Ear malformation in a child with Goldenhar syndrome and its appropriate audiological management

Authors

  • Andrés González Fernández Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain https://orcid.org/0000-0002-6260-5705
  • Manuela del Carmen Zapata Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain
  • José Zubicaray Ugarteche Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain https://orcid.org/0000-0001-5513-2172

DOI:

https://doi.org/10.23938/ASSN.1102

Keywords:

Goldenhar Disease, . Audiology, Middle ear, External ear, Child

Abstract

Goldenhar syndrome is a rare congenital disorder characterized by defects in the development of structures derived from the first and the second branchial arches. This condition encompasses a range of symptoms, including craniofacial, ocular, vertebral, and auricular abnormalities.

We present the case of a 6-year-old girl with right temporal bone hypoplasia and preauricular tag from birth, leading to a diagnosis of Goldenhar syndrome. She exhibited various middle and external ear defects, and her audiological treatment was crucial in ensuring optimal neurological and speech development. In adolescence, if the Eustachian tube remains stable, surgical repair of the ossicular chain may be considered.

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Author Biographies

Andrés González Fernández, Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain

Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain. https://ror.org/005dknr06

Manuela del Carmen Zapata, Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain

Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain. https://ror.org/005dknr06

José Zubicaray Ugarteche, Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain

Servicio Navarro de Salud-Osasunbidea. University Hospital of Navarra. Otolaryngology and Head and Nek Surgery Department. Pamplona. Spain. https://ror.org/005dknr06

References

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GUPTA S, SAMDANI S, VAISHNAV JK, SINGH Y, GROVER M. Cochlear implantation in Goldenhar syndrome. Indian J Otolaryngol Head Neck Surg 2022; 74(Suppl 3): 4159-4163. https://doi.org/10.1007/s12070-021-02874-5

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SLEIFER P, GORSKY NDES, GOETZE TB, ROSA RF, ZEN PR. Audiological findings in patients with oculo-auriculo-vertebral spectrum. Int Arch Otorhinolaryngol 2015; 19(1): 5-9. https://doi.org/10.1055/s-0034-1390137

ROOIJERS W, TIO PAE, VAN DER SCHROEFF MP, PADWA BL, DUNAWAY DJ, FORREST CR et al. Hearing impairment and ear anomalies in craniofacial microsomia: a systematic review. Int J Oral Maxillofac Surg 2022; 51(10): 1296-1304. https://doi.org/10.1016/j.ijom.2022.01.005

SKARZYŃSKI H, POROWSKI M, PODSKARBI-FAYETTE R. Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome). Int J Pediatr Otorhinolaryngol 2009; 73(7): 915-921. https://doi.org/10.1016/j.ijporl.2009.01.015

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Published

2025-02-13

How to Cite

1.
González Fernández A, del Carmen Zapata M, Zubicaray Ugarteche J. Ear malformation in a child with Goldenhar syndrome and its appropriate audiological management. An Sist Sanit Navar [Internet]. 2025 Feb. 13 [cited 2026 Feb. 14];48(1):e1102. Available from: https://recyt.fecyt.es/index.php/ASSN/article/view/110541

Issue

Section

Clinical notes

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