady

Categories: Genetic diseases, Rare diseases, Neuronal diseases

Aliases & Classifications for Auditory Neuropathy

About this section

Aliases & Descriptions for Auditory Neuropathy:

Name: Auditory Neuropathy 46 25 66
Auditory Neuropathy Spectrum Disorder 66
 
Auditory Dys-Synchrony 46

Classifications:



Summaries for Auditory Neuropathy

About this section
NIH Rare Diseases:46 Auditory neuropathy is a rare condition that can affect a person's ability to hear. although sounds enter the inner ear normally, signals from the inner ear to the brain are not transmitted properly. as a result, the condition may be associated with mild to severe hearing loss and poor speech-perception abilities (difficulty understanding speech clearly). auditory neuropathy can be associated with other neurological disorders such as friedrich ataxia, stevens-johnson syndrome, ehlers-danlos syndrome, and charcot-marie-tooth syndrome. the exact underlying cause of auditory neuropathy is not completely understood; however, researchers have proposed many possible explanations including damage to the hair cells of the inner ears, faulty connections between the hair cells and the auditory nerve (the nerve connecting the inner ear to the brain), damage to the auditory nerve, and/or a combination of these abnormalities. in many cases, auditory neuropathy occurs sporadically in people with no family history of the condition; however, the condition does run in some families suggesting that genetic factors may play a role in some cases. treatment varies based on the severity of the condition but aims to improve hearing (i.e. hearing aids and cochlear implants) and communication skills. last updated: 12/28/2015

MalaCards based summary: Auditory Neuropathy, also known as auditory neuropathy spectrum disorder, is related to auditory neuropathy, autosomal dominant, 1 and deafness, autosomal recessive 9, and has symptoms including diplacusis and dysacusis. An important gene associated with Auditory Neuropathy is DIAPH3 (Diaphanous Related Formin 3). Affiliated tissues include brain, testes and cortex, and related mouse phenotypes are hearing/vestibular/ear and nervous system.

Wikipedia:69 Auditory neuropathy (AN) is a variety of hearing loss in which the outer hair cells within the cochlea... more...

Related Diseases for Auditory Neuropathy

About this section

Diseases in the Auditory Neuropathy family:

Auditory Neuropathy, Autosomal Dominant, 1

Diseases related to Auditory Neuropathy via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 52)
idRelated DiseaseScoreTop Affiliating Genes
1auditory neuropathy, autosomal dominant, 112.3
2deafness, autosomal recessive 911.6
3optic atrophy 711.4
4deafness, x-linked 511.4
5deafness, autosomal recessive 5911.3
6neuropathy10.9
7trichothiodystrophy 5, nonphotosensitive10.6AIFM1, AUNX1
8nonsyndromic hearing loss and deafness, mitochondrial10.6GJB2, GJB6
9myocardial infarction 210.6GJB2, GJB6
10noonan syndrome with multiple lentigines10.5GJB2, GJB6
11deafness, autosomal dominant 3a10.5GJB2, GJB6
12pseudohermaphrodism anorectal anomalies10.5GJB2, GJB6
13punctate palmoplantar keratoderma type 210.4GJB2, GJB6
14continuous spike-wave during slow sleep syndrome10.4GJB2, GJB6
15self-healing papular mucinosis10.4DIAPH3, GJB2, GJB6
16troyer syndrome10.4GJB2, GJB6
17dementia, familial british10.3GJB2, GJB6
18endometritis10.3GJB2, GJB6, OTOF
19urethral false passage10.3OPA1, TMEM126A
20ectodermal dysplasia 2, clouston type10.2GJB2, GJB6
21diabetes mellitus, 6q24-related transient neonatal10.2DFNB59, GJB2, MT-RNR1
22sensorineural hearing loss10.1
23thumb deformity, alopecia, pigmentation anomaly10.1GJB2, GJB6, MT-RNR1
24sublingual gland cancer10.1GJB2, GJB6, MT-RNR1
25nonsyndromic hearing loss and deafness, autosomal recessive10.1GJB2, GJB6, MT-RNR1
26dihydrolipoamide dehydrogenase deficiency10.1GJB2, GJB6, MT-RNR1
27hyperimmunoglobulin syndrome10.1GJB2, GJB6, MT-RNR1
28erythrokeratodermia variabilis et progressiva10.0GJB2, GJB6
29localized lichen myxedematosus with mixed features of different subtypes9.9DFNB59, GJB2, GJB6, OTOF, SLITRK6
30deafness and myopia9.9
31brown-vialetto-van laere syndrome9.8
32neuronitis9.8
33pyelitis9.7DFNB59, GJB2, GJB6, MT-RNR1, OTOF
34biotinidase deficiency9.7
35friedreich ataxia9.7
36charcot-marie-tooth disease9.7
37chikungunya9.7
38leukodystrophy9.7
39tooth disease9.7
40cervicitis9.7
41astrocytoma9.7
42agnosia9.7
43myopathy9.7
44dyslexia9.7
45pilocytic astrocytoma9.7
46waardenburg's syndrome9.7
47mitochondrial disorders9.7
48ataxia9.7
49cytomegalovirus infection9.7
50headache9.7

