Inner ear abnormalities in patients with Goldenhar syndrome.
OBJECTIVE
The objective of this study is to investigate the inner ear malformations in patients with Goldenhar syndrome and to hypothesize the potential embryopathogenesis of these malformations.


METHODS
Retrospective case review.


METHODS
Tertiary referral center.


METHODS
Fourteen patients with Goldenhar syndrome.


METHODS
Each patient underwent hearing tests and high-resolution computed tomography (CT) of the temporal bone. In six patients, magnetic resonance imaging of the temporal bone also was performed.


RESULTS
Among the 14 patients with Goldenhar syndrome, 13 had outer and middle ear anomalies and 5 (36%) had inner ear malformations, including one case of common cavity.


CONCLUSIONS
Our observations regarding inner ear anomalies in Goldenhar syndrome correlate with the reported cases in the literature and may help to hypothesize the embryological origin of these malformations, which can caused by an early developmental arrest in the fourth gestational week. Specialists evaluating patients with Goldenhar syndrome should be aware of the possibility of inner ear malformations, which could be diagnosed earlier with appropriate imaging studies.
Bisdas
Sotirios
S
Department of Neuroradiology, Hannover Medical School, Hannover, Germany. sbisdas@yahoo.com
Lenarz
Minoo
M
Lenarz
Thomas
T
Becker
Hartmut
H
D000328:Adult; D002648:Child; D002675:Child, Preschool; D000013:Congenital Abnormalities / Q000175:diagnosis; D004431:Ear, External / Q000002:abnormalities; D007758:Ear, Inner / Q000002:abnormalities*; D004432:Ear, Middle / Q000002:abnormalities; D005260:Female; D006053:Goldenhar Syndrome / Q000150:complications*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed