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Temporal Bone Changes in Patients With Goldenhar Syndrome With Special Emphasis on Inner Ear Abnormalities

Hennersdorf, Florian*; Friese, Natascha; Löwenheim, Hubert; Tropitzsch, Anke; Ernemann, Ulrike*; Bisdas, Sotirios*

doi: 10.1097/MAO.0000000000000278
Pediatric Otology

Objective Goldenhar syndrome is a developmental disorder presenting with orofacial and vertebral anomalies, which are also accompanied by abnormalities in other organs. We examined temporal bone changes with special emphasis on inner ear abnormalities in these patients.

Study Design A retrospective review of 7 new cases in addition to a previously published series of 14 cases with clinically diagnosed Goldenhar syndrome was carried out to search for inner ear anomalies. In addition, temporal bone imaging studies from the literature were summarized and compared with our results.

Setting Departments of Neuroradiology and Otorhinolaryngology at a university hospital.

Patients In addition to the previous series of 14 patients, 7 new patients with Goldenhar syndrome were identified.

Interventions Patients underwent otologic examination, audiometric studies, and high-resolution computed tomography (CT) or magnetic resonance imaging (MRI) of the temporal bone.

Main Outcome Measure Temporal bone changes and specifically inner ear malformations.

Results Nineteen of 21 patients showed changes of the external and middle ear correlating with the literature. Seven of 21 patients showed inner ear abnormalities constituting one-third of all patients. These ranged from mild such as vestibular enlargement to severe defects such as cochlear hypoplasia and common cavity.

Conclusion Inner ear abnormalities were present in one-third of patients. Although in some cases, these might not be of clinical significance, some patients show severe defects of the inner ear requiring more complex hearing loss therapy. Therefore, imaging of the temporal bone structures is important in the care of these patients.

*Department of Diagnostic and Interventional Neuroradiology, Hoppe-Seyler-Straße 3; and †Department of Otorhinolaryngology, Elfriede-Aulhorn-Straße 5, Tübingen, Germany

Address correspondence and reprint requests to Sotirios Bisdas, M.D, Ph.D., Department of Diagnostic and Interventional Neuroradiology, Hoppe-Seyler-Straße 3, 72076 Tübingen Germany; E-mail:

The authors disclose no conflicts of interest.

Copyright © 2014 by Otology & Neurotology, Inc. Image copyright © 2010 Wolters Kluwer Health/Anatomical Chart Company