Graphical network of the top 20 diseases related to Auditory Neuropathy:



Diseases related to auditory neuropathy

Symptoms for Auditory Neuropathy

About this section

UMLS symptoms related to Auditory Neuropathy:


diplacusis, dysacusis

Drugs & Therapeutics for Auditory Neuropathy

About this section

Drugs for Auditory Neuropathy (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
EthanolPhase 3197164-17-5702
Synonyms:
(C6-C9)Alkyl alcohol
02483_FLUKA
02851_FLUKA
02853_FLUKA
02854_FLUKA
02855_FLUKA
02856_FLUKA
02856_SIAL
02857_FLUKA
02857_SIAL
02858_FLUKA
02858_SIAL
02860_FLUKA
02865_FLUKA
02865_SIAL
02870_FLUKA
02870_SIAL
02875_FLUKA
02877_FLUKA
02878_FLUKA
02882_FLUKA
02882_SIAL
02883_FLUKA
02884_FLUKA
02890_FLUKA
02890_SIAL
02891_FLUKA
02891_SIAL
1-Hydroxyethane
100C.NPA
121182-78-3
187380_ALDRICH
187380_SIAL
24102_RIEDEL
24102_SIAL
24103_RIEDEL
24103_SIAL
24105_RIEDEL
24105_SIAL
24106_RIEDEL
24106_SIAL
24194_RIEDEL
24194_SIAL
245119_ALDRICH
245119_SIAL
270741_ALDRICH
270741_SIAL
277649_ALDRICH
277649_SIAL
2858_SIGMA
29221_FLUKA
32205_RIEDEL
32205_SIAL
32221_RIEDEL
32221_SIAL
32294_RIEDEL
32294_SIAL
34870_SIAL
34963_RIEDEL
39278_FLUKA
40210_ALDRICH
40210_RIEDEL
41322_FLUKA
458600_ALDRICH
458600_SIAL
459828_ALDRICH
459828_SIAL
459836_ALDRICH
459836_SIAL
459844_SIAL
48075_SUPELCO
493511_SIAL
493538_ALDRICH
493538_SIAL
493546_ALDRICH
493546_SIAL
64-17-5
676829_SIAL
68475-56-9
71076-86-3
71329-38-9
8000-16-6
8024-45-1
AC1L19TW
AC1Q31MM
AHD 2000
AI3-01706
ALCOHOL 5% IN D5-W
Absolute Alcohol
Absolute Ethanol
Absolute alcohol
Absolute ethanol
Absolute ethyl alcohol
Aethanol
Aethanol [German]
Aethylalkohol
Alcare Hand Degermer
Alcohol
Alcohol (USP)
Alcohol (ethyl)
Alcohol Anhydrous
Alcohol [USP]
Alcohol anhydrous
Alcohol dehydrated
Alcohol denatured
Alcohol etílico
Alcohol, Absolute
Alcohol, Dehydrated
Alcohol, Diluted
Alcohol, Grain
Alcohol, anhydrous
Alcohol, dehydrated
Alcohol, denatured
Alcohol, diluted
Alcohol, ethyl
Alcohols
Alcohols, C1-3
Alcohols, C30
Alcohols, C6-9
Alcool Ethylique
Alcool Etilico
Alcool ethylique
Alcool etilico
Alcool éthylique
Algrain
Alkohol
Alkohol [German]
Alkoholu Etylowego
Alkoholu etylowego
Aminoethanol
Anhydrol
Anhydrol PM 4085
Anhydrous alcohol
Anhydrous ethanol
Beta-Aminoethanol
Beta-Aminoethyl Alcohol
Beta-Ethanolamine
Beta-Hydroxyethylamine
C00469
C2H5OH
C2H6O
CCRIS 945
CDA 19
CDA 19-200
CHEBI:16236
CHEMBL545
CID702
Caswell No. 426
Caswell No. 430
Colamine
Cologne Spirit
Cologne spirit
Cologne spirits
 
