Alexa N. Pearce, Soroush Farsi, Carissa C. Saadi, Alaina D. Baggett, John L. Dornhoffer, Robert A. Saadi
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引用次数: 0
Abstract
Objective
Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare congenital disorder characterized by vascularizing keratitis, hyperkeratosis, and profound sensorineural hearing loss. Skin debris and chronic otitis may render conventional hearing aids ineffective, and visual impairment may preclude use of sign language. This study assesses the unique challenges and outcomes associated with cochlear implantation (CI) in this patient population.
Data sources
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol, PubMed, Web of Science, and Embase were queried from each database's inception to present day (November 2024) for articles describing CI outcomes in KID syndrome patients.
Review methods
Studies were examined for participant demographics, age at time of CI, lateralization of CI, previous hearing aid use, CI model, and follow-up length. A qualitative synthesis of post-operative complications and long-term outcomes was employed for all studies.
Results
Of 224 abstracts initially identified, 9 met the inclusion criteria, comprising 13 patients and 17 ears. Notable complications included postoperative infection (30.8 %, n = 4) and superficial wound dehiscence (15.4 %; n = 2). One patient required revision surgery with local tissue rearrangement, and one other patient was offered revision due to complications but refused. Ten ears (58.8 %) had very limited or no complications after CI. The average hearing improvement was 45 dB (28 dB–60 dB).
Conclusion
CI in patients with KID syndrome presents distinctive challenges but is a safe and effective treatment option. Close monitoring of the postoperative incision is advised to expediently manage any wound healing issues, but long-term results appear to be successful in most cases.
目的:角膜炎-鱼鳞病-耳聋(KID)综合征是一种罕见的先天性疾病,其特征为血管性角膜炎、角化过度和深度感音神经性听力损失。皮肤碎片和慢性中耳炎可能使传统的助听器失效,视力障碍可能妨碍使用手语。本研究评估了该患者群体中与人工耳蜗植入(CI)相关的独特挑战和结果。根据系统评价和荟萃分析协议的首选报告项目,PubMed, Web of Science和Embase从每个数据库的建立到现在(2024年11月)查询描述KID综合征患者CI结果的文章。研究检查了参与者的人口统计学特征、CI时的年龄、CI偏侧、助听器使用史、CI模型和随访时间。所有研究均采用术后并发症和长期结果的定性综合。结果初步鉴定的224篇摘要中,9篇符合纳入标准,包括13例患者和17只耳朵。并发症包括术后感染(30.8%,n = 4)和浅表创面裂开(15.4%,n = 2)。1例患者需要局部组织重排翻修手术,另1例患者因并发症接受翻修但拒绝。10耳(58.8%)CI术后并发症非常有限或无并发症。平均听力改善45 dB (28 dB - 60 dB)。结论ci治疗KID综合征具有独特的挑战性,是一种安全有效的治疗方法。建议密切监测术后切口,以方便处理任何伤口愈合问题,但长期结果似乎在大多数情况下是成功的。
期刊介绍:
The purpose of the International Journal of Pediatric Otorhinolaryngology is to concentrate and disseminate information concerning prevention, cure and care of otorhinolaryngological disorders in infants and children due to developmental, degenerative, infectious, neoplastic, traumatic, social, psychiatric and economic causes. The Journal provides a medium for clinical and basic contributions in all of the areas of pediatric otorhinolaryngology. This includes medical and surgical otology, bronchoesophagology, laryngology, rhinology, diseases of the head and neck, and disorders of communication, including voice, speech and language disorders.