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