D000431
D00068
DB00898
Dehydrated Ethanol
Dehydrated alcohol
Dehydrated ethanol
Denatured Alcohol
Denatured Alcohol Cd-10
Denatured Alcohol Cd-5
Denatured Alcohol Cd-5a
Denatured Alcohol Sd-1
Denatured Alcohol Sd-13a
Denatured Alcohol Sd-17
Denatured Alcohol Sd-23a
Denatured Alcohol Sd-28
Denatured Alcohol Sd-30
Denatured Alcohol Sd-39b
Denatured Alcohol Sd-39c
Denatured Alcohol Sd-3a
Denatured Alcohol Sd-40m
Denatured Ethanol
Denatured alcohol
Denatured ethanol
Desinfektol EL
Diluted Alcohol
Distilled spirits
E2385_SIGMA
E7023_ALDRICH
E7023_SIAL
E7148_ALDRICH
E7148_SIAL
E7517_SIGMA
EINECS 200-578-6
EINECS 270-649-4
EOH
EOX
ETA
Envision Conditioner Pdd 9020
Esumiru WK 88
EtOH
Etanolo
Etanolo [Italian]
Ethanol (9CI)
Ethanol 200 Proof
Ethanol 200 proof
Ethanol Absolute
Ethanol Absolute Bp
Ethanol Anhydrous
Ethanol Extra Pure
Ethanol Vapor
Ethanol [JAN]
Ethanol solution
Ethanol, Silent Spirit
Ethanol, undenatured
Ethanolum anhydricum
Ethicap
Ethyl Alcohol
Ethyl Alcohol & Water, 10%
Ethyl Alcohol & Water, 20%
Ethyl Alcohol & Water, 30%
Ethyl Alcohol & Water, 40%
Ethyl Alcohol & Water, 5%
Ethyl Alcohol & Water, 50%
Ethyl Alcohol & Water, 60%
Ethyl Alcohol & Water, 70%
Ethyl Alcohol & Water, 80%
Ethyl Alcohol & Water, 95%
Ethyl Alcohol & Water, 96%
Ethyl Alcohol Anhydrous
Ethyl Alcohol, Anhydrous
Ethyl Alcohol, Denatured
Ethyl Hydrate
Ethyl Hydroxide
Ethyl alc
Ethyl alcohol
Ethyl alcohol anhydrous
Ethyl alcohol in alcoholic beverages
Ethyl alcohol usp
Ethyl hydrate
Ethyl hydroxide
Ethylalcohol
Ethylalcohol [Dutch]
Ethylol
Ethylolamine
Ethyloxy Group
Etylowy alkohol
FEMA No. 2419
FEMA Number 2419
Fermentation alcohol
Glycinol
Grain alcohol
HSDB 531
HSDB 82
HYDROXYETHYL GROUP
Hinetoless
Hydroxyethane
I14-12648
IMS 99
Infinity Pure
Jaysol
Jaysol S
LS-1539
LTBB002977
Lux
Methylated Spirit Mineralised
Methylated spirit
Methylcarbinol
MolPort-001-785-844
Molasses alcohol
NCGC00091458-01
NCI-C03134
NSC 85228
NSC85228
Oxydimethylene Group
Potato alcohol
Punctilious ethyl alcohol
Pyro
QMHAIh@
Reagent Alcohol
Ru-Tuss Expectorant
SDA 3A
SDA 40-2
SDM No. 37
SY Fresh M
Sekundasprit
Silent spirit
Spirit
Spirits of wine
Spiritus vini
Spirt
Synasol
Tecsol
Tecsol C
Thanol
Thiofaco M-50
UNII-3K9958V90M
USAF EK-1597
Undenatured Ethanol
WLN: Q2
absolute alcohol
alcohol
alcohol etilico
bmse000297
etanol
ethanol
ethyl alcohol
grain alcohol
nchem.651-comp3c
nchembio.552-comp10
nchembio.94-comp20
spiritus vini
Äthanol
Äthylalkohol
éthanol
2
MentholPhase 221842216-51-516666
Synonyms:
(−
()-Menthol
(+)-Neo-menthol
(+)-p-Menthan-3-ol
(+-)-(1R*,3R*,4S*)-Menthol
(+-)-Menthol
(+/-)-Menthol
(+/-)-p-Menthan-3-ol
(-)-(1R,3R,4S)-Menthol
(-)-Menthyl alcohol
(-)-menthol
(-)-p-Menthan-3-ol
(-)-trans-p-Menthan-cis-ol
(1R)-(-)-Menthol
(1R,2S,5R)-(-)-menthol
(1R,2S,5R)-Menthol
(1R,3R,4S)-(-)-MENTHOL
(1R,3R,4S)-(-)-Menthol
(1R-(1-alpha,2-beta,5-alpha))-5-Methyl-2-(1-methylethyl)cyclohexanol
(1S, 2S, 5R)-(+)-Neomenthol
(1S,2R,5R)-(+)-Isomenthol
(1S,2R,5S)-(+)-Menthol
(1S,2R,5S)-Menthol
(1alpha,2beta,5alpha)-5-Methyl-2(1-methylethyl)cyclohexanol
(1r,2s,5r)-(-)-menthol
(L)-MENTHOL
(R)-(-)-Menthol
(r)-(-)-menthol
)-Menthol
--MENTHOL
1-Menthol
1490-04-6
15356-60-2
15356-70-4
15785_RIEDEL
15785_SIAL
19863P
2-Isopropyl-5-methylcyclohexanol
20747-49-3
2216-51-5
3-p-Menthol
4-Isopropyl-1-methylcyclohexan-3-ol
491-02-1
5-Methyl-2-(1-methylethyl)-cyclohexanol
5-Methyl-2-(1-methylethyl)cyclohexanol
5-methyl-2-(propan-2-yl)cyclohexanol
5-methyl-2-propan-2-ylcyclohexan-1-ol
551376_ALDRICH
551376_FLUKA
588733_ALDRICH
613290_ALDRICH
613290_FLUKA
63660_FLUKA
63670_ALDRICH
63670_FLUKA
63975-60-0
6C6A4A8C-A054-468C-A1F0-F29E39838CF2
89-78-1
98167-53-4
AC1L1B2E
AC1L28FR
AC1Q1NQ2
AC1Q2QQM
AI3-08161
AI3-52408
AKOS000119740
AR-1J3337
BB_NC-0057
BRN 1902288
BRN 3194263
BSPBio_003062
C00400
C10H20O
CCRIS 3728
CCRIS 375
CCRIS 4666
CCRIS 9231
CHEBI:15409
CHEBI:545611
CHEMBL256087
CHEMBL470670
CID1254
CID16666
Caswell No. 540
D-(-)-Menthol
D-p-Menthan-3-ol
D00064
D008610
D04849
D04918
DB00825
DivK1c_000820
EINECS 201-939-0
EINECS 207-724-8
EINECS 216-074-4
EINECS 218-690-9
EINECS 239-387-8
EINECS 239-388-3
EPA Pesticide Chemical Code 051601
FEMA No. 2665
Fisherman's friend lozenges
Fisherman's friend lozenges (TN)
HMS1922G13
HMS2092L14
HMS502I22
 
