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Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities.
Otol Neurotol. 2014 Jun; 35(5):826-30.ON

Abstract

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.

Authors+Show Affiliations

*Department of Diagnostic and Interventional Neuroradiology, Hoppe-Seyler-Straβe 3; and †Department of Otorhinolaryngology, Elfriede-Aulhorn-Straβe 5, Tübingen, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24686288

Citation

Hennersdorf, Florian, et al. "Temporal Bone Changes in Patients With Goldenhar Syndrome With Special Emphasis On Inner Ear Abnormalities." Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, vol. 35, no. 5, 2014, pp. 826-30.
Hennersdorf F, Friese N, Löwenheim H, et al. Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities. Otol Neurotol. 2014;35(5):826-30.
Hennersdorf, F., Friese, N., Löwenheim, H., Tropitzsch, A., Ernemann, U., & Bisdas, S. (2014). Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities. Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 35(5), 826-30. https://doi.org/10.1097/MAO.0000000000000278
Hennersdorf F, et al. Temporal Bone Changes in Patients With Goldenhar Syndrome With Special Emphasis On Inner Ear Abnormalities. Otol Neurotol. 2014;35(5):826-30. PubMed PMID: 24686288.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Temporal bone changes in patients with Goldenhar syndrome with special emphasis on inner ear abnormalities. AU - Hennersdorf,Florian, AU - Friese,Natascha, AU - Löwenheim,Hubert, AU - Tropitzsch,Anke, AU - Ernemann,Ulrike, AU - Bisdas,Sotirios, PY - 2014/4/2/entrez PY - 2014/4/2/pubmed PY - 2015/1/31/medline SP - 826 EP - 30 JF - Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology JO - Otol Neurotol VL - 35 IS - 5 N2 - 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. SN - 1537-4505 UR - https://www.unboundmedicine.com/medline/citation/24686288/Temporal_bone_changes_in_patients_with_Goldenhar_syndrome_with_special_emphasis_on_inner_ear_abnormalities_ L2 - https://doi.org/10.1097/MAO.0000000000000278 DB - PRIME DP - Unbound Medicine ER -