Cerebellopontine angle schwannomas arising from the intermediate nerve: a scoping review

Intermediate nerve schwannomas (INS) are extremely rare lesions in literature. They have been described mimicking facial nerve schwannomas, but not vestibular schwannomas (VS). We aimed to review the previously published cases, as well as the evidence to believe that they are far more common, though usually misdiagnosed as facial or VS. We performed a review of PubMed/Medline and Embase of "intermediate nerve schwannoma," "facial nerve schwannoma," "greater superficial petrosal nerve schwannoma," "geniculate ganglion schwannoma," and "chorda tympani schwannoma" to identify all cases of INS, following the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) statement. Furthermore, 2 cases operated at our center are shown to exemplify the proposed hypotheses. No article was excluded from review. Thirteen cases of INS, 11 cases of chorda tympani schwannoma, and 18 cases of greater superficial petrosal nerve schwannoma were found in literature. In facial nerve schwannomas, the predilection of schwannomas for sensory nerves, and the ability to preserve the motor facial nerve during tumor resection support the hypothesis of intermediate nerve as the nerve of origin. For VSs, the different arachnoidal arrangement of medial VS, the sharing of pia mater by the intermediate nerve and vestibular nerve, and the medial Obersteiner-Redlich zone of the intermediate nerve, support the hypothesis of intermediate nerve origin of some VS. The correct identification of the intermediate nerve as a nerve of origin of cerebellopontine angle schwannomas is of uttermost importance, especially when mistaken for VS, as this may account for the heterogeneity of facial and cochlear outcomes after surgery.