Frequency of Profound Congenital Hearing Loss in Healthy Newborn Infants in Fars Province

Document Type : Original Article


1 Neonatal Research Centre, Department of Pediatrics, Shiraz U niversity of Medical Sciences, Shiraz , Iran

2 Medical student, Shiraz , Iran


Introduction 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.
Methods 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.
Results 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.
Conclusion 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.


1.Korver AM, Konings S, Dekker FW, Beers M, Wever CC, Frijns JH, et al. Newborn hearing screening vs later hearing screening and developmental outcomes in children with permanent childhood hearing impairment. JAMA 2010; 304:1701-8.
 2. McCann DC, Worsfold S, Law CM, Mullee M, Petrou S, Stevenson J, et al. Reading and communication skills after universal newborn screening for permanent childhood hearing impairment. Arch Dis Child 2009; 94:293-7.
3. Kennedy C, McCann D, Campbell MJ, Kimm L, Thornton R. Universal newborn screening for permanent childhood hearing impairment: an 8-year follow-up of a controlled trial. Lancet 2005; 366:660-2.
4. Erenberg A, Lemons J, Sia C, Trunkel D, Ziring P. Newborn and infant hearing loss: detection and intervention.American Academy of Pediatrics. Task Force on Newborn and Infant Hearing, 1998- 1999. Pediatrics 1999; 103:527-30.
5. Thompson DC, McPhillips H, Davis RL, Lieu TA, Homer CJ, Helfand M. Universal newborn hearing screening: summary of evidence. JAMA 2001; 286:2000-2010.
6. Atas B, Altunhan H, Eryilmaz MA, Atas E. Frequency of congenital hearing loss in 43,503 healthy newborn infants in Konya, Turkey. J Pak Med Assoc. 2011 Aug;61(8):727-8.
7. Ghasemi M, Zamanian A,Tale MR, Raufsaeb  A,  Farhadi M, Mahmoudian S. Neonatal hearing screening with TEOAE in Mashhad city.Iranian Journal of otorhinolaryngology 2006;18:21.
8. Farhadi M, Mahmoudian S, Mohammad K, Daneshi A.The pilot study of a nation wide neonatal hearing screening in Iran: Akbarabadi and Mirzakouchak-khan hospitals in Tehran( June 2003-October 2004).HAKIM  2006;9:65-75.
9. Lotfi Y, Movallali G. A universal newborn hearing screening in Iran. Iranian Rehabilitation Journal  2007;5:8-11.
10. Bielecki I, Horbulewicz A, Wolan T. Risk factors associated with hearing loss in infants: an analysis of 5282 referred neonates. Int J Pediatr Otorhinolaryngol 2011; 75:925.
11. Porter HL, Neely ST, Gorga MP. Using benefit-cost ratio to select Universal Newborn Hearing Screening test criteria. Ear Hear 2009; 30:447.
12.  Headley GM, Campbell DE, Gravel JS. Effect of neonatal test environment on recording transient-evoked otoacoustic emissions. Pediatrics 2000; 105:1279.
13. Stewart DL, Mehl A, Hall JW 3rd , Thomson V, Carroll M, Hamlett J.  Universal newborn hearing screening with automated auditory brainstem response: a multisite investigation. J Perinatol 2000; 20:S128.
14. Doyle KJ, Rodgers P, Fujikawa S, Newman E. External and middle ear effects on infant hearing screening test results. Otolaryngol Head Neck Surg 2000; 122:477.