Facial canal bifurcation with inner ear anomalies in a case of Klippel-Feil syndrome: A case report

Research output: Contribution to journalArticle

Abstract

We present a case of Klippel-Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel-Feil syndrome.

Original languageEnglish
Pages (from-to)32-35
Number of pages4
JournalInternational Journal of Pediatric Otorhinolaryngology Extra
Volume8
Issue number2
DOIs
Publication statusPublished - Mar 2013

Fingerprint

Klippel-Feil Syndrome
Inner Ear
Vestibulocochlear Nerve
Bilateral Hearing Loss
Cochlear Implantation
Temporal Bone
Facial Nerve
Tomography
Magnetic Resonance Imaging
Brain

Keywords

  • Facial canal anomaly
  • Inner ear malformation
  • Internal acoustic canal stenosis
  • Klippel-Feil syndrome

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Pediatrics, Perinatology, and Child Health

Cite this

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title = "Facial canal bifurcation with inner ear anomalies in a case of Klippel-Feil syndrome: A case report",
abstract = "We present a case of Klippel-Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel-Feil syndrome.",
keywords = "Facial canal anomaly, Inner ear malformation, Internal acoustic canal stenosis, Klippel-Feil syndrome",
author = "Tehrani, {S. J T} and {Mohd Zaki}, Faizah and Kew, {Thean Yean} and Asma Abdullah",
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AU - Abdullah, Asma

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N2 - We present a case of Klippel-Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel-Feil syndrome.

AB - We present a case of Klippel-Feil syndrome in a 2-year-old Chinese girl who presented with global developmental delay and bilateral profound hearing loss. High resolution computed tomography (HRCT) of temporal bones showed bilateral common cavity malformations with hypoplastic internal auditory canals and anomalous labyrinthine segments of both facial canals. The labyrinthine segment of the right facial canal was duplicated. Her brain magnetic resonance imaging (MRI) showed aplasia of both vestibulocochlear nerves, while the cisternal and intracanalicular segments of both facial nerves were of normal caliber. These congenital anomalies, coupled with global developmental delay, precluded her candidacy for cochlear implantation. This case report is aimed at highlighting the association of inner ear malformations and in particular facial canal anomalies in Klippel-Feil syndrome.

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