start-ver=1.4
cd-journal=joma
no-vol=70
cd-vols=
no-issue=8
article-no=
start-page=1343
end-page=1349
dt-received=
dt-revised=
dt-accepted=
dt-pub-year=2006
dt-pub=20068
dt-online=
en-article=
kn-article=
en-subject=
kn-subject=
en-title=
kn-title=Using assessment of higher brain functions of children with GJB2-associated deafness and cochlear implants as a procedure to evaluate language development
en-subtitle=
kn-subtitle=
en-abstract=
kn-abstract=<p><b>Objective:</b> While investigators have reported that patients with GJB2-associated deafness and cochlear implants have preferable language development, the mechanisms of this phenomenon remains unknown. The goat of the present study was to assess higher brain functions of patients with GJB2-retated and GJB2-unrelated deafness as a method of evaluating language development.<br />
<b>Methods:</b> Eight children with cochlear implants were subjected to genetic testing for GJB2 and underwent the Raven colored progressive matrices test, Rey's auditory verbal learning test, Rey's complex figure test, the standardized Language test for aphasia, the picture vocabulary test, and the standardized comprehension test for abstract words.<br />
<b>Results:</b>Three children were diagnosed with GJB2-related deafness, and five children were diagnosed with GJB2-unrelated deafness. All three GJB2-related cases demonstrated normal range higher brain functions and fair language development. By contrast, one GJB2-unrelated case showed a semantic disorder, another demonstrated a visual cognitive disorder with dyslexia, and another had attention deficit-hyperactivity disorder.<br />
<b>Conclusions:</b>Children with GJB2-unrelated deafness showed a high frequency of heterogeneous disorders that can affect proper language development. This difference between children with GJB2-retated and GJB2-unrelated deafness may account for the improved language development in children with GJB2-related deafness and cochlear implants. Further, genetic diagnosis of the non-syndromic hearing toss represents a useful tool for the preoperative prediction of outcomes following a cochlear implant procedure.</p>
en-copyright=
kn-copyright=

en-aut-name=KawasakiAkihiro
en-aut-sei=Kawasaki
en-aut-mei=Akihiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=1
ORCID=

en-aut-name=FukushimaKunihiro
en-aut-sei=Fukushima
en-aut-mei=Kunihiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=2
ORCID=

en-aut-name=KataokaYuko
en-aut-sei=Kataoka
en-aut-mei=Yuko
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=3
ORCID=

en-aut-name=FukudaShoichiro
en-aut-sei=Fukuda
en-aut-mei=Shoichiro
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=4
ORCID=

en-aut-name=NishizakiKazunori
en-aut-sei=Nishizaki
en-aut-mei=Kazunori
kn-aut-name=
kn-aut-sei=
kn-aut-mei=
aut-affil-num=5
ORCID=

affil-num=1
en-affil=
kn-affil=Okayama University

affil-num=2
en-affil=
kn-affil=Okayama University

affil-num=3
en-affil=
kn-affil=Okayama University

affil-num=4
en-affil=
kn-affil=Okayama University

affil-num=5
en-affil=
kn-affil=Okayama University
en-keyword=cochlear implant
kn-keyword=cochlear implant
en-keyword=higher brain function
kn-keyword=higher brain function
en-keyword=GJB2
kn-keyword=GJB2
en-keyword=language development
kn-keyword=language development
en-keyword=non-syndromic hearing loss
kn-keyword=non-syndromic hearing loss
en-keyword=learninng disability
kn-keyword=learninng disability
END