Ear malformation in a child with Goldenhar syndrome and its appropriate audiological management
DOI:
https://doi.org/10.23938/ASSN.1102Keywords:
Goldenhar Disease, . Audiology, Middle ear, External ear, ChildAbstract
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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JAYAPRAKASAN SK, WAHEED MD, BATOOL S, PIMENTEL CAMPILLO J, NAGEYE ME, HOLDER SS. Goldenhar syndrome: An atypical presentation with developmental and speech delay. Cureus 2023; 15(3): e36225. https://doi.org/10.7759/cureus.36225
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
BISDAS S, LENARZ M, LENARZ T, BECKER H. Inner ear abnormalities in patients with Goldenhar syndrome. Otol Neurotol 2005; 26(3): 398-404. https://doi.org/10.1097/01.mao.0000169796.83695.56
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
GOETZE TB, SLEIFER P, ROSA RF, DA SILVA AP, GRAZIADIO C, ZEN PR. Hearing characterization in oculoauriculovertebral spectrum: A prospective study with 10 patients. Am J Med Genet A 2017; 173(2): 309-314. https://doi.org/10.1002/ajmg.a.38017
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