Abstract: Objective To study on the effect of ganciclovir on the hearing in the infants with cytomegalovirus(CMV) infection and Connexin 26 gene mutations,and its role in improving the hearing impairment of such infants. Methods The infants with positive CMV-DNA in blood and urine were screened.RT-PCR and DNA sequencing were performed and Connexin 26 gene mutations were found in 70 infants.22 infants with hepatic injury were assigned into Group Ⅰ(22 cases,receiving ganciclovir treatment),while 48 infants without liver function damage were divided into ganciclovir treatment group(Group Ⅱ,24 cases) and non ganciclovir treatment group (Group Ⅲ,24 cases).The treatment induced the induction dose of 5 mg/kg Ganciclovir,q12h,continuous 14 days,and then changed to QD maintenance therapy with same dose,continuous 7 days.The liver function index and CMV-DNA copy number were re-detected.The changes of brainstem auditory evoked potential were tracked.The liver function,CMV-DNA copy number and brain stem evoked potential between the two groups were compared. Results After ganciclovir treatment,the liver function in Group Ⅰ was improved obviously(ALT:t=8.610 9,P<0.000 1,AST:t=15.007 7,P<0.000 1,TBil:t=10.993 3,P<0.000 1),CMV-DNA copy number in both Group Ⅰ and Group Ⅱ decreased significantly(t=5.460 4,P<0.000 1),but the hearing impairment by BAEP was improved,even worse in some children,while the infant hearing impairment in Group Ⅲ had no significant deterioration. Conclusion Ganciclovir treatment is effective on the hepatitis caused by cytomegalovirus infection and other inflammatory diseases,but cannot improve the sensorineural hearing loss(neurosensory deafness),especially the hearing impairment associated with Connexin 26 gene mutation in infants.