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Aural A tresia in N ewborn H earing S creening : a M ulticentr e S tudy in Turkey

Aural A tresia in N ewborn H earing S creening : a M ulticentr e S tudy in Turkey. S uren B asar F , K irkim G , G unduz B , K ulak K ayikci M , G enc A , Konukseven O , G okdoğan C , G uven AG. Introduction. Aural atresia is a malformation of temporal bone .

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Aural A tresia in N ewborn H earing S creening : a M ulticentr e S tudy in Turkey

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  1. Aural Atresia in Newborn Hearing Screening: a MulticentreStudy in Turkey Suren BasarF,Kirkim G, Gunduz B,Kulak Kayikci M, Genc A, Konukseven O, Gokdoğan C, Guven AG

  2. Introduction • Aural atresia is a malformation of temporal bone. • It can be seen as microtia, auricularaplasia, external ear canal atresia, middle and inner ear atresia.

  3. Disorders of development involving the first andsecond branchial arches are encompassed by the termcraniofacial microsomia (CFM). • Aural atresia andmicrotiaare characteristic findings in CFM.

  4. Prevalence • 1/6000 for every birth, • 1/10.000 – 20.000 forseriousauriculardeformity

  5. Aim • The aim of this multicentric study is to define the percentage of auricular atresia in newborns screened by neonatal hearing screeningprogramme.

  6. Materials and methods • Retrospective study (1998-2012) • 45124 newborns • 5 different centers.

  7. Centers contributed to data acquisition; • Ondokuz Mayis University, Samsun • Dokuz Eylul University, İzmir • Hacettepe University, Ankara • Gazi University, Ankara • Ataturk Research and Training Hospital, Ankara

  8. 67/45124 →0.15% 56 unilateral (83.5%) 11 bilateral (16.5%)

  9. Assessment criteria • Test age • Consanguinity • Family history • Prenatal history • Birth weight • Atretic ear • Hearing status in the other ear • Other anomalies • CT/MR results • Mother age

  10. Results • Average test agefor unilateral aural atresia 22 days, • Consanguinity 14/67 (20,8%) • Family history 1/67 (1,4%)

  11. Results cont.. • Prenatal history; 4/67 (5,9%) -Type 1 Diabetes -Antibiotic use (2 mothers) -Hx of hospitalization for high fever • Prematurity 7/67 (10,4%)

  12. Results cont.. • 56 unilateral atresia, Right ear (60,7% ) left ear (39,3%) 42 males (62,6%) , 25 females (37,4%) • Contralateral ears- TEOAE , AABR & behavioral audiometry. 5 /56ears - mild or moderate SNHL

  13. Results cont.. • Other anomalies; • Cleft palate, 4 babies • Trisomy 18, 1 baby • Treacher Collins, 1 baby • Multisyndromic, 1 baby • Average mother age - 27.

  14. Results cont.. • 32 babies(47.7%)-CT scan • 1baby with bilateral atresia - bilateral Michel deformity • 1 baby with unilateral atresia -bilateral SSCD

  15. Discussion • Assessment criteria and aural atresia revealed no significant relation with eachother. • Inner ear anomalies 2/32 (6,2%) were rarely seen in aural atresia • 51/56 ( 91%) contralateral ears have normal hearing and CT scans.

  16. Discussioncont.. • The present series will underestimate the exact percentage of innerearanomaliesbecauseCT scans are not routinely obtained in infants with aural atresia.

  17. Discussioncont.. • Higher test age is related with the time spent between the primary and tertiary care centers

  18. Discussioncont.. • Thepercentage of aural atresia is higher (0.15%) in our study than the literature. WHY? • Awareness is high in screening centers for babies who have atresia and they refer babies to the tertiary care centers • The study was conducted in tertiary screening centers in ourcountry

  19. Discussioncont.. • Unilateral hearing loss causessome difficulties like localization problems and diminisned speechunderstanding in noise. • Babies withaural atresia must be followed frequently and rehabilitated as soon as possible

  20. Discussioncont.. • Children with auricular atresia require global attention with respect to -early family guidence, -expected and unexpected hearing loss, -language development, -associated medical conditions and -both auricular and otologic reconstruction issues.

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