Welcome to the Hereditary Hearing Loss Homepage

The Hereditary Hearing Loss Homepage aims to give an up-to-date overview of the genetics of hereditary hearing impairment for researchers and clinicians working in the field.

This website lists all known gene localizations and identifications for monogenic nonsyndromic hearing impairment. Inclusion of a locus or gene requires support from at least one publication.  Key references are provided and are selected using the following criteria:

  • First publication(s) to identify the locus or gene.
  • Publications that refine the mapping of the locus.
  • First publication(s) to dispute or refute the association of the locus or gene with hearing loss.

Evidence levels for each gene, in the form of ClinGen gene-disease validity classifications, are also listed when available. 

Summary of genes identified to date*

Total nonsyndromic hearing loss associated genes: 150
Autosomal dominant nonsyndromic hearing loss associated genes: 63
Autosomal recessive nonsyndromic hearing loss associated genes: 83
Sex-linked nonsyndromic hearing loss associated genes: 7
Mitochondrial nonsyndromic hearing loss associated genes: 9
Auditory neuropathy associated genes: 5

* Disputed or refuted genes are excluded from the counts.  Genes that are associated with both autosomal dominant and recessive hearing loss are only counted once in "total nonsyndromic hearing loss genes".

Most recent update: 2/17/24  (See updates log for details.)