Abstract
Intralabyrinthine schwannomas (ILS) are a rare differential diagnosis of sudden hearing loss and vertigo. In an own case series of 12 patients, 6 tumors showed an intracochlear, 3 an intravestibular, 1 a transmodiolar including the cerebellopontine angle (CPA), 1a transotic including the CPA, and 1 a multilocular location. The tumors were removed surgically in 9 patients, whereas 3 patients decided for a “wait-and-test-and-scan” strategy. Of the surgical patients, 3 underwent labyrinthectomy and cochlear implant (CI) surgery in a single-stage procedure; 1 patient had extended cochleostomy with CI surgery; 3 underwent partial or subtotal cochleoectomy, with partial cochlear reconstruction and CI surgery (n = 1) or implantation of electrode dummies for possible later CI after repeated MRI follow-up (n = 2); and in 2 patients, the tumors of the internal auditory canal and cerebellopontine angle exhibiting transmodiolar or transmacular growth were removed by combined translabyrinthine–transotic resection. For the intracochlear tumors, vestibular function could mostly be preserved after surgery. In all cases with CI surgery, hearing rehabilitation was successful, although speech discrimination was limited for the case with subtotal cochleoectomy. Surgical removal of intracochlear schwannomas via partial or subtotal cochleoectomy is, in principle, possible with preservation of vestibular function. In the authors’ opinion, radiotherapy of ILS is only indicated in isolated cases. Cochlear implantation during or after tumor resection (i. e., as synchronous or staged surgeries) is an option for hearing rehabilitation in cartain cases and represents a therapeutic approach in contrast to a “wait-and-test-and-scan” strategy.
Highlights
Definition and classificationIntralabyrinthine schwannomas (ILS) are benign neoplasms originating from the peripheral branches of the cochlear nerve or the inferior or superior vestibular nerves
Because of their location and their management, they are a special subtype of vestibular schwannomas that typically occur in the internal auditory canal (IAC) and in the cerebellopontine angle (CPA)
The tumors may be found in the cochlea, in the vestibule, or growing through the fundus into the IAC even up to the CPA
Summary
Intralabyrinthine schwannomas (ILS) are benign neoplasms originating from the peripheral branches of the cochlear nerve or the inferior or superior vestibular nerves. Because of their location and their management, they are a special subtype of vestibular schwannomas (acoustic neuromas) that typically occur in the internal auditory canal (IAC) and in the cerebellopontine angle (CPA). The tumors may be found in the cochlea, in the vestibule, or growing through the fundus into the IAC even up to the CPA They may grow from the inner ear into the middle ear and can present in different combinations of the aforementioned locations. There are several classifications, e. g., according to Jackler [11], who differentiates between “schwannoma of the vestibule”
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