摘要/Abstract
摘要： 目的·探索中国内地首批人工听觉脑干植入（auditory brainstem implantation，ABI）在先天性耳聋低龄患儿中的安全性和有效性。方法·回顾性分析上海交通大学医学院附属第九人民医院耳鼻咽喉头颈外科2019年1月—4月实施ABI手术的连续先天性耳聋伴严重内耳和/或听神经畸形的低龄儿童病例，收集患儿听力学（助听下平均听阈，婴幼儿有意义听觉整合量表、听觉行为分级、有意义使用言语量表和言语可懂度分级得分）、影像学（CT、MRI）、手术记录、临床记录等资料，评估手术安全性及术后1年内听觉重建效果。结果·共有4例患儿纳入研究，手术时年龄为（26.3±7.8）月龄，均为双侧极重度感音神经性聋，1例为双侧共同腔畸形伴蜗神经未发育，3例为双侧Michel畸形伴蜗神经未发育。4例患儿均成功实施右侧ABI手术，手术操作时间（354.4±31.7）min，手术出血5～15 mL。开机时的激活电极数为（8.5±1.0）个。开机12个月后，激活电极数为（10.0±1.6）个，婴幼儿有意义听觉整合量表得分为（31.5±1.7）分，听觉行为分级为（4.3±0.8）分，有意义使用言语量表为（14.5±3.7）分，言语可懂度分级为2.0分，助听下平均听阈为（38.8±5.9）dBHL。结论·ABI手术在先天性耳聋低龄患儿中可安全实施，并可有效重建听力，促进言语发育。
关键词: 人工听觉脑干植入, 先天性耳聋, 低龄儿童
Abstract:
Objective · To investigate the safety and effectiveness of the first series of auditory brainstem implantation (ABI) in young children with congenital deafness in China mainland. Methods · The consecutive pediatric cases with congenital deafness and major malformations of inner ear and/or auditory nerve undergoing ABI surgery in the Department of Otolaryngology-Head and Neck Surgery, Shanghai Ninth People's Hospital, from January to April in 2019 were retrospectively studied. Their audiological data [aided pure tone average (PTA), scores of Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), Categories of Auditory Performance (CAP), Meaningful Use of Speech Scale (MUSS), and Speech Intelligibility Rating (SIR)], radiological data (CT and MRI), and operation and medical records were collected to evaluate the operation safety and hearing rehabilitation outcomes within 1 year after surgery. Results · Four children were included in the study with the age of (26.3±7.8) months at the time of surgery. These patients presented bilaterally profound sensorineural hearing loss. One case had bilateral common cavity malformations with cochlear nerves aplasia, and three had bilateral Michel malformations with cochlear nerves aplasia. All 4 patients were successfully implanted in right side; the pure operation time was (354.4±31.7) min with 5–15 mL hemorrhage. At the first mapping, the average number of active electrodes was 8.5±1.0. Twelve months later, the number of active electrodes was 10.0±1.6, IT-MAIS score was (31.5±1.7) score, CAP was (4.3±0.8) score, MUSS was (14.5±3.7) score, SIR was 2.0 score, and aided PTA was (38.8±5.9) dBHL. Conclusion · ABI can be safely operated in young children with bilateral profound hearing loss, and can effectively restore the hearing and promote speech development.
Key words: auditory brainstem implantation (ABI), congenital hearing loss, young children
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