Molecular Mechanism of Hearing and Different Types of Genetic Hearing Loss in Iran
|
N. Mahdieh (PhD) * , B. Rabbani (PhD) |
|
|
Abstract: (10136 Views) |
BACKGROUND AND
OBJECTIVE: Hearing impairment is the most common sensory defect in
human beings this highly heterogeneous disorder affecting 1 in 1000 among
general population that half of them is due to genetic agents. Albeit, this
frequency in Iran is higher than other countries and it needs to pay more
attention to this condition. Ear acts as a connector between human and
environment hearing process is composed of a series of complex events thorough
the ear so that sound waves lead to physical vibrations in inner ear producing
electrical signals. This signal is processed within the hearing regions in two
sides of the brain. Sensorineural hearing loss (HL) is classified into acquired
HL, genetic HL and deafness of unknown origin. If other than
deafness, other problems occur in affected individuals, HL is belonged to
syndromic form of deafness while in many cases, hearing loss occurs without
any other problems. METHODS: In this study, mechanisms of hearing,
various types of hearing loss and genetics studies in Iran in comparison with
other population of the world are investigated and reviewed. FINDINGS: GJB2
mutations are the most common cause of autosomal recessive non-syndromic
hearing loss (ARNSHL) in most populations and even up to 50% of ARNSHL in some
cohorts is due to these mutations. GJB2 mutation frequency is 14-17% among
Iranian populations. More than 90% of genetics studies of HL have been
performed on GJB2 mutations. However, there are some reports about other loci
involving in HL. CONCLUSION: Mutations in more than ten genes lead to ARNSHL in our
country that GJB2 has more important role than other genes. It showed GJB2
connexin 26 in Iran |
|
Keywords: Hearing loss, Iranian population, GJB2 |
|
Full-Text [PDF 702 kb]
(3435 Downloads)
|
Type of Study: Research |
Subject:
Biochemical Received: 2013/03/5 | Accepted: 2013/07/10 | Published: 2014/08/9
|
|
|
|