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Keywords:

  • Genetic hearing impairment;
  • cochlear implantation;
  • auditory rehabilitation;
  • cochlear implant outcome

Abstract

Objectives:

The purpose of this study is to determine if the performance of the first implanted member of a family predicts the performance of subsequently implanted family members.

Study Design:

Retrospective chart review.

Methods:

Seventy-one cochlear implant recipients, each belonging to a family with two or more implanted members, were the subjects of this study. Routine audiometric measurements and age-appropriate speech perception tests were performed pre- and postoperatively. In addition, length of deafness, age at implantation, etiology, and length of device usage were correlated to outcome.

Results:

All implant recipients within a family showed improvement postimplantation, and the predictive component between family members was strong. Presence of GJB2 mutations and greater age at implantation were predictive of poorer rehabilitative outcome, while length of device usage was associated with improved speech perception scores.

Conclusions:

On average, if the first implanted family member performs well with a cochlear implant, those following will do well; however, GJB2-related deafness and increased age at implantation are associated with poorer outcome. These findings have important implications for members of families considering cochlear implantation. Laryngoscope, 119:131–136, 2009