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Autosomal recessive nonsyndromic hearing loss 1A(DFNB1A)

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: Connexin 26 deafness; Deafness nonsyndromic, Connexin 26 linked; Deafness, autosomal recessive 1A; DFNB 1 Nonsyndromic Hearing Loss and Deafness; DFNB1A; GJB6-Related DFNB 1 Nonsyndromic Hearing Loss and Deafness; Nonsyndromic Hearing Loss and Deafness, DFNB1
Genes (locations): GJB2 (13q12.11); GJB3 (1p34.3); GJB6 (13q12.11)
Monarch Initiative: MONDO:0009076
OMIM®: 220290


Nonsyndromic hearing loss and deafness (DFNB1) is characterized by congenital non-progressive mild-to-profound sensorineural hearing impairment. No other associated medical findings are present. [from GeneReviews]

Clinical features

From HPO
Sensorineural hearing impairment
MedGen UID:
Concept ID:
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Abnormal vestibular function
MedGen UID:
Concept ID:
An abnormality of the functioning of the vestibular apparatus.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Recent clinical studies

Clinical prediction guides

Scott DA, Carmi R, Elbedour K, Yosefsberg S, Stone EM, Sheffield VC
Am J Hum Genet 1996 Aug;59(2):385-91. PMID: 8755925Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines


    • NICE, 2019
      Cochlear implants for children and adults with severe to profound deafness (2019 Update)
    • NICE, 2009
      National Institute for Health and Clinical Excellence, Cochlear implants for children and adults with severe to profound deafness, 2009 [See 2019 Update, TA566]

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