HSDB 5662
HSDB 593
Headache crystals
Hexahydrothymol
I06-1216
I14-7371
IDI1_000820
KBio1_000820
KBio2_000785
KBio2_003353
KBio2_005921
KBio3_002562
KBioSS_000785
L-(-)-Menthol
L-(-)-menthol
L-Menthol
L-menthol
LMPR0102090001
LS-2353
LS-57201
LS-886
LS-89531
LS-89533
Levomenthol
Levomenthol [INN:BAN]
Levomentholum
Levomentholum [INN-Latin]
Levomentol
M0321
M0545
M2772_SIAL
MENTHOL
MLS002207256
Menthacamphor
Menthol
Menthol (USP)
Menthol (VAN)
Menthol natural
Menthol natural, brazilian
Menthol racemic
Menthol racemique
Menthol racemique [French]
Menthol solution
Menthol, (1alpha,2beta,5alpha)-Isomer
Menthomenthol
Menthyl alcohol
MolPort-000-849-729
MolPort-001-793-392
NCGC00159382-02
NCGC00159382-03
NCGC00164247-01
NCGC00164247-02
NCI-C50000
NINDS_000820
NOOLISFMXDJSKH-KXUCPTDWBX
NSC 2603
NSC 62788
NSC2603
NSC62788
Neoisomenthol
Peppermint camphor
RACEMIC MENTHOL U.S.P.
Racementhol
Racementhol [INN:BAN]
Racementholum
Racementholum [INN-Latin]
Racementol
Racementol [INN-Spanish]
Racemic menthol
Robitussin Cough Drops
SDCCGMLS-0066659.P001
SMR001306785
SPBio_000869
SPECTRUM1503134
STK802468
Spectrum2_000855
Spectrum3_001561
Spectrum5_001060
Spectrum_000305
Tra-kill tracheal mite killer
U.S.P. Menthol
U.S.p. Menthol
UNII-BZ1R15MTK7
UNII-L7T10EIP3A
UNII-YS08XHA860
W266507_ALDRICH
W266523_ALDRICH
W266590_ALDRICH
WLN: L6TJ AY1&1 BQ D1
WLN: L6TJ AY1&1 DQ D1 -L
ZINC01482164
cis-1 ,3-trans-1,4-(+-)-menthol
cis-1,3-trans-1,4-(+-)-menthol
d,l-Menthol
d-Menthol
d-Neomenthol
dl-3-p-Menthanol
dl-Menthol
dl-Menthol (JP15)
l-(-)-Menthol
l-Menthol
l-Menthol (JP15)
l-Menthol (TN)
l-Menthol (natural)
nchembio862-comp1
p-Menthan-3-ol
rac-Menthol
3
Oxaliplatin136661825-94-35310940, 9887054, 43805, 6857599, 9887054
Synonyms:
CHEMBL1201055
CID9887054
D01790
DACPLAT
Diaminocyclohexane Oxalatoplatinum
Eloxatin
Eloxatin (TN)
Elplat
Foloxatine
L-OHP
Oxalatoplatin
 
