Hearing Loss and Language Delay in a Child with Goldenhar Syndrome: A Case Report with Literature Review

Mohamed Laachoubi *

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Bensimimou Salma

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Youssef Oukessou

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Sami Rouadi

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Redallah Abada

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Mohamed Roubal

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

Mohamed Mahtar

Otorhinolaryngology and Head and Neck Surgery Department, Faculty of Medicine and Pharmacy, IBN ROCHD University Hospital, Hassan II University, Casablanca, Morocco.

*Author to whom correspondence should be addressed.


Abstract

Goldenhar syndrome is a rare congenital disorder that involves the first and second branchial arches. It manifests mainly with asymmetric incomplete facial development, ear malformations, epibulbar dermoids and/or coloboma, and vertebral anomalies. It is characterized by a wide spectrum of signs and symptoms. Systemic anomalies may be associated. The etiology is still unclear. Ear malformations and hearing loss are very common. Early identification of auricular abnormalities is crucial in order to prevent secondary language and cognitive developmental delays. The purpose of this case report is to describe the clinical presentation of Goldenhar syndrome in a 4-year-old female child who presented with language delay and to discuss the diagnosis and treatment of ear abnormalities and hearing loss.

Keywords: Goldenhar syndrome, ear malformations, hearing loss


How to Cite

Laachoubi , M., Salma , B., Oukessou , Y., Rouadi, S., Abada , R., Roubal , M., & Mahtar , M. (2023). Hearing Loss and Language Delay in a Child with Goldenhar Syndrome: A Case Report with Literature Review. Asian Journal of Case Reports in Surgery, 6(2), 374–378. Retrieved from https://journalajcrs.com/index.php/AJCRS/article/view/436

Downloads

Download data is not yet available.

References

Goldenhar M. Associations malformatives de l’oeil et l’oreille, en particulier le syndrome dermoide epibulbaire-appendices auriculaires-fistula auris congenita et ses relations avec la dysostose mandibulo-faciale. J Genet Hum. 1952;1:243–282.

Lam CH. A theory on the embryogenesis of oculo-auriculo-vertebral (Goldenhar) syndrome. J Craniofac Surg. 2000; 11(6):547-52

Monahan R, Seder K, Patel P, Alder M, Grud S, O'Gara M. Hemifacial microsomia. Etiology, diagnosis and treatment. J Am Dent Assoc. 2001;132( 10):1402-8. DOI: 10.14219/jada.archive.2001.005

Hartsfield JK. Review of the etiologic heterogeneity of the oculo-auriculo-vertebral spectrum (Hemifacial Microsomia). Orthod Craniofac Res. 2007; 10: 121-8.

Anna EF, Lau GW, Marquezan M, de Souza Araújo MT, Polley JW, Figueroa AA. Combined maxillary and mandibular distraction osteogenesis in patients with hemifacial microsomia. Am J Orthod Dentofacial Orthop. 2015;147(5):566-77.

Goswami M, Bhushan U, Jangra B. Goldenhar Syndrome: A Case Report with Review. International Journal of Clinical Pediatric Dentistry. 2016;9(3):278–280

Senggen E, Laswed T, Meuwly JY, Maestre LA, Jaques B, Meuli R, et al. First and second branchial arch syndromes: multimodality approach. Pediatr Radiol 2011;41(4):549-61.

Ferrari F, D'Orazio F, Patriarca L, Piccorossi A, Di Fabio S, Barile A, et al. A Female Case of Goldenhar Syndrome with Mandibular Hypoplasia and Aural Involvement. British Journal of Medicine & Medical Research. 2015;11:8

Relhan V, Mittal S, Mahajan K, Garg VK. Goldenhar syndrome with rare clinical features. Indian J Paediatr Dermatol. 2017: 18(4):317-20

Salmon MA, Lindenbaum RH. Developmental Defects and Syndromes. London: HMM Publishers. 1978;40.

Ferrari F, Orazio F, Patriarca L, Piccorossi A, Fabio S, Barile A, et al. A Female Case of Goldenhar Syndrome with Mandibular Hypoplasia and Aural Involvement. Br J Med Med Res 2016; 11(8): 1–5.

Gorlin RJ, Cohen Jr MM, Hennekam RCM. Syndromes of the head and neck 4th ed. Am J Med Genet. 2002; 113:312.

Schmitzer S, Burcel M, D_asc_alescu D, et al. Goldenhar Syndrome -ophthalmologist’s perspective. Rom J Ophthalmol. 2018; 62:96e104.

Rooijers W, Tio PAE, van der Schroeff MP, Padwa BL, Dunaway DJ, Forrest CR, Koudstaal MJ, Caron CJJM. Hearing impairment and ear anomalies in craniofacial microsomia: A systematic review. Int. J. Oral Maxillofac. Surg. 2022; 51:1296–1304.

Mandelbaum RS, Volpicelli EJ, Martins DB, Park SH, Dubina E, Ishiyama A, Bradley JP, Lee JC. Evaluation of 4 outcomes measures in microtia treatment: exposures, infections, aesthetics, and psychosocial ramifications. Plast Reconstr Surg Glob Open 2017;5: e1460.

Skarzynski H, Porowski M, Podskarbi-Fayette R. Treatment of otological features of the oculoauriculovertebral dysplasia (Goldenhar syndrome). Int J Pediatr Otorhinolaryngol. 2009; 73:915–21.

Renkema RW, the ERN CRANIO Working Group on Craniofacial Microsomia. European guideline craniofacial microsomia. J Craniofac Surg 2020;31 (Suppl 8):2385–484.