ABSTRACT
Deafness is one of the most important sensory disturbances at birth. Hearing loss can affect the development of speaking and learning during life. Early diagnosis and intervention improve language outcome. The current study aimed to determine the frequency of profound congenital hearing loss in healthy newborn infants in Fars province. In a multicenter prospective study, from August 2010 to August 2011, 12573 newborns were screened for hearing loss prior to discharge from the wellborn nursery at nine teaching and private hospitals in Fars province. A three-stage hearing screening protocol using transient evoked otoacoustic emissions [TEOAE] screening with referral for diagnostic second TEOAE and auditory brainstem evoked response [ABER] assessment was employed. All neonates with abnormal initial TEOAE screening, followed with phone call for language outcome after one year. Newborns with hospital admission or drug administration excluded from the study. The overall pass rate in the initial screening was 91.8%, thus 1019[8.1/1000] neonates referred for diagnostic audiological assessments. Out of 1019 infants scheduled for follow-up study, only 619 neonates returned. Using follow-up letters and phone calls, it was managed to improve the response rate for language outcome after one year. Two infants were identified with profound bilateral congenital hearing impairment. These infants were immediately referred for cochlear implantation. Findings of the current study showed that the frequency of profound congenital hearing loss is 0.159/1000 normal newborn infants in Fars province. The initial TEOAE has high false positive that may be due to occlusion of external ear canal by vernix in the early postnatal period