Open main menu
Home
Random
Recent changes
Special pages
Community portal
Preferences
About Wikipedia
Disclaimers
Incubator escapee wiki
Search
User menu
Talk
Dark mode
Contributions
Create account
Log in
Editing
Sensorineural hearing loss
(section)
Warning:
You are not logged in. Your IP address will be publicly visible if you make any edits. If you
log in
or
create an account
, your edits will be attributed to your username, along with other benefits.
Anti-spam check. Do
not
fill this in!
===Genetic=== Hearing loss can be inherited. More than 40 genes have been implicated in the cause of deafness.<ref>{{Cite journal |vauthors=Matsunaga T |date=December 2009 |title=Value of genetic testing in the otological approach for sensorineural hearing loss |journal=The Keio Journal of Medicine |volume=58 |issue=4 |pages=216–22 |doi=10.2302/kjm.58.216 |pmid=20037285 |doi-access=free}}</ref> There are 300 syndromes with related hearing loss, and each syndrome may have causative genes.{{citation needed|date=February 2023}} [[Recessive]], [[Dominant gene|dominant]], [[X-linked]], or [[Mitochondrial gene|mitochondrial]] genetic mutations can affect the structure or metabolism of the inner ear. Some may be [[Point mutation|single point mutations]], whereas others are due to [[chromosomal abnormalities]]. Some genetic causes give rise to a late onset hearing loss. Mitochondrial mutations can cause SNHL i.e. m.1555A>G, which makes the individual sensitive to the ototoxic effects of [[aminoglycoside antibiotics]].{{cn|date=December 2024}} * The most common cause of recessive genetic congenital hearing impairment in developed countries is [[DFNB1]], also known as Connexin 26 deafness or [[GJB2]]-related deafness. * The most common syndromic forms of hearing impairment include (dominant) [[Stickler syndrome]] and [[Waardenburg syndrome]], and (recessive) [[Pendred syndrome]] and [[Usher syndrome]]. * Mitochondrial mutations causing deafness are rare: [[MT-TL1]] mutations cause [[MIDD]] (Maternally inherited deafness and diabetes) and other conditions which may include deafness as part of the picture. * [[TMPRSS3]] gene was identified by its association with both congenital and childhood onset autosomal recessive deafness. This gene is expressed in fetal cochleae and many other tissues, and is thought to be involved in the development and maintenance of the [[inner ear]] or the contents of the [[perilymph]] and [[endolymph]]. It was also identified as a tumor associated gene that is overexpressed in [[ovarian tumor]]s. * [[Charcot–Marie–Tooth disease]]<ref name="Papadakis 2003">{{Cite journal |vauthors=Papadakis CE, Hajiioannou JK, Kyrmizakis DE, Bizakis JG |date=May 2003 |title=Bilateral sudden sensorineural hearing loss caused by Charcot-Marie-Tooth disease |journal=The Journal of Laryngology and Otology |volume=117 |issue=5 |pages=399–401 |doi=10.1258/002221503321626465 |pmid=12803792}}</ref> an inherited neurological disorder with delayed onset that can affect the ears as well as other organs. The hearing loss in this condition is often ANSD (auditory neuropathy spectrum disorder) a neural cause of hearing loss. * [[Muckle–Wells syndrome]], a rare inherited [[autoinflammatory]] disorder, can lead to hearing loss. * [[Autoimmune disease]]: although probably rare, it is possible for autoimmune processes to target the cochlea specifically, without symptoms affecting other organs. [[Granulomatosis with polyangiitis]], an autoimmune condition, may precipitate hearing loss.
Edit summary
(Briefly describe your changes)
By publishing changes, you agree to the
Terms of Use
, and you irrevocably agree to release your contribution under the
CC BY-SA 4.0 License
and the
GFDL
. You agree that a hyperlink or URL is sufficient attribution under the Creative Commons license.
Cancel
Editing help
(opens in new window)