The Building Of AIF Conditional Knock-out Mice And The Application In Deafness Diseases Such As Auditory Neuropathy

Auditory neuropathy(AN) is a specific kind of clinical common diseaseof hearing loss. The main characters of the disease include the change of the auditory brainstem responses(ABR), or the outcome is absent or abnormal. And the normal outcome of the evoked otoacoustic emissions(EOAE), the function of which reflects the function of the inner and outer hair cells.We know that Starr first named it as AN in 1996, and then it interested a lot of doctors to study on this special disease of hearing.The results of the uditory tests in AN patients,was distinguished from other sensorineural hearing loss.This also include theabsenceor threshold elevation of the acoustic reflex(AR).The mainly elevation of the pure tone auditory threshold test is at lower frequencies. And the abnormal electronystagram(ENG) and/or vestibular evoked myogenic potentials(VEMP).The speech discrimination disability is the main complain of AN patients, and the patients are difficulty in verbal communication, especially in noises. However, we don’t know the effective treatment of AN, and we still don’t know the causes and changes in the course of the hearing loss.Tow years ago, we followed up a bunch of adult AN patients.And we analysed the changes in auditory tests of the patients. We want to know the natural history and the prognosis of the AN disease.Many disease can call for the causes in some congenital AN patients, but we don’t know more about the posteriority AN patients’ clear history, such as the risk factors at all.We detected Some AN patients with genes mutations, but we didn’t know if the genes’ variations is the cause of the AN patients. Thus, we designed the AIF Conditional Knock-out Mice to explore if the patients carry variations in related genes. This research are expected to reveal new auditory neuropathy genes and their mechanism of deafness, will h elp provide reliable genetic diagnosis and genetic counseling for families and patients, and could provides the possible prevention and treatment thought for clinicians.