Details of Disease

General Information of Disease (ID: DISC5R5F)

Disease Name	Meniere disease
Synonyms	Meniere's disease; Meniere disease; otogenic vertigo; Mnire's vertigo
Disease Class	AB31: Episodic vestibular syndrome
Definition	A disease of the inner ear (labyrinth) that is characterized by fluctuating sensorineural hearing loss; tinnitus; episodic vertigo; and aural fullness. It is the most common form of endolymphatic hydrops.
Disease Hierarchy	DISUEW2W: Hereditary otorhinolaryngologic disease DISLG2RO: Hereditary neuromuscular disease DISUPJBC: Endolymphatic hydrops DISRDGIM: Inherited auditory system disease DISC5R5F: Meniere disease
ICD Code	ICD-11 ICD-11: AB31.0 ICD-10 ICD-10: H81 Expand ICD-10 H81
Disease Identifiers	MONDO ID MONDO_0007972 MESH ID D008575 UMLS CUI C0025281 OMIM ID 156000 MedGen ID 7530 Orphanet ID 45360 SNOMED CT ID 13445001

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 7 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Cinnarizine	DM7U5QJ	Approved	Small molecular drug	[1]
Dimenhydrinate	DM264B3	Approved	Small molecular drug	[2]
Diphenhydramine	DMKQTBA	Approved	Small molecular drug	[3]
Famciclovir	DMJHLSD	Approved	Small molecular drug	[4]
Meclizine	DMS7T13	Approved	Small molecular drug	[5]
Penciclovir	DMOUMDV	Approved	Small molecular drug	[6]
SPI-1005	DM6XFHS	Approved	Small molecular drug	[7]
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⏷ Show the Full List of 7 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 5 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CRLF1	TT6YF5K	Limited	Biomarker	[8]
HRH4	TTXJ178	Limited	Genetic Variation	[9]
MTNR1B	TT32JK8	Limited	Genetic Variation	[10]
SIK1	TT1H6LC	Limited	Genetic Variation	[11]
PRKCB	TTYPXQF	moderate	Biomarker	[12]
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This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC44A2	DTSF91X	Limited	Biomarker	[13]
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This Disease Is Related to 16 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
AQP2	OTQLBKK6	Limited	Altered Expression	[14]
DTNA	OTVBIRH2	Limited	Autosomal dominant	[15]
FAM136A	OT9WY5AH	Limited	Unknown	[16]
HMX2	OTBRXY6D	Limited	Genetic Variation	[17]
PLXNA3	OTMZIBVG	Limited	Genetic Variation	[18]
SEMA3D	OTD5TJV1	Limited	Genetic Variation	[19]
TLR10	OTQ1KVJO	Limited	Genetic Variation	[20]
TSHZ1	OTYQ9ECW	Limited	Genetic Variation	[13]
ADD1	OTTF68DC	moderate	Genetic Variation	[21]
AQP6	OTXS6UYY	moderate	Altered Expression	[22]
FCGR2C	OTNLMNYB	moderate	Biomarker	[23]
HCFC1	OT0UCK62	moderate	Genetic Variation	[24]
KCNE3	OTKWKR91	moderate	Biomarker	[25]
AQP4	OTA9MYD5	Strong	Genetic Variation	[26]
COCH	OTBEHD89	Strong	Genetic Variation	[27]
KCNE1	OTZNQUW9	Strong	Genetic Variation	[28]
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⏷ Show the Full List of 16 DOT(s)

