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.

Background: In most cases, intralabyrinthine schwannoma (ILS) occurs in patients with unilateral hearing deterioration or neurofibromatosis type II (NF II). The pattern of localization of these tumors varies but mostly affects the cochlea. Extirpation of the cochlear schwannoma, if hidden by the cochlea modiolus, is difficult under the aspect of complete removal. Therefore, a tissue removal device (TRD) was designed and tested in temporal bones. The principle of handling the new device is a pushing and pipe cleaner handling inside the cochlea. This present study aimed to describe the first in vivo experience with the newly developed TRD for removing cochlear intralabyrinthine schwannomas. Methods: In three patients, the TRD was used for the tumor removal of cochlear schwannomas. In two patients with a cochlear schwannoma in combination with a cochlea implantation and one patient suffering from NF II, a cochlear schwannoma was removed with the TRD. The access was performed with a posterior tympanotomy, an enlarged round window approach and an additional second turn access. The device was inserted and extracted gradually from the second turn access until the rings were visible in the second turn access. By pushing and pipe cleaner handling, the tumors were removed. An MRI control was performed on the day postoperatively with a T1 GAD sequence. Results: Tumor removal with the TRD was performed in a 15-min procedure without any complications. An MRI control confirmed complete removal on the postoperative day in all cases. Conclusions: In vivo handling of the device confirmed straightforward handling for the tumor removal. MRI scanning showed complete removal of the tumor by the TRD.

OBJECTIVE: To evaluate the literature and summarize cochlear implantation (CI) outcomes after intralabyrinthine schwannoma (ILS) excision and tumor observation with CI.
METHODS: OVID Medline, Embase, Web of Science; conception to 2024.
METHODS: A literature review was performed using subject headings, MeSH terms, and keywords. Abstracts and full texts were reviewed by two independent reviewers and adjudicated by a third. Inclusion criteria included studies with ILS and CI with reported audiologic outcomes. Subjects were analyzed into two groups, ILS resection with CI and in situ ILS with CI. Patients with NF2 were included. The main outcome of interest was CI audiometric performance level, with secondary outcomes of CI user status and open-set speech attainment.
RESULTS: There were 29 articles with a total of 93 patients who met inclusion criteria. The resection group had 17% low performers, 44% intermediate performers, and 38% high performers. The in situ group had 40% low performers, 32% intermediate performers, 27% high performers. The resection group had 69 patients with 97% maintaining user status and 92% with open-set speech recognition. The observation group had 24 patients, with 87% user rate and 86% achieving open-set speech recognition. There was a greater percentage of NF2 diagnosis seen in the in situ group.
CONCLUSIONS: There is a paucity of literature on CI and ILS. Patients are managed with both resection of tumor and implantation in situ. Early data are limited, with improvement in hearing outcomes and high user rates in both populations.
METHODS: N/A Laryngoscope, 134:3910-3920, 2024.

Intralabyrinthine schwannomas (ILSs) are rare tumors involving the otic capsule. Notably, they are often misdiagnosed because their symptoms mimic those of other, more common inner ear pathologies. Diagnosis requires high-resolution contrast-enhanced magnetic resonance imaging (MRI), which reveals filling defects (using a T2-weighted MRI sequence) or focal enhancement (using a T1-weighted MRI sequence with gadolinium enhancement) in the inner ear. A 52-year-old male patient with intractable vertigo or single-sided deafness should raise suspicion of this clinical entity as a differential diagnosis. Translabyrinthine excision of the tumor along with auditory rehabilitation using a cochlear implant can provide good outcomes with minimal morbidity in carefully selected cases. Here, we present an interesting case of a transmodiolar ILS mimicking Meniere\'s disease, wherein surgery using the translabyrinthine approach and an extended cochleostomy yielded favorable outcomes.

The present video reports the surgical removal of an intralabyrinthine schwannoma. The video contains patient\'s medical history, preoperative radiological evaluations and detailed description of surgical steps of the procedure, consisting in labyrinthectomy, cochleostomy and insertion of a dummy electrode in the preserved cochlear lumen within the context of a subtotal petrosectomy.

Intralabyrinthine schwannomas (ILS) are rare benign tumors, often responsible for hearing loss. MRI is important in establishing the diagnosis. We present the example of a 48-year-old lady who reported a 3-years history of right-sided sensorineural deafness. MRI demonstrated a loss of the normal hypersignal of the second turn of the right cochlea compatible with intracochlear schwannoma.

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature.

Intracochlear schwannoma is very rare, and complete loss of hearing is inevitable after the removal of this tumor. Here, we discuss cochlear implantation (CI) performed simultaneously with the removal of an intracochlear schwannoma.
Retrospective single-center study.
Tertiary medical institute.
Simultaneous CI and intracochlear schwannoma removal were performed in 4 subjects. After subtotal cochleostomy, the tumors were removed meticulously, with preservation of the modiolus. A new slim modiolar electrode (Nucleus CI632) was placed in a manner that hugged the modiolus. The surgical outcomes of functional gain, word recognition score (WRS), sound localization, and hearing in noise and speech intelligibility tests were investigated.
Intracochlear schwannomas were removed successfully from the 4 patients, with no remnant tumor. The mean aided hearing threshold 6 months after surgery was 25.0 ± 1.8 dB, and the mean-aided WRS with a 60 dB stimulus was 36.0 ± 18.8% (range 16%-60%). The Categorical Auditory Performance (CAP) score of the 3 single-sided deafness patients under contralateral ear masking was 7. The CAP score of the patient with bilateral sensorineural hearing loss was 6, which improved from a preoperative score of 0.
When an intracochlear schwannoma does not completely invade the modiolus, CI with simultaneous tumor removal can be performed successfully, resulting in good hearing performance. A slim modiolar electrode can be placed stably at the modiolus after schwannoma removal.

OBJECTIVE: Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme.
METHODS: We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones.
RESULTS: ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation.
CONCLUSIONS: Our case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing.

We present the case of a 65 years old patient who developed a complete, sensorineural hearing loss on the right side due to an intravestibular schwannoma. Our video shows a transcanal, endoscopic approach with complete schwannoma removal on the right side, subsequent e-BERA recordings, and cochlear implantation.