Dictionary of Human Evolution and Biology

  • -id > 9:3
   

Deafness, Neurosensory

Impairment of the sense of hearing, either congenitally or through postnatal los; the term can refer to either partial or complete deafness. Although over seventy genes are known to cause congenital hearing los, in the most common mode, hearing los is due to mutations in the gap junction protein connexin, an autosomal recessive. Hearing los that is genetic in origin accounts for about half of all cases. Nonsyndromic deafness is a genetic defect in a single gene that causes multiple medical problems, only one of which is deafness, as in Waardenburg syndrome; 70% of cases of inherited deafness are nonsyndromic (i.e. 35% of all cases). Syndromic deafness is a genetic defect in a single gene that causes a single medical problem, deafness, as in Pendred syndrome; 30% of cases of inherited deafness are nonsyndromic (i.e. 15% of all cases). Aka hereditary hearing los.

See inherited deafness.

Full-Text Search Entries