BACKGROUND: Vestibular schwannomas - benign tumours originating from the vestibular nerve - are rare during pregnancy. The intricate interplay between the gravid uterus, maternal physiology and neoplastic growth imposes complexities that demand a careful and tailored approach.
METHODS: This article reports a case of a pregnant woman in her 30 s diagnosed with a large vestibular schwannoma exhibiting brainstem compression, peritumoral oedema and cranial nerve encasement at 36 + 5 weeks of gestation. A multi-disciplinary team collaborated to devise a treatment plan considering the delicate balance between fetal well-being and the urgent need for intervention. A conservative approach involving close monitoring, corticosteroid therapy to manage peritumoral oedema, and detailed fetal assessments was initially employed. As the patient neared full term, a carefully planned caesarean section was performed, followed by a successful craniotomy to resect the vestibular schwannoma. Both the mother and the newborn showed favourable outcomes postoperatively. In addition, a literature review of cases of vestibular schwannoma in pregnancy was undertaken to inform optimal management strategies and enhance understanding of this complex scenario.
CONCLUSIONS: This case highlights the complexity of managing vestibular schwannomas in pregnant women, and underscores the importance of a tailored, collaborative approach. The condition was resolved successfully, emphasizing the significance of timely diagnosis, meticulous planning and a patient-centred approach in these rare and intricate cases.

Sporadic vestibular schwannomas (VSs) are rare in children. When occurred in the pediatric population, they usually appear bilaterally and are related to neurofibromatosis type 2 (NF2). The current study reports a 4-year-old boy without family history of VS or NF2 who presented with a large (5.7-cm) VS involving the right cerebellopontine angle and internal auditory canal. Through seven-staged surgical interventions and two stereotactic γ‑knife radiosurgery, the disease was stabilized. At 2-year follow-up, the child had right ear hearing loss, grade IV facial palsy, and normal motor function and gait. No definite evidence of gene mutation regarding NF2 can be identified after sequence analysis and deletion/duplication testing. This case highlights the significance of considering the possibility of sporadic VSs, even in very young children. It emphasizes the importance of not overlooking initial symptoms, as they may indicate the presence of a large tumor and could potentially result in delayed diagnosis.

Acoustic neuromas are benign neoplasms of the brain composed of Schwann cells, arising most commonly from the nerve sheath of the vestibular division of the VIII cranial nerve. They usually manifest as unilateral hearing loss, tinnitus, and unsteadiness. Some patients may present atypically with symptoms like orofacial pain, hemifacial numbness, sudden onset hearing loss, or trigeminal neuralgia. Here we report an interesting case of acoustic neuroma in which the patient presented with unilateral facial numbness and tooth pain. Persistent atypical symptoms should always raise clinical suspicion of this pathology, necessitating the need for higher radiological investigations (CT or MRI) to aid in the early diagnosis and treatment.

BACKGROUND: Tinnitus is very common in patients with vestibular schwannoma (VS). We analyzed the related factors of tinnitus after surgery.
METHODS: One hundred seventy-three patients diagnosed with unilateral VS operated via the retrosigmoid approach were included in the study. All patients underwent relevant examinations and completed the THI scale before surgery and 6 months after surgery. The prognosis of tinnitus was evaluated according to the changes in THI.
RESULTS: Of the 129 preoperative tinnitus patients, postoperative tinnitus resolved in 12.4%, improved in 29.5%, remained unchanged in 28.6%, and worsened in 29.5%. 18.2% of 44 patients without preoperative tinnitus appeared new-onset tinnitus postoperatively. Thirty-six patients never had tinnitus. Patients with smaller tumor sizes (≤ 3 cm) were more likely to experience preoperative tinnitus. Younger patients and those with serviceable hearing preoperatively were more likely to report their tinnitus unchanged or worsened. A new onset of postoperative tinnitus in the preoperative non-tinnitus group was found in better preoperative hearing function.
CONCLUSIONS: In this study, 70% of patients had persistent tinnitus after vestibular schwannoma resection. The prognosis of tinnitus was influenced by age and preoperative hearing function. Tinnitus is a bothersome symptom and is often underestimated by doctors. Assessment of tinnitus is mandatory during the management of vestibular schwannoma.

OBJECTIVE: Distinguishing between sporadic and germline/mosaic NF2-related schwannomatosis is important to ensure that patients have appropriate long-term care. With this report, we describe a unique case of a patient with 4 ipsilateral schwannomas and identify a combination of sequencing modalities that can accurately diagnose mosaic NF2-related schwannomatosis.
METHODS: We present a 32-year-old woman with a familial history of vestibular schwannoma in her father and right-sided schwannomas involving the apical and basal turns of cochlea, lateral semicircular canal, and internal auditory canal (IAC). Genetic analysis of blood and frozen tissue from 2 tumors (intralabyrinthine and IAC tumors) was performed using next-generation sequencing (NGS), multiplex ligation-dependent probe amplification (MLPA), and optical genome mapping (OGM).
RESULTS: Germline testing for NF2, LZTR1, and SMARCB1 was negative. Tumor genetic testing revealed a shared NF2 pