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Audiol > Volume 6(1); 2010 > Article
Audiology 2010;6(1): 93-97.
Published online: June 30, 2010.
doi: http://dx.doi.org/10.21848/audiol.2010.6.1.93
KID 증후군 아동의 인공와우이식 후 청능재활 사례
조수진1, 이미숙2
1대불대학교 언어치료청각학과
2지음청각언어연구소
Aural Rehabilitation after Cochlear Implantation in a Child with Keratitis-Ichthyosis-Deafness (KID) Syndrome
Soojin Cho1, Misook Lee2
1Department of Speech Pathology & Audiology, Daebul University, Yeongam, Korea
2Jeeum Auditory Rehabilitation Center, Daegu, Korea
Correspondence  Misook Lee ,Tel: (053) 471-8011, Fax: (053) 471-8013, Email: zzangms@jeeum.co.kr
Received: April 27, 2010; Revised: May 31, 2010   Accepted: June 7, 2010.  Published online: June 30, 2010.
ABSTRACT
Keratitis-ichthyosis-deafness (KID) syndrome is a rare ectodermal dysplasia characterized by vascularizing keratitis, profound sensorineural hearing loss (SNHL), and progressive erythrokeratoderma. It is a clinical triad that indicates a failure in development and differentiation of multiple stratifying epithelia. KID syndrome has been associated with mutations in the gene encoding connexin 26, a gap junction protein. The aim of this case review was to investigate auditory performances after cochlear implantation and emphasize the importance of auditory rehabilitation. The results of this case suggested that auditory performances and communication skills of a child with KID syndrome have improved through the cochlear implantation and aural rehabilitation. In particular listening tests of Listening Progress Profile (Lip Profile) and Meaningful Auditory Integration Scale (MAIS), the scores have improved from 52% to 97% and 20% to 80%, respectively. The results of this study suggested that auditory rehabilitation and cochlear implantation in children with (KID) syndrome can be effective and important.
Key Words: Aural rehabilitation·Cochlear implantation·Keratitis-ichthyosis-deafness (KID) syndrome.
중심단어: 인공와우이식· 청능재활· KID 증후군.
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