Experimental Animal Models with features of Auditory Neuropathy
LI Peng
DING Dalian
ZENG Xiangli
Richard Salvi
Abstract：Clinically, auditory neuropathy is characterized by normal or near normal otoacoustic emissions, audiomet?ric thresholds and cochlear microphonic potentials, but abnormal or absent auditory brainstem response waveforms and cen?tral auditory processing deficits such as poor speech perception, difficulty hearing in noise and poor temporal processing. The pathological changes underlying auditory neuropathy are believed to originate from the inner hair cells-auditory nerve system and may involve missing inner hair cells, synaptic dysfunction between the inner hair cell and type I auditory neurons, and de?generation or demyelination of auditory nerve fibers that project to the cochlear nucleus. This paper describes various experi?mental animal models that have been developed to mimic certain aspects of auditory neuropathy. These include animal mod?els with missing inner hair cell induced by deletion of the Slc19a2 or Ggt1 genes, degeneration of type I afferent terminals by glutamate excitotoxicity, demyelination of auditory nerve fibers by adriamycin, hyperbilirubin and glycerin, surgical compres?sion of the auditory nerve, autoimmune diseases, and carboplatin-induced degeneration of inner hair cells and type I auditory nerve fibers in chinchillas. These different experimental models have provided unique insights of auditory function and simu?late different clinical manifestations and pathological features of auditory neuropathy.
Keywords：Auditory neuropathyEexperimental animal model
Publication Date：2015-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：8( 49-56 )