Oxalatoplatinum
Oxaliplatin (JAN/USAN/INN)
Oxaliplatin [Usan:Inn:Ban]
Oxaliplatino [Spanish]
Oxaliplatinum [Latin]
Oxaloplatine [French]
Oxaloplatino [Spanish]
Transplatin
oxaliplatin
oxaliplatine
oxaliplatino
oxaliplatinum

Interventional clinical trials:

idNameStatusNCT IDPhase
1A Study of Sativex® for Relief of Peripheral Neuropathic Pain Associated With Allodynia.CompletedNCT00711880Phase 3
2Interest of Pregabalin (Lyrica) on the Treatment of Reflex Earache in Head and Neck Cancer.RecruitingNCT02924675Phase 2
3Using Game-based Exercise to Improve Balance in Cancer PatientsNot yet recruitingNCT02773329Phase 2
4Auditory Neuropathy and Cochlear ImplantsCompletedNCT01023932
5Hearing Loss and Dizziness in Patients Receiving Oxaliplatin for Solid TumorsCompletedNCT00305799
6Auditory Brainstem Implantation in Young ChildrenRecruitingNCT01904448Phase 0
7Giant Axonal Neuropathy Natural History StudyActive, not recruitingNCT01503125
8Photobiomodulation Therapy Using the Realief Therapy System for the Treatment of Chemotherapy-associated Peripheral NeuropathyActive, not recruitingNCT02000908
9Follow-up Survey of Patients Who Were Treated for Medulloblastoma or Primitive Neuroectodermal Tumors of the Central Nervous in NorwayEnrolling by invitationNCT02851355

