Health knowledge made personal
Join this community!
› Share page:
Go
Search posts:

What is the mechanism of deafness in Jervell and Lange-Nielsen syndrome ?

Posted Feb 22 2010 8:23am

The same channels , that  create the  deadly prolonged  QT interval  by  delaying the  repolarisation  in the heart  is  responsible in the for the deafness  as it interfere with inner ear

Mechanotransduction of sound into neural signals .

For proper auditory function  , the cochlear hair cells   needs  a continuous flow of endolymph which  maintain a voltage gradient for  nerve  signal  transmission  .The lymph secretion is  is regulated by potassium channels  KCNQ1 and KCNE1 . Mutations of this gene impairs  the K + content of the endolymph. It results in  a compromised  endocochlear potential (Difference between peri lymph and endolymph potenial )  .This result in irreversible deafness .

Link to   a good   illustration from Medical physiology

Post a comment
Write a comment:

Related Searches