Hereditary deafness has proved to be extremely heterogeneous genetically with more than
40 genes mapped or cloned for non-syndromic dominant deafness and 30 for autosomal …

▪ Abstract Hereditary isolated hearing loss is genetically highly heterogeneous. Over 100
genes are predicted to cause this disorder in humans. Sixty loci have been reported and 24 …

Congenital profound deafness has a known genetic origin in more than 50% of all cases.
The majority of the non syndromic hearing loss (NSHL) show an autosomal recessive …

Summary Progress in the Human Genome Project, availability of cochlea-specific cDNA
libraries, and development of murine models of deafness have resulted in rapid discovery of …

Deafness is an etiologically heterogeneous trait with many known genetic and
environmental causes. Genetic factors account for at least half of all cases of profound …

Non‐syndromic deafness is a paradigm of genetic heterogeneity. More than 70 loci have
been mapped, and 25 of the nuclear genes responsible for non‐syndromic deafness have …

Hearing impairment is a sensory disability that affects millions of people all over the world.
Though not lifethreatening, it can become a major burden in social and professional life. In …

Approximately one in 2000 children is born with a genetic hearing impairment, mostly
inherited as a non‐syndromic, autosomal recessive trait, for which more than 30 different …

Mutations in the GJB2 gene have been identified in many patients with childhood deafness,
35delG being the most common mutation in Caucasoid populations. We have analyzed a …

Mutations in GJB2 are the most common cause of hereditary congenital hearing loss in
many countries and are found in about half of persons with severe‐to‐profound congenital …