Abstract

(1) Background: Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells. When they originate from neural elements within the vestibule or cochlea, they are defined as intralabyrinthine schwannomas (ILSs). Cochlear implant (CI) has been reported as a feasible solution for hearing restoration in these patients. (2) Methods: Two patients with single-sided deafness (SSD) due to sudden sensorineural hearing loss and ipsilateral tinnitus were the cases. MRI detected an ILS. CI was positioned using a standard round window approach without tumor removal. (3) Results: The hearing threshold was 35 dB in one case and 30 dB in the other 6 mo after activation. Speech audiometry with bisillables in quiet was 21% and 27% at 65 dB, and the tinnitus was completely resolved or reduced. In the localization test, a 25.9° error azimuth was obtained with CI on, compared to 43.2° without CI. The data log reported a daily use of 11 h and 14 h. In order to not decrease the CI’s performance, we decided not to perform tumor exeresis, but only CI surgery to restore functional binaural hearing. (4) Conclusions: These are the sixth and seventh cases in the literature of CI in patients with ILS without any tumor treatment and the first with SSD. Cochlear implant without tumor removal can be a feasible option for restoring binaural hearing without worsening the CI’s performance.

Highlights

  • Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells or neurolemmocytes that support the neurons. They originate in the internal auditory canal (IAC) or in the cerebellopontine angle cistern (CPA), but they can occur anywhere along the nerve

  • In the rare case that these tumors originate primarily from the most terminal portion of the eight cranial nerves, they can be considered a distinct clinical-pathological disease defined as intralabyrinthine schwannomas (ILSs) [1]

  • We wanted to add to the scant literature on the topic some information about how to manage the hearing restoration in these patients, presenting the first clinical cases in the literature of patients with single-sided deafness that underwent cochlear implant without tumor removal

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Summary

Introduction

Schwannomas of the vestibulocochlear nerve are benign, slow-growing tumors, arising from the Schwann cells or neurolemmocytes that support the neurons. They originate in the internal auditory canal (IAC) or in the cerebellopontine angle cistern (CPA), but they can occur anywhere along the nerve. In the rare case that these tumors originate primarily from the most terminal portion of the eight cranial nerves (neural elements within vestibule, cochlea, or semicircular canals), they can be considered a distinct clinical-pathological disease defined as intralabyrinthine schwannomas (ILSs) [1]. We wanted to add to the scant literature on the topic some information about how to manage the hearing restoration in these patients, presenting the first clinical cases in the literature of patients with single-sided deafness that underwent cochlear implant without tumor removal

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