The outcome of cochlear implantation among children with genetic syndromes



To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes.


Study design: case–control study.


A cochlear implantation tertiary referral center.


All pediatric cochlear implantation recipients with Waardenburg syndrome, Usher syndrome, Dandy–Walker syndrome, or albinism. A control group was appropriately matched to the syndromic group with regard to age at implantation and duration of device use.


Cochlear implantation.

Main outcome measures

Subjects’ auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group.


A total of 25 subjects (13 syndromic and 12 non-syndromic) participated in the study. Neither auditory ability nor speech intelligibility scores differed significantly by group. The final PTA of both the groups showed normal-to-mild hearing loss: 26 dB HL in the syndromic group and 23 dB HL for the control group.


Cochlear implant recipients with genetic syndromes achieved similar levels auditory perception and speech intelligibility as their peers with a genetic syndrome. The presence of any of the genetic syndromes described herein should not be a contraindication to cochlear implant provision, as it would have a positive impact on the patients’ sensory perception and lifestyle.

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Corresponding author

Correspondence to Farid Alzhrani.

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The authors declare that they have no competing interests.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Alzhrani, F., Alhussini, R., Hudeib, R. et al. The outcome of cochlear implantation among children with genetic syndromes. Eur Arch Otorhinolaryngol 275, 365–369 (2018).

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  • Cochlear implant
  • Waardenburg syndrome
  • Usher syndrome
  • Dandy–Walker syndrome
  • Albinism
  • Deafness
  • Hearing loss