Search NIH Clinical Center for Auditory Neuropathy

Genetic Tests for Auditory Neuropathy

About this section

Genetic tests related to Auditory Neuropathy:

id Genetic test Affiliating Genes
1 Auditory Neuropathy25

Anatomical Context for Auditory Neuropathy

About this section

MalaCards organs/tissues related to Auditory Neuropathy:

34
Brain, Testes, Cortex, Bone

Animal Models for Auditory Neuropathy or affiliated genes

About this section

MGI Mouse Phenotypes related to Auditory Neuropathy:

39
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053778.6DFNB59, GJB2, GJB6, GSC, OPA1, OTOF
2MP:00036317.5AIFM1, DFNB59, GJB2, GJB6, GRIA3, GSC

Publications for Auditory Neuropathy

About this section

Articles related to Auditory Neuropathy:

(show top 50)    (show all 310)
idTitleAuthorsYear
1
Diverse etiologies manifesting auditory neuropathy characteristics from infants with profound hearing loss and clinical implications. (27260582)
2016
2
Detection Rates of Cortical Auditory Evoked Potentials at Different Sensation Levels in Infants with Sensory/Neural Hearing Loss and Auditory Neuropathy Spectrum Disorder. (27587922)
2016
3
Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency. (27144235)
2016
4
Cortical development and neuroplasticity in Auditory Neuropathy Spectrum Disorder. (26070426)
2015
5
Saccular dysfunction in children with sensorineural hearing loss and auditory neuropathy/auditory dys-synchrony. (26246016)
2015
6
OPA1-related auditory neuropathy: site of lesion and outcome of cochlear implantation. (25564500)
2015
7
Auditory Neuropathy/Dys-Synchrony Disorder: Diagnosis and Management. (26296649)
2015
8
Auditory neuropathy in individuals with Type 1 diabetes. (24846202)
2014
9
Auditory neuropathy/dyssynchrony revisited. (25090808)
2014
10
Cochlear implantation in children with auditory neuropathy spectrum disorders. (24869444)
2014
11
Speech and language outcomes of cochlear implantation in children with isolated auditory neuropathy versus cochlear hearing loss. (24232062)
2013
12
Objective Measures of Electrode Discrimination With Electrically Evoked Auditory Change Complex and Speech-Perception Abilities in Children With Auditory Neuropathy Spectrum Disorder. (24231629)
2013
13
Persistence of otoacoustic emissions in children with auditory neuropathy spectrum disorders. (23411132)
2013
14
Temporal processing and speech perception in noise by listeners with auditory neuropathy. (23409105)
2013
15
Genetic analysis of auditory neuropathy spectrum disorder in the Korean population. (23562982)
2013
16
Involvement of peripheral vestibular nerve in individuals with auditory neuropathy. (23143643)
2013
17
Analysis of speech perception outcomes among patients receiving cochlear implants with auditory neuropathy spectrum disorder. (24080979)
2013
18
Auditory and communicative abilities in the auditory neuropathy spectrum disorder and mutation in the Otoferlin gene: clinical cases study. (22832688)
2012
19
Auditory neuropathy spectrum: the importance of adequate diagnosis. (24333894)
2012
20
A prevalent founder mutation and genotype-phenotype correlations of OTOF in Japanese patients with auditory neuropathy. (22575033)
2012
21
Perception of temporally modified speech in auditory neuropathy. (21047293)
2011
22
Impairments of the medial olivocochlear system increase the risk of noise-induced auditory neuropathy in laboratory mice. (21956602)
2011
23
Clinical Application of the P1 Cortical Auditory Evoked Potential Biomarker in Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder. (24078765)
2011
24
Auditory neuropathy spectrum disorder in late preterm and term infants with severe jaundice. (20832127)
2010
25
Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss. (19409623)
2009
26
