Abstract:

Objective    To explore the mechanism of hereditary hearing loss. Methods     Cochlea from cCx26 mice on P8（the 8th postnatal day）, P12 （the 12th postnatal day）and P21（the 21st postnatal day） were examined with scanning microscope (SEM).  Results    On P8 and P12, the external morphology of inner hair cells (IHC), outer hair cells (OHC) and supporting cells developed normally. But in P21 stage, extensive coalesce or absence of stereo cilia of OHC and loss of OHC in the basal and middle turn of cochlea were seen. And the cuticular plate was shriveled and damaged， however， the IHCs in the cochlea were intact without any obvious change. Conclusion     Mutation of GJB2 may cause the absence of stereo cilia of OHC, the shrinkage and the damage of Corti′s cuticular plate in mice. All these morphologic changes of cochlea may occur before P21.

Key words: Connexin26; Mutation; Hair Cell, Auditory; Scanning Electron Microscopy