Gumus Birgul, Incesulu Armagan, Pinarbasli Mehmet Ozgur
Department of Otorhinolaryngology, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey.
Case Rep Otolaryngol. 2017;2017:3913187. doi: 10.1155/2017/3913187. Epub 2017 Oct 2.
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.
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.
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.
角膜炎-鱼鳞病-耳聋（KID）综合征是一种伴有听力丧失以及视觉和角化障碍的综合征。在此类患者中，由于外耳道频繁被上皮碎屑堵塞以及外耳道皮肤干燥紧绷，助听器无法有效用于听力损失的康复。此外，重度或极重度听力损失也限制了传统助听器的益处。另一方面，人工耳蜗植入是文献中仅在有限病例中使用过的一种方法。
本研究展示了在我们诊所对两名被诊断为KID综合征的儿童进行人工耳蜗植入的结果。人工耳蜗植入手术前后的听力学评估采用纯音听力测定、声导抗测听和听觉脑干反应（ABR），术后随访采用纯音听力测定。
皮肤问题、视觉障碍和其他附加问题使KID综合征患者植入后的短期和长期康复变得复杂。由于术后可能出现皮肤并发症，应进行密切监测。应向家庭和康复团队警示可能出现的视觉障碍和皮肤并发症。