Adults with auditory neuropathy: comparison of auditory steady-state response and pure-tone audiometry. (20503800)
2009
27
Transplantation of mouse embryonic stem cells into the cochlea of an auditory-neuropathy animal model: effects of timing after injury. (18449604)
2008
28
The SP-AP compound wave in patients with auditory neuropathy. (18612852)
2008
29
Current understanding of auditory neuropathy. (19904452)
2008
30
Cochlear implantation in 3 adults with auditory neuropathy/auditory dys-synchrony. (18949967)
2008
31
Enhanced survival of bone-marrow-derived pluripotent stem cells in an animal model of auditory neuropathy. (17632425)
2007
32
Cortical auditory evoked potentials in the assessment of auditory neuropathy: two case studies. (17715648)
2007
33
Auditory neuropathy in a patient with mitochondrial myopathy and multiple mtDNA deletions. (16719954)
2006
34
Transient infantile auditory neuropathy and its clinical implications. (16769129)
2006
35
Multiple auditory steady-state responses in children and adults with normal hearing, sensorineural hearing loss, or auditory neuropathy. (16676823)
2006
36
Auditory neuropathy in systemic sclerosis: a speech perception and evoked potential study before and after cochlear implantation. (16763823)
2006
37
Auditory neuropathy in children: diagnostic criteria and audiological test results. (16340284)
2005
38
A novel missense mutation in a C2 domain of OTOF results in autosomal recessive auditory neuropathy. (16097006)
2005
39
Auditory neuropathy in hyperbilirubinemia: is there a correlation between serum bilirubin, neuron-specific enolase levels and auditory neuropathy? (15726842)
2004
40
Auditory neuropathy/dyssynchrony: its diagnosis and management. (12809326)
2003
41
Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. (12525542)
2003
42
Consequences of neural asynchrony: a case of auditory neuropathy. (11548236)
2000
43
Auditory neuropathy in children. (11603772)
2000
44
Hereditary motor and sensory neuropathy associated with auditory neuropathy in a Gypsy family. (10653194)
2000
45
Auditory neuropathy and a mitochondrial disorder in a child: case study. (10522621)
1999
46
Clinical findings for a group of infants and young children with auditory neuropathy. (10386850)
1999
47
Auditory deprivation of the central auditory system resulting from selective inner hair cell loss: animal model of auditory neuropathy. (10803909)
1999
48
Transient deafness due to temperature-sensitive auditory neuropathy. (9657592)
1998
49
Temporal distortion products (kernel slices) evoked by maximum-length-sequences in auditory neuropathy: evidence for a cochlear pre-synaptic origin. (9076248)
1997
50
Auditory neuropathy. (8673487)
1996

Variations for Auditory Neuropathy

About this section

Clinvar genetic disease variations for Auditory Neuropathy:

5
id Gene Variation Type Significance SNP ID Assembly Location
1MT-RNR1m.1095T> Csingle nucleotide variantdrug responsers267606618GRCh37Chr MT, 1095: 1095

Expression for genes affiliated with Auditory Neuropathy

About this section
Search GEO for disease gene expression data for Auditory Neuropathy.

Pathways for genes affiliated with Auditory Neuropathy

About this section

GO Terms for genes affiliated with Auditory Neuropathy

About this section

Cellular components related to Auditory Neuropathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1connexin complexGO:000592210.5GJB2, GJB6
2gap junctionGO:00059219.9GJB2, GJB6
3cell junctionGO:00300549.1GJB2, GJB6, GRIA3, OTOF

Biological processes related to Auditory Neuropathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cochlea developmentGO:009010210.1OTOF, SLITRK6
2sensory perception of soundGO:00076059.1DFNB59, GJB2, GJB6, OTOF, SLITRK6

Sources for Auditory Neuropathy

About this section
2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet