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<p>To determine the incidence of syndromic associations among children undergoing cochlear implantation (CI) and to analyse associated temporal bone anomalies, intraoperative surgical challenges, postoperative auditory outcomes, and parental satisfaction. <i>Design</i>: Prospective observational study. <i>Setting</i>: Department of ENT, Central Hospital, South Eastern Railway, a tertiary care centre in Eastern India. <i>Subjects</i>: A total of 139 children aged 1–7 years who underwent CI between June 2017 and June 2023 were included; those with prior middle ear surgery were excluded. Ethics approval and parental consent were obtained. <i>Methodology</i>: All children underwent detailed clinical and multidisciplinary evaluation. Radiological evaluation comprised HRCT temporal bone with 3D reconstruction and MRI brain. Syndromic diagnosis was primarily clinical, supported by imaging and genetic testing where feasible. All surgeries were performed using the standard posterior tympanotomy approach. Intraoperative challenges and modifications were documented. Postoperatively, all children received Auditory Verbal Therapy (AVT) and were followed up at 6, 12, and 24 months. Auditory outcomes were assessed using CAP and SIR scores; the Friedman test was used for analysis (<i>p</i> &lt; 0.05). Parental satisfaction was recorded. The incidence of syndromic associations was 4.32%. Syndromes included Nager syndrome, Waardenburg syndrome, CHARGE syndrome, Susac syndrome, and two cases of Congenital Rubella Syndrome. Anatomical variations included facial nerve anomalies, enlarged vestibular aqueduct, malformed or rotated cochlea, and narrowed internal auditory canal. Surgical challenges included anticipated perilymph gusher, limited posterior tympanotomy requiring canal wall dislocation, and difficulty identifying the round window, necessitating cochleostomy. One child developed delayed postoperative facial nerve palsy, which improved with conservative management. CAP and SIR scores improved significantly over time (<i>p</i> &lt; 0.001), in all children by 24 months. All parents reported satisfactory outcomes. Syndromic association alone should not be considered a contraindication to CI when guided by detailed imaging and structured rehabilitation.</p>

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Syndromic Associations in Paediatric Cochlear Implantation: Incidence, Anatomical Variations, Surgical Challenges, and Outcomes from a Tertiary Care Centre in Eastern India

  • 期刊论文

  • 发表日期：

    2026年7月6日

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  • Kailash Prasad Verma,
  • Ambily Sahadevan,
  • Athul Anand,
  • R. Amith,
  • Akshay Jain

摘要

To determine the incidence of syndromic associations among children undergoing cochlear implantation (CI) and to analyse associated temporal bone anomalies, intraoperative surgical challenges, postoperative auditory outcomes, and parental satisfaction. Design: Prospective observational study. Setting: Department of ENT, Central Hospital, South Eastern Railway, a tertiary care centre in Eastern India. Subjects: A total of 139 children aged 1–7 years who underwent CI between June 2017 and June 2023 were included; those with prior middle ear surgery were excluded. Ethics approval and parental consent were obtained. Methodology: All children underwent detailed clinical and multidisciplinary evaluation. Radiological evaluation comprised HRCT temporal bone with 3D reconstruction and MRI brain. Syndromic diagnosis was primarily clinical, supported by imaging and genetic testing where feasible. All surgeries were performed using the standard posterior tympanotomy approach. Intraoperative challenges and modifications were documented. Postoperatively, all children received Auditory Verbal Therapy (AVT) and were followed up at 6, 12, and 24 months. Auditory outcomes were assessed using CAP and SIR scores; the Friedman test was used for analysis (p < 0.05). Parental satisfaction was recorded. The incidence of syndromic associations was 4.32%. Syndromes included Nager syndrome, Waardenburg syndrome, CHARGE syndrome, Susac syndrome, and two cases of Congenital Rubella Syndrome. Anatomical variations included facial nerve anomalies, enlarged vestibular aqueduct, malformed or rotated cochlea, and narrowed internal auditory canal. Surgical challenges included anticipated perilymph gusher, limited posterior tympanotomy requiring canal wall dislocation, and difficulty identifying the round window, necessitating cochleostomy. One child developed delayed postoperative facial nerve palsy, which improved with conservative management. CAP and SIR scores improved significantly over time (p < 0.001), in all children by 24 months. All parents reported satisfactory outcomes. Syndromic association alone should not be considered a contraindication to CI when guided by detailed imaging and structured rehabilitation.