Heterogeneity of Hereditary Hearing Loss in Iran: a Comprehensive Review

Arch Iran Med. 2016 Oct 1;19(10):720-728.


A significant contribution to the causes of hereditary hearing impairment comes from genetic factors. More than 120 genes and 160 loci have been identified to be involved in hearing impairment. Given that consanguine populations are more vulnerable to most inherited diseases, such as hereditary hearing loss (HHL), the genetic picture of HHL among the Iranian population, which consists of at least eight ethnic subgroups with a high rate of intermarriage, is expected to be highly heterogeneous. Using an electronic literature review through various databases such as PubMed, MEDLINE, and Scopus, we review the current picture of HHL in Iran. In this review, we present more than 39 deafness genes reported to cause non-syndromic HHL in Iran, of which the most prevalent causative genes include GJB2, SLC26A4, MYO15A, and MYO7A. In addition, we highlight some of the more common genetic causes of syndromic HHL in Iran. These results are of importance for further investigation and elucidation of the molecular basis of HHL in Iran and also for developing a national diagnostic tool tailored to the Iranian context enabling early and efficient diagnosis of hereditary hearing impairment.

Publication types

  • Review

MeSH terms

  • Connexin 26
  • Connexins / genetics
  • Consanguinity*
  • Delivery of Health Care
  • Genetic Testing
  • Hearing Loss, Sensorineural / epidemiology*
  • Hearing Loss, Sensorineural / genetics*
  • Humans
  • Iran / epidemiology
  • Membrane Transport Proteins / genetics
  • Mutation
  • Myosin VIIa
  • Myosins / genetics
  • Sulfate Transporters


  • Connexins
  • GJB2 protein, human
  • MYO15A protein, human
  • MYO7A protein, human
  • Membrane Transport Proteins
  • Myosin VIIa
  • SLC26A4 protein, human
  • Sulfate Transporters
  • Connexin 26
  • Myosins