Skip to main content

Advertisement

SpringerLink
Log in

Cochlear Implantation in Goldenhar Syndrome

  • Clinical Report
  • Published:
Indian Journal of Otolaryngology and Head & Neck Surgery Aims and scope Submit manuscript

Abstract

Goldenhar syndrome is a rare genetic condition characterized by hemifacial microsomia, mandibular hypoplasia, auricular malformations, and epibulbar dermoids. The syndrome has both sporadic and familial occurrence. Incidence of congenital hearing loss in these patients is 1:1000 in children with a male to female ratio of 3:2. In our case study we report a case of Goldenhar Syndrome who underwent cochlear implantation. The patient had right side microtia, right hemifacial microsomia and right side torticollis, pterygium in her right eye, right hypoplastic thumb and unilateral right side kidney. Radiologically, there was narrow duplicated internal auditory canal on right side with absent right cochlear nerve with normal anatomy on left side and the left side showed malformed facial nerve at tympani segment and second genu. Therefore, the patient was planned for left side cochlear implantation. Intraoperatively, there were malformed ossicles with anomalous facial nerve covering whole of oval window and partially the round window. Thus, a separate cochleostomy was done. Impedance was < 5 Hertz in all electrodes and electrically evoked action potential (ECAP) thresholds were obtained on all electrodes.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Andrews TM, Shott SR (1992) Laryngeal manifestations of goldenhar syndrome. Am J Otol 13:312–315

    Article  CAS  Google Scholar 

  2. Cullins SL, Pridjian G, Sutherland CM (1994) Goldenhar’s syndrome associated with tamoxifen given to the mother during gestation. JAMA 271:1905–1906

    Article  CAS  Google Scholar 

  3. Lessick M, Vasa J, Israel R (1991) Severe manifestations of oculoauriculovertebralspectrum in a cocaine-exposed infant. J Med Genet 28:803–804

    Article  CAS  Google Scholar 

  4. Tuna EB, Orino D, Ogawa K, Yildirim M, Seymen F, Gencay K et al (2011) Craniofacial and dental characteristics of Goldenhar Syndrome: A report of two cases. J Oral Sci 53:121–124

    Article  Google Scholar 

  5. Bisdas S, Lenarz M, Lenarz T, Becker H (2005) Inner ear abnormalities in patients with goldenhar syndrome. OtolNeurotol 26(3):398–404

    Google Scholar 

  6. Phelps PD, Lloyd GA, Poswillo DE (1983) The ear deformities in craniofacial microsomia and oculo-auriculo-vertebral dysplasia. J LaryngolOtol 97(11):995–1005

    Article  CAS  Google Scholar 

  7. Carvalho GJ, Song CS, Vargervik K, Lalwani AK (1999) Auditory andfacial nerve dysfunction in patients with hemifacialmicrosomia. Arch Otolaryngol Head Neck Surg 125(2):209–12

    Article  CAS  Google Scholar 

  8. Sleifer P, GorskyNdeS Goetze TB, Rosa RF, Zen PR (2015) Audiologicalfindings in patients with oculo-auriculo-vertebral spectrum. Int Arch Otorhinolaryngol 19(1):5–9

    Google Scholar 

  9. Yanagihara N, Yanagihara H, Kabasawa I (1979) Goldenhar’s syndromeassociated with anomalous internal auditory meatus. J LaryngolOtol 93(12):1217–1222

    Article  CAS  Google Scholar 

  10. Scholtz AW, Fish JH, Kammen-Jolly K, Ichiki H, Hussl B, KreczyA Schrott-Fischer A (2001) Goldenhar’s syndrome: congenital hearingdeficit of conductive or sensorineural origin temporal bone histopathologyicstudy. Otol Neurotol 22:501–5

    Article  CAS  Google Scholar 

  11. Caldarelli DD, Hutchinson JG, Pruzansky S, Valvassori GE (1980) A comparison of microtia and temporal bone anomalies in hemifacial microsomia and mandibulofacial dysostosis. Cleft Palate J 17(2):103–110

    CAS  Google Scholar 

  12. Cousley RRJ, Calvert ML (1997) Current concepts in the understandingand management of hemifacial microsomia. Br J Plast Surg. https://doi.org/10.1016/S0007-1226(97)91303-5

    Article  Google Scholar 

  13. Grabb WC (1965) The first and second branchial arch syndrome. Plast Reconstr Surg 36:485–508

    Article  CAS  Google Scholar 

  14. Durcan DJ, Shea JJ, Sleeckx JP (1967) Bifurcation of the facialnerve. Arch Otolaryngol. https://doi.org/10.1001/archotol.1967.00760050621005

    Article  Google Scholar 

  15. Van Meter TD, Weaver DD (1996) Oculo-auriculo-vertebral spectrumand the CHARGE association: clinical evidence for acommon pathogenetic mechanism. Clin Dysmorphol 5(3):187–196

    Google Scholar 

  16. Castellino A, Rayamajhi P, Kurkure R, Kameswaran M (2020) Posterior semi-circular canal electrode misplacement in Goldenhar’s syndrome. Cochlear Implants Int. https://doi.org/10.1080/14670100.2020.1802905

    Article  Google Scholar 

  17. Skarzyński H, Porowski M, Podskarbi-Fayette R (2009) Treatment ofotological features of the oculoauriculovertebral dysplasia (Goldenharsyndrome). Int J Pediatr Otorhinolaryngol 73(7):915–921

    Article  Google Scholar 

  18. MacArdle BM, Bailey CM, Phelps PD, Bradley J, Brown T, Wheeler A (2002) Cochlear implants in children with craniofacial syndromes: Assessment and outcomes: Implantescoclearesenniños con síndromescraneofaciales: evaluación y resultados. Int J Audiol 41(6):347–356

    Article  CAS  Google Scholar 

  19. Umashankar A, Jayachandran D (2019) Measurement of cochlear implant outcomes in goldenhar syndrome: a case report. Indian J Case Reports 5(5):448–450

    Article  Google Scholar 

  20. Bouhabel S, Arcand P, Saliba I (2012) Congenital aural atresia boneanchoredhearing aid vs. external auditory canal reconstruction. Int J Pediatr Otorhinolaryngol 76(2):272–277

    Article  Google Scholar 

  21. Santarelli G, Redfern RE, Benson AG (2015) Bone-anchored hearing aidimplantation in a patient with goldenhar syndrome. Ear Nose Throat J 94(12):E1-3

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Otorhinolaryngology and Head and Neck Surgery, SMS Medical College and Hospital, Jaipur, India

    Shubhangi Gupta, Sunil Samdani, Yogendra Singh & Mohnish Grover

  2. Department of Otorhinolaryngology and Head and Neck Surgery, RVRS Medical College, Bhilwara, India

    Jairaj Kumar Vaishnav

Authors
  1. Shubhangi Gupta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sunil Samdani
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jairaj Kumar Vaishnav
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yogendra Singh
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mohnish Grover
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Mohnish Grover.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Gupta, S., Samdani, S., Vaishnav, J.K. et al. Cochlear Implantation in Goldenhar Syndrome. Indian J Otolaryngol Head Neck Surg 74 (Suppl 3), 4159–4163 (2022). https://doi.org/10.1007/s12070-021-02874-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12070-021-02874-5

Keywords

Search

Navigation