title: Does microtia predict severity of temporal bone CT abnormalities in children with persistent conductive hearing loss?
abstract: OBJECTIVE
  This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia.
  
  
  METHODS
  From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience.
  
  
  RESULTS
  Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL.
  
  
  CONCLUSIONS
  Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes.
authors: Division of Pediatric Radiology, Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, MD 21287-0842, USA. atekes1@jhmi.edu
authors: Aylin
authors: A
authors: Tekes
authors: Stacey L
authors: SL
authors: Ishman
authors: Katherine M
authors: KM
authors: Baugher
authors: David J
authors: DJ
authors: Brown
authors: Sandra Y
authors: SY
authors: Lin
authors: David E
authors: DE
authors: Tunkel
authors: Aynur
authors: A
authors: Unalp-Arida
authors: Thierry A G M
authors: TA
authors: Huisman
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000000981:diagnostic imaging* / Q000503:physiopathology; D065817:Congenital Microtia; D004423:Ear / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D005260:Female; D006314:Hearing Loss, Conductive / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D011859:Radiography; D012189:Retrospective Studies; D013701:Temporal Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology
keywords: Children; Conductive hearing loss; Microtia; Temporal bone CT