Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.


A form of non-syndromic sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Patients may have mild thrombocytopenia and enlarged platelets, although most of DFNA1 affected individuals do not have significant bleeding tendencies.




Hereditary low-frequency hearing loss
Hereditary low-frequency sensorineural hearing loss
Konigsmark syndrome
Non-syndromic neurosensory deafness autosomal dominant type 1
Non-syndromic sensorineural deafness autosomal dominant type 1


Non-syndromic deafness


Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice.

UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again