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.

Objective: Hearing loss is a common symptom associated with vestibular schwannoma (VS), either because of the tumor's effects on the cochlear nerve or due to active treatments such as surgery or stereotactic radiosurgery (SRS). Treatment decisions for VS are based on factors including tumor size, hearing status, patient symptoms, and institutional preference. The study aimed to investigate long‐term auditory outcomes in VS patients undergoing active treatments with a hearing preservation intent.Data Sources: A systematic literature review was conducted following Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guidelines, searching Scopus, Pubmed, and Web of Science databases from inception to January 2024.Review Methods: Studies meeting inclusion criteria, including a minimum 5‐year follow‐up and assessment of pre‐ and posttreatment hearing outcomes, were included. Pooled prevalence estimates for serviceable hearing after SRS and microsurgery were calculated using MetaXL software. Risk of bias assessment was performed with the Risk of Bias in Non‐randomized Studies of Interventions tool.Results: Nine studies met the inclusion criteria, with 356 patients included for analysis. The pooled prevalence of maintaining serviceable hearing after SRS at 10 years was 18.1% (95% confidence interval [CI]: 1.7%‐43.3%), with wide prediction intervals indicating variability in outcomes. Microsurgery demonstrated a higher prevalence of maintaining long‐term serviceable hearing, with a pooled estimate of 74.5% (95% CI: 63.5%‐84.1%).Conclusion: This systematic review underscores the importance of long‐term follow‐up in evaluating auditory outcomes in VS treatment. Despite the biases inherent to pretreatment patients selection, hearing preservation microsurgery for sporadic VS removal demonstrated favorable and stable long‐term serviceable hearing.

Objective. To report surgical outcomes of a cohort of small sporadic vestibular schwannoma and discuss the role of early surgery. Methods. Retrospective descriptive analysis of 234 consecutive patients with small vestibular schwannoma operated on with translabyrinthine or hearing preservation surgical approaches. Outcome measures were control of disease, postoperative facial nerve function, complications and hearing outcome. Results. A translabyrinthine approach was performed in 59% of cases, hearing preservation surgery in 40% and transmeatal approach in 1%, respectively. Complete resection was achieved in 100% of cases. Overall, postoperative major and minor complications were 8.5% in the series, with complete recovery and no sequalae. Facial nerve function at one year postoperatively was House-Brackmann scale (HB) 1-2 in 95% of cases. The rate of hearing preservation was 70% A-B-C classes of Tokyo classification and 77% AB classes of AAOHNS classification. Conclusions. Early surgery in small vestibular schwannoma is a valid option, due to good functional outcomes, low morbidity and definitive cure. Early surgery is associated with better outcomes when considered in relation to non-surgical treatments reported in the literature.

Objective: to evaluate recent contributions to the literature on prognostic factors of hearing preservation in small vestibular schwannoma microsurgery. Methods: review of the most recent studies. Results: factors such as tumor size, preoperative hearing status, tumor growth rate, tumor origin, surgical approach, radiological characteristics, results of preoperative neurophysiological tests, preoperative symptoms and demographic features have been investigated and some of them reported to be significant in the prediction of hearing preservation. Conclusions: tumor size and preoperative hearing status are the most impactful factors and play a key role in patient selection for hearing preservation surgery. Other features such as fundal extension, tumor origin and impaired ABR could have prognostic value on hearing preservation. Tumor growth rate, preoperative impedance, cVEMPs and age have also recently been found to be significant, but more studies are needed. The role of preoperative tinnitus, vertigo and gender is lacking and controversial, whereas the differences between available surgical approaches have been smoothed out in recent years.

Objectives: The objectives were to present the real-time estimated values of cochlear implant (CI) electrode insertion speed (IS) during intraoperative sessions using the Cochlear Nucleus SmartNav System to assess whether this measure affected CI outcomes and to determine whether real-time feedback assists expert surgeons in achieving slow insertion. Methods: The IS was measured in 52 consecutive patients (65 implanted ears) using the CI632 electrode. The IS values were analyzed in relation to procedure repetition over time, NRT ratio, and CI audiological outcomes. Results: The average IS was 0.64 mm/s (SD = 0.24); minimum and maximum values were 0.23 and 1.24 mm/s, respectively. The IS significantly decreased with each array insertion by the operator (p = 0.006), and the mean decreased by 24% between the first and last third of procedures; however, this reduction fell within the error range of SmartNav for IS (+/−0.48 mm/s). No correlation was found between IS and the NRT ratio (p = 0.51), pure-tone audiometry (PTA) at CI activation (p = 0.506), and PTA (p = 0.94) or word recognition score (p = 0.231) at last evaluation. Conclusions: The estimated IS reported by SmartNav did not result in a clinically significant reduction in insertion speed or an improvement in CI hearing outcomes. Real-time feedback of IS could potentially be used for training, but its effectiveness requires confirmation through additional studies and more accurate tools. Implementation of IS assessment in clinical practice will enable comparisons between measurement techniques and between manual and robot-assisted insertions. This will help define the optimal IS range to achieve better cochlear implant (CI) outcomes.

Background: Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review. Methods: A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations. Results: A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment. Conclusions: The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount.