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European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
Feb, 2018;275 (2): 365-369.

The outcome of cochlear implantation among children with genetic syndromes.

Farid Alzhrani, Rayan Alhussini, Rawan Hudeib, Tuqa Alkaff, Tahera Islam, Abdulrahman Alsanosi
Author Information
  1. Farid Alzhrani: King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, PO Box 245, Riyadh, 11411, Saudi Arabia. faalzhrani@ksu.edu.sa. ORCID
  2. Rayan Alhussini: King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, PO Box 245, Riyadh, 11411, Saudi Arabia.
  3. Rawan Hudeib: King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, PO Box 245, Riyadh, 11411, Saudi Arabia.
  4. Tuqa Alkaff: College of Medicine, King Saud University, Riyadh, Saudi Arabia.
  5. Tahera Islam: College of Medicine and Research Center, King Saud University, Riyadh, Saudi Arabia.
  6. Abdulrahman Alsanosi: King Abdullah Ear Specialist Center (KAESC), College of Medicine, King Saud University, PO Box 245, Riyadh, 11411, Saudi Arabia.
PMID: 29204917 DOI: 10.1007/s00405-017-4832-0

Abstract

OBJECTIVE: To assess the outcome and efficacy of cochlear implantation in children with genetic syndromes.
METHOD: Study design: case-control study.
SETTING: A cochlear implantation tertiary referral center.
PATIENTS: 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.
INTERVENTION: Cochlear implantation.
MAIN OUTCOME MEASURES: Subjects' auditory abilities, speech intelligibility, and pure tone thresholds were compared between the syndromic and non-syndromic group.
RESULTS: 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.
CONCLUSIONS: 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.

Keywords

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

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MeSH Term

Albinism
Case-Control Studies
Child
Child, Preschool
Cochlear Implantation
Dandy-Walker Syndrome
Female
Follow-Up Studies
Humans
Infant
Male
Speech Intelligibility
Speech Perception
Treatment Outcome
Usher Syndromes
Waardenburg Syndrome
Journal Article

Word Cloud

Created with Highcharts 10.0.0syndromeimplantationgroupcochleargeneticsyndromessyndromicCochlearauditoryspeechintelligibilityimplantoutcomechildrenstudyrecipientsWaardenburgUshercontrolnon-syndromicHLperceptionOBJECTIVE:assessefficacyMETHOD:Studydesign:case-controlSETTING:tertiaryreferralcenterPATIENTS:pediatricDandy-WalkeralbinismappropriatelymatchedregardagedurationdeviceuseINTERVENTION:MAINOUTCOMEMEASURES:Subjects'abilitiespuretonethresholdscomparedRESULTS:total25subjects1312participatedNeitherabilityscoresdifferedsignificantlyfinalPTAgroupsshowednormal-to-mildhearingloss:26 dB23 dBCONCLUSIONS:achievedsimilarlevelspeerspresencedescribedhereincontraindicationprovisionpositiveimpactpatients'sensorylifestyleamongAlbinismDandy–WalkerDeafnessHearingloss

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