Preservation of function is an important goal during surgical management of cochleovestibular schwannomas. We here demonstrate the relief of vertigo and the preservation of function of all five vestibular receptors after removal of an intracochlear schwannoma with extension to the fundus of the internal auditory canal. A 61-year-old male with a five-year history of left-sided deafness, tinnitus, vertigo attacks, and an MRI consistent with an intracochlear schwannoma with limited extension through the modiolus to the fundus of the internal auditory canal (IAC) underwent transcanal, transcochlear total tumor removal and-due to a cerebrospinal fluid leak from the fundus of the IAC-revision surgery with lateral petrosectomy and blind sac closure of the external auditory canal. Despite complete removal of the cochlear partition of the inner ear (total cochlectomy), the patient\'s vestibular receptors remained functional, and the vertigo symptoms disappeared. These results show that vestibular labyrinthine function may not only be preserved after partial or subtotal cochlectomy but also after complete cochlear removal. This further confirms the vestibular labyrinth\'s robustness and encourages surgical management of transmodiolar schwannomas with limited extension to the fundus of the IAC.

A primary intracochlear schwannoma (ICS) is a unique type of vestibular schwannoma (VS); the tumor originates from the terminal branches of the cochlear nerve and is confined to the cochlea. An ICS is the most common subtype of schwannoma in the inner ear. As an ICS is clinically rare, diagnosis and treatment remain challenging. We report a rare case of cochlear implantation (CI) in a patient with neurofibromatosis type 2 and an ICS. The patient exhibited bilateral, profound, sensorineural hearing loss. The tumor on one side was a common VS treated via tumor and acoustic nerve resection and that on the other side an ICS. To ensure auditory rehabilitation via CI, we performed CI while removing part of the ICS via an enlarged round window. Auditory rehabilitation was satisfactory. Thus, ICS patients, especially those who urgently require auditory rehabilitation, can undergo simultaneous CI and (total or partial) tumor removal. However, the long-term results require close observation.