Outcomes and Considerations of Cochlear Implantation in Patients With Incomplete Partition Type-III Malformation: A...

Cochlear implantation (CI) has been shown to be feasible and beneficial in patients with inner ear malformations (IEMs); however, incomplete partition type III (IP-III) malformations are rarely considered. In this scoping review, we evaluate the benefit of CI in patients with the relatively rare IP-III malformation.

A review in accordance with PRISMA was performed. A search of MEDLINE, EMBASE, Cochrane CENTRAL, CINAHL, and Web of Science was performed. Cohort studies, case studies, and conference abstracts evaluating complications or outcomes of CI in patients with confirmed IP-III malformation were included.

Our search identified a total of 40 studies after screening 1060 articles. Twenty-five studies (62.5%) reported CSF gusher as an intraoperative complication for a total of 120/122 patients (98.4%) in these studies. The most reported audiologic outcomes were speech perception testing with 10 studies, Categories of Auditory Performance (CAP) scores with 9 studies, and Speech Intelligibility Rating (SIR) with 8 studies. All 4 studies that directly compared IP-III malformation to other malformations using these outcome measures concluded that performance was statistically similar to at least one other malformation (100%). When compared with children with normal ears, only one-sixth of studies (16.7%) reported statistically similar results in IP-III malformation.

CSF gusher is an expected intraoperative complication of CI in patients with IP-III. Expectations regarding performance with CI in the setting of IP-III malformations should be tempered. Nonetheless, CI remains a viable option in patients with IP-III malformations with auditory outcomes comparable to those seen in other IEMs.