Cochlear implantation in adults and pediatrics with enlarged vestibular aqueduct: a systematic review on the surgical findings and patients' performance

Purpose: Cochlear implantation (CI) has been considered a safe and effective management option for patients with severe to profound hearing loss. Patients with enlarged vestibular aqueduct (EVA) could be challenging with some variations in surgical approaches, intraoperative surgical notes, and clinical outcomes. This study aimed to review the surgical and clinical outcomes of cochlear implantation among patients with EVA.

Materials and methods: A systematic literature search was carried out in five major databases. All original studies reporting cochlear implantation in patients with EVA were included for qualitative data synthesis. The risk of bias was independently assessed through the National Intuitional of Health tool. The review protocol was registered in PROSPERO (reference number: CRD42021225900).

Results: A total of 34 studies with 4035 subjects were included. Of them, 853 (21.14%) had EVA and underwent CI. Mondini malformation was the most frequently associated anomaly (n = 78, 11.1%). Unilateral implantation was performed in 258 cases while bilateral in 119 subjects. Postoperative complications included CSF/perilymph gusher (n = 112), CSF oozing (n = 18), and partial electrode insertion (n = 6). Closing the cochleostomy with temporalis fascia, muscle, connective tissue, or fibrin glue was the most frequently reported approach to manage CSF/perilymph gusher (n = 67, 56.7%) while packing was performed in six patients.

Conclusion: Patients with EVA demonstrated audiometric and speech performance improvement after CI. However, many patients had intra- or postoperative complications. Further research is needed as the outcomes may be affected by associated temporal bone pathology, the timing of implant, and hearing condition.