autosomal dominant nonsyndromic hearing loss
MONDO:0019587Mondo
Findings
No curated finding names autosomal dominant nonsyndromic hearing loss yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Autosomal dominant form of nonsyndromic deafness.
Definition from the Mondo Disease Ontology (MONDO:0019587), read 2026-09-29. CC BY 4.0.
Genes
49 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- ATP2B2HGNC:815
- Definitive · ClinGen · Autosomal dominant · 2025
- LMX1AHGNC:6653
- Definitive · ClinGen · Autosomal dominant · 2025
- ATP11AHGNC:13552
- Moderate · ClinGen · Autosomal dominant · 2025
- CD164HGNC:1632
- Moderate · ClinGen · Autosomal dominant · 2024
- Supportive · Orphanet · Autosomal dominant · 2021
- COL11A1HGNC:2186
- Moderate · ClinGen · Autosomal dominant · 2025
- PLS1HGNC:9090
- Moderate · ClinGen · Autosomal dominant · 2026
- Supportive · Orphanet · Autosomal dominant · 2021
- ABCC1HGNC:51
- Supportive · Orphanet · Autosomal dominant · 2021
- Limited · ClinGen · Autosomal dominant · 2025
- ACTG1HGNC:144
- Supportive · Orphanet · Autosomal dominant · 2021
- CCDC50HGNC:18111
- Supportive · Orphanet · Autosomal dominant · 2021
- CEACAM16HGNC:31948
- Supportive · Orphanet · Autosomal dominant · 2021
- COCHHGNC:2180
- Supportive · Orphanet · Autosomal dominant · 2021
- COL11A2HGNC:2187
- Supportive · Orphanet · Autosomal dominant · 2021
- CRYMHGNC:2418
- Supportive · Orphanet · Autosomal dominant · 2021
- DIABLOHGNC:21528
- Supportive · Orphanet · Autosomal dominant · 2021
- DIAPH3HGNC:15480
- Supportive · Orphanet · Autosomal dominant · 2021
- DMXL2HGNC:2938
- Supportive · Orphanet · Autosomal dominant · 2021
- EYA4HGNC:3522
- Supportive · Orphanet · Autosomal dominant · 2021
- GJB2HGNC:4284
- Supportive · Orphanet · Autosomal dominant · 2021
- GJB3HGNC:4285
- Supportive · Orphanet · Autosomal dominant · 2021
- GJB6HGNC:4288
- Supportive · Orphanet · Autosomal dominant · 2021
- GRHL2HGNC:2799
- Supportive · Orphanet · Autosomal dominant · 2021
- GSDMEHGNC:2810
- Supportive · Orphanet · Autosomal dominant · 2021
- HGNC:9679HGNC:9679
- Supportive · Orphanet · Autosomal dominant · 2021
- HOMER2HGNC:17513
- Supportive · Orphanet · Autosomal dominant · 2021
- KCNQ4HGNC:6298
- Supportive · Orphanet · Autosomal dominant · 2021
- KITLGHGNC:6343
- Supportive · Orphanet · Autosomal dominant · 2021
- MCM2HGNC:6944
- Supportive · Orphanet · Autosomal dominant · 2021
- MYH14HGNC:23212
- Supportive · Orphanet · Autosomal dominant · 2021
- MYH9HGNC:7579
- Supportive · Orphanet · Autosomal dominant · 2021
- MYO1CHGNC:7597
- Supportive · Orphanet · Autosomal dominant · 2021
- Disputed Evidence · ClinGen · Autosomal dominant · 2018
- MYO6HGNC:7605
- Supportive · Orphanet · Autosomal dominant · 2021
- MYO7AHGNC:7606
- Supportive · Orphanet · Autosomal dominant · 2021
- OSBPL2HGNC:15761
- Supportive · Orphanet · Autosomal dominant · 2021
- P2RX2HGNC:15459
- Supportive · Orphanet · Autosomal dominant · 2021
- PDE1CHGNC:8776
- Supportive · Orphanet · Autosomal dominant · 2021
- Limited · ClinGen · Autosomal dominant · 2026
- POU4F3HGNC:9220
- Supportive · Orphanet · Autosomal dominant · 2021
- SIX1HGNC:10887
- Supportive · Orphanet · Autosomal dominant · 2021
- SLC17A8HGNC:20151
- Supportive · Orphanet · Autosomal dominant · 2021
- Limited · Labcorp Genetics (formerly Invitae) · Autosomal dominant · 2019
- SLC44A4HGNC:13941
- Supportive · Orphanet · Autosomal dominant · 2021
- TBC1D24HGNC:29203
- Supportive · Orphanet · Autosomal dominant · 2021
- TECTAHGNC:11720
- Supportive · Orphanet · Autosomal dominant · 2021
- TJP2HGNC:11828
- Supportive · Orphanet · Autosomal dominant · 2021
- Limited · Ambry Genetics · Autosomal dominant · 2019
- TMC1HGNC:16513
- Supportive · Orphanet · Autosomal dominant · 2021
- TNCHGNC:5318
- Supportive · Orphanet · Autosomal dominant · 2021
- TRRAPHGNC:12347
- Supportive · Orphanet · Autosomal dominant · 2021
- WFS1HGNC:12762
- Supportive · Orphanet · Autosomal dominant · 2021
- HGNC:13664HGNC:13664
- Limited · Ambry Genetics · Autosomal dominant · 2025
- NCOA3HGNC:7670
- Limited · PanelApp Australia · Autosomal dominant · 2025
- RIPOR2HGNC:13872
- Limited · ClinGen · Autosomal dominant · 2024
Where it sits
- Narrower terms (76)
- autosomal dominant auditory neuropathy 1
- autosomal dominant nonsyndromic hearing loss 1
- autosomal dominant nonsyndromic hearing loss 10
- autosomal dominant nonsyndromic hearing loss 11
- autosomal dominant nonsyndromic hearing loss 12
- autosomal dominant nonsyndromic hearing loss 13
- autosomal dominant nonsyndromic hearing loss 15
- autosomal dominant nonsyndromic hearing loss 16
- autosomal dominant nonsyndromic hearing loss 17
- autosomal dominant nonsyndromic hearing loss 18
- autosomal dominant nonsyndromic hearing loss 20
- autosomal dominant nonsyndromic hearing loss 21
- autosomal dominant nonsyndromic hearing loss 22
- autosomal dominant nonsyndromic hearing loss 23
- autosomal dominant nonsyndromic hearing loss 24
- autosomal dominant nonsyndromic hearing loss 25
Other names
5 names
Resolves to: autosomal dominant nonsyndromic hearing loss
- Also called
- autosomal dominant isolated neurosensory hearing loss type DFNAautosomal dominant isolated sensorineural hearing loss type DFNAautosomal dominant non-syndromic neurosensory hearing loss type DFNAautosomal dominant non-syndromic sensorineural hearing loss type DFNAautosomal dominant nonsyndromic hearing impairment