References

1	Association of cinnarizine and betahistine in prophylactic therapy for Mnire's disease with and without migraine. Acta Otorhinolaryngol Ital. 2014 Oct;34(5):349-53.
2	Dimenhydrinate FDA Label
3	Drugs@FDA. U.S. Food and Drug Administration. U.S. Department of Health & Human Services. 2015
4	Famciclovir FDA Label
5	Meclizine FDA Label
6	Penciclovir FDA Label
7	Sound Pharmaceuticals announces positive topline results from the SPI-1005 Phase 2b Meniere's Disease clinical trial. Jun 25, 2019
8	Author's reply to "Letter to the editor on the article: Saccular measurements in routine MRI can predict hydrops in Menire's disease by Simon F et al.".Eur Arch Otorhinolaryngol. 2018 Jan;275(1):313-314. doi: 10.1007/s00405-017-4814-2. Epub 2017 Nov 22.
9	Histamine H4 receptor gene polymorphisms: a potential contributor to Meniere disease.BMC Med Genomics. 2019 May 27;12(1):71. doi: 10.1186/s12920-019-0533-4.
10	Polymorphisms in genes involved in the free-radical process in patients with sudden sensorineural hearing loss and Mnire's disease.Free Radic Res. 2013 Jul;47(6-7):498-506. doi: 10.3109/10715762.2013.793319. Epub 2013 May 7.
11	Endolymphatic hydrops and ionic transporters: genetic and biohumoral aspects.J Neurol. 2019 Sep;266(Suppl 1):47-51. doi: 10.1007/s00415-019-09399-6. Epub 2019 Jun 5.
12	A novel missense variant in PRKCB segregates low-frequency hearing loss in an autosomal dominant family with Meniere's disease.Hum Mol Genet. 2016 Aug 15;25(16):3407-3415. doi: 10.1093/hmg/ddw183. Epub 2016 Jun 21.
13	SLC44A2 single nucleotide polymorphisms, isoforms, and expression: Association with severity of Meniere's disease?.Genomics. 2016 Dec;108(5-6):201-208. doi: 10.1016/j.ygeno.2016.11.002. Epub 2016 Nov 6.
14	Mnire's Disease Pathophysiology: Endolymphatic Sac Immunohistochemical Study of Aquaporin-2, V2R Vasopressin Receptor, NKCC2, and TRPV4.Otolaryngol Head Neck Surg. 2018 Apr;158(4):721-728. doi: 10.1177/0194599818756829. Epub 2018 Feb 13.
15	Classification of Genes: Standardized Clinical Validity Assessment of Gene-Disease Associations Aids Diagnostic Exome Analysis and Reclassifications. Hum Mutat. 2017 May;38(5):600-608. doi: 10.1002/humu.23183. Epub 2017 Feb 13.
16	Identification of two novel mutations in FAM136A and DTNA genes in autosomal-dominant familial Meniere's disease. Hum Mol Genet. 2015 Feb 15;24(4):1119-26. doi: 10.1093/hmg/ddu524. Epub 2014 Oct 9.
17	Whole-exome sequencing suggests multiallelic inheritance for childhood-onset Mnire's disease.Ann Hum Genet. 2019 Nov;83(6):389-396. doi: 10.1111/ahg.12327. Epub 2019 May 20.
18	Association between polymorphisms in genes encoding methylenetetrahydrofolate reductase and the risk of Mnire's disease.J Neurogenet. 2013 Jun;27(1-2):5-10. doi: 10.3109/01677063.2013.770510. Epub 2013 Mar 13.
19	Variable expressivity and genetic heterogeneity involving DPT and SEMA3D genes in autosomal dominant familial Meniere's disease.Eur J Hum Genet. 2017 Feb;25(2):200-207. doi: 10.1038/ejhg.2016.154. Epub 2016 Nov 23.
20	Allelic variants in TLR10 gene may influence bilateral affectation and clinical course of Meniere's disease.Immunogenetics. 2013 May;65(5):345-55. doi: 10.1007/s00251-013-0683-z. Epub 2013 Feb 1.
21	Gly460Trp alpha-adducin mutation as a possible mechanism leading to endolymphatic hydrops in Mnire's syndrome.Otol Neurotol. 2008 Sep;29(6):824-8. doi: 10.1097/MAO.0b013e318180a4b1.
22	Immunohistochemical localization and mRNA expression of aquaporins in the macula utriculi of patients with Meniere's disease and acoustic neuroma.Cell Tissue Res. 2010 Jun;340(3):407-19. doi: 10.1007/s00441-010-0975-7. Epub 2010 May 12.
23	Polymorphisms of CD16A and CD32 Fc receptors and circulating immune complexes in Mnire's disease: a case-control study.BMC Med Genet. 2011 Jan 5;12:2. doi: 10.1186/1471-2350-12-2.
24	Sequence variants in host cell factor C1 are associated with Mnire's disease.Otol Neurotol. 2008 Jun;29(4):561-6. doi: 10.1097/MAO.0b013e318168d23b.
25	Polymorphisms in KCNE1 or KCNE3 are not associated with Mnire disease in the Caucasian population.Am J Med Genet A. 2010 Jan;152A(1):67-74. doi: 10.1002/ajmg.a.33114.
26	Polymorphisms in genes encoding aquaporins 4 and 5 and estrogen receptor in patients with Mnire's disease and sudden sensorineural hearing loss.Life Sci. 2013 Mar 21;92(10):541-6. doi: 10.1016/j.lfs.2013.01.019. Epub 2013 Jan 24.
27	Distinct vestibular phenotypes in DFNA9 families with COCH variants.Eur Arch Otorhinolaryngol. 2016 Oct;273(10):2993-3002. doi: 10.1007/s00405-015-3885-1. Epub 2016 Jan 13.
28	Mnire's Disease: Molecular Analysis of Aquaporins 2, 3 and Potassium Channel KCNE1 Genes in Brazilian Patients.Otol Neurotol. 2016 Sep;37(8):1117-21. doi: 10.1097/MAO.0000000000001136.