autosomal dominant nonsyndromic hearing loss 23
Findings
No curated finding names autosomal dominant nonsyndromic hearing loss 23 yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the SIX1 gene.
Definition from the Mondo Disease Ontology (MONDO:0011519), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal dominant inheritance
HPO, annotations 2026-09-02
Features
2 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Conductive hearing impairmentHPOHP:0000405
- Frequent (30% to 79% of cases)
- Sensorineural hearing impairmentHPOHP:0000407
Genes
1 gene
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.
- SIX1HGNC:10887
- Definitive · G2P · Autosomal dominant · 2025
- Strong · PanelApp Australia · Autosomal dominant · 2025