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Case Reports
. 2017:2017:3913187.
doi: 10.1155/2017/3913187. Epub 2017 Oct 2.

Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

Birgul Gumus  1 , Armagan Incesulu  1 , Mehmet Ozgur Pinarbasli  1
Affiliations
Case Reports

Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

Birgul Gumus et al. Case Rep Otolaryngol. 2017.

Abstract

Background: Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature.

Case report: This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry.

Conclusion: Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

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Figures

Figure 1
Figure 1
Hearing thresholds with CI in the 3rd month for Case 1.
Figure 2
Figure 2
Hearing thresholds with CI in the 1st year for Case 1.
Figure 3
Figure 3
Hearing thresholds with CI in the 1st year for Case 2.
See this image and copyright information in PMC

References

    1. Barker E. J., Briggs R. J. S. Cochlear implantation in children with keratitis-ichthyosis-deafness (KID) syndrome: Outcomes in three cases. Cochlear Implants International. 2009;10(3):166–173. doi: 10.1002/cii.374. - DOI - PubMed
    1. Choung Y.-H., Shin Y. R., Kim H. J., et al. Cochlear implantation and connexin expression in the child with keratitis-ichthyosis-deafness syndrome. International Journal of Pediatric Otorhinolaryngology. 2008;72(6):911–915. doi: 10.1016/j.ijporl.2008.02.023. - DOI - PubMed
    1. Arndt S., Aschendorff A., Schild C., et al. A novel dominant and a de novo mutation in the GJB2 gene (Connexin-26) cause keratitis-ichthyosis-deafness syndrome: Implication for cochlear implantation. Otology and Neurotology. 2010;31(2):210–215. doi: 10.1097/MAO.0b013e3181cc09cd. - DOI - PubMed
    1. Karaduman A. Kalıtsal keratinizasyon bozuklukları. Turkderm. 2011;45(2):73–80.
    1. Hampton S. M., Toner J. G., Small J. Cochlear implant extrusion in a child with keratitis, ichthyosis and deafness syndrome. Journal of Laryngology and Otology. 1997;111:465–467. - PubMed

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