A URAL A TRESIA IN N EWBORN H EARING S CREENING : A M ULTICENTRE S TUDY IN T URKEY Suren Basar F, Kirkim G, Gunduz B, Kulak Kayikci M, Genc A, Konukseven O, Gokdoğan C, Guven AG

I NTRODUCTION Aural atresia is a malformation of temporal bone. It can be seen as microtia, auricular aplasia, external ear canal atresia, middle and inner ear atresia.

Disorders of development involving the first and second branchial arches are encompassed by the term craniofacial microsomia (CFM). Aural atresia and microtia are characteristic findings in CFM.

P REVALENCE 1/6000 for every birth, 1/ – for serious auricular deformity

A IM The aim of this multicentric study is to define the percentage of auricular atresia in newborns screened by neonatal hearing screening programme.

M ATERIALS AND METHODS Retrospective study ( ) newborns 5 different centers.

C ENTERS CONTRIBUTED TO DATA ACQUISITION ; Ondokuz Mayis University, Samsun Dokuz Eylul University, İzmir Hacettepe University, Ankara Gazi University, Ankara Ataturk Research and Training Hospital, Ankara

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

A SSESSMENT 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

R ESULTS Average test age for unilateral aural atresia 22 days, Consanguinity 14/67 (20,8%) Family history 1/67 (1,4%)

R ESULTS 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%)

R ESULTS 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 /56 ears - mild or moderate SNHL

R ESULTS CONT.. Other anomalies; Cleft palate, 4 babies Trisomy 18, 1 baby Treacher Collins, 1 baby Multisyndromic, 1 baby o Average mother age - 27.

R ESULTS CONT.. 32 babies (47.7%) - CT scan 1 baby with bilateral atresia - bilateral Michel deformity 1 baby with unilateral atresia -bilateral SSCD

D ISCUSSION 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.

D ISCUSSION CONT.. The present series will underestimate the exact percentage of inner ear anomalies because CT scans are not routinely obtained in infants with aural atresia.

D ISCUSSION CONT.. Higher test age is related with the time spent between the primary and tertiary care centers

D ISCUSSION CONT.. The percentage 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 our country

D ISCUSSION CONT.. Unilateral hearing loss causes some difficulties like localization problems and diminisned speech understanding in noise. Babies with aural atresia must be followed frequently and rehabilitated as soon as possible

D ISCUSSION CONT.. 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.