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Published on: February 29, 2020
[Audiological evaluation of a child with Klippel-Feil syndrome]
Günay Kirkim1, Taner Kemal Erdağ, Mustafa Bülent Serbetçioğlu
1Department of Otolaryngology, Medicine Faculty of Dokuz Eylül University, Izmir, Turkey. gunay.kirkim@deu.edu.tr
Insights
Children with Klippel-Feil syndrome (KFS) often experience hearing loss due to ear anomalies. Early audiological and otologic evaluations are crucial for managing KFS-related hearing impairment.
Area of Science:
- Otolaryngology
- Genetics
- Pediatrics
Background:
- Klippel-Feil syndrome (KFS) is a congenital disorder characterized by the fusion of cervical vertebrae.
- KFS can be associated with various physical anomalies, including those affecting the auditory system.
Observation:
- A 6-year-old boy with KFS presented with hearing loss and speech disorder.
- Clinical and CT imaging revealed bilateral external and middle ear anomalies.
- Audiological assessment indicated severe bilateral conductive hearing impairment.
Findings:
- The patient was fitted with a bone conduction hearing aid.
- Further consultations identified additional physical anomalies.
- The case highlights the link between KFS and auditory system malformations.
Implications:
- Routine audiological and otologic screening is recommended for all children diagnosed with KFS.
- Multidisciplinary consultations are essential for comprehensive patient care.
- Informing parents about potential complications aids in proactive management and follow-up.
Abstract:
A 6-year-old boy with formerly diagnosed Klippel-Feil syndrome (KFS) presented with complaints of hearing loss, speech disorder, and problems related to his hearing aid. Clinical examination and computed tomography showed bilateral external and middle ear anomalies and audiological examination revealed bilateral severe conductive hearing impairment. The patient was prescribed a bone conduction hearing aid and, with necessary consultations, was examined for additional physical anomalies. All children with a diagnosis of KFS should be evaluated with audiologic and otologic examinations, consultations should be implemented with other disciplines concerning the presenting problems, a regular follow-up should be scheduled, and the parents should be informed on possible complications.
