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Summary: ORIGINAL ARTICLE
Connexin-Associated Deafness and Speech
Perception Outcome of Cochlear Implantation
Riki Taitelbaum-Swead, MA; Zippora Brownstein, PhD; Chava Muchnik, PhD; Liat Kishon-Rabin, PhD;
Jona Kronenberg, MD; Lela Megirov, MD; Moshe Frydman, MD; Minka Hildesheimer, PhD; Karen B. Avraham, PhD
Objective: To compare performance after cochlear im-
plantation in children with mutations in connexin (Cx)
26 (GJB2) or Cx30 (GJB6) and children with deafness
of unknown etiology.
Design: Genetic analysis and speech perception evalu-
ation was performed in the children with and without
Cx mutations who had undergone cochlear implanta-
tion. Speech perception performance was retrospec-
tively analyzed 6, 12, 24, 36, and 48 months after im-
plantation. Test material was selected according to the
child's age and cognitive and language abilities.
Setting: The study took place at speech and hearing and
genetic centers of a hospital in the central part of Israel
and the genetics departments of 3 additional centrally lo-
cated hospitals.
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