Cochlear implantation in Klippel Feil syndrome: pitfalls and outcomes
DOI:
https://doi.org/10.18203/issn.2454-5929.ijohns20251748Keywords:
Klippel-Feil syndrome, Cochlear implantation, Inner ear malformations, Cochlear anomalyAbstract
Cochlear implantation surgery involves multidisciplinary teamwork aiming for electrode insertion, which is as atraumatic as possible with an optimal intracochlear electrode position. Congenital inner ear anomalies often co-exist with musculoskeletal anomalies in syndromic children and can challenge cochlear implant surgery. The authors present a case of Klippel-Feil syndrome with congenital torticollis with bilateral inner ear anomalies who underwent cochlear implantation with multiple intra-operative surgical challenges. Multidisciplinary cooperation between the anesthetists, auditory-verbal therapy team, and the developmental pediatrics team is essential for a good outcome.
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Gruber J, Saleh A, Bakhsh W, Rubery PT, Mesfin A. The Prevalence of Klippel-Feil Syndrome: A Computed Tomography–Based Analysis of 2,917 Patients. Spine Deformity. 2018;6(4):448-53. DOI: https://doi.org/10.1016/j.jspd.2017.12.002
Stewart C, Otto AL, Fisher M, Niewchas A, Alkhatib S, Simonsen A, et al. Otolaryngological presentations of Klippel Feil syndrome: a systematic review. Cureus. 2024;16(11):73986. DOI: https://doi.org/10.7759/cureus.73986
Kenna MA, Irace AL, Strychowsky JE, Kawai K, Barrett D, Manganella J, et al. Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children. JAMA Otolaryngol Neck Surg. 2018;144(3):238–43. DOI: https://doi.org/10.1001/jamaoto.2017.2917
Reddy YM, Lepcha A, Augustine AM, Philip A. Cochlear implant in Klippel-Feil syndrome: challenges and concerns. Indian J Otolaryngol Head Neck Surg. 2021;74(3):3901. DOI: https://doi.org/10.1007/s12070-021-02728-0
M, Özkan H. Klippel-Feil syndrome and associated ear anomalies. Am J Otolaryngol. 2008;29(5):319–25. DOI: https://doi.org/10.1016/j.amjoto.2007.09.009
Manchanda S, Bhalla AS, Kumar R, Kairo AK. Duplication Anomalies of the Internal Auditory Canal: Varied Spectrum. Indian J Otolaryngol Head Neck Surg. 2017;71(3):294. DOI: https://doi.org/10.1007/s12070-017-1087-4
Aziz SSJA. A rare case of Klippel-Feil syndrome with cholesteatoma, thrice operated with final outcome of cochlear implant. IIUM Med J Malays. 2022;21(3):76. DOI: https://doi.org/10.31436/imjm.v21i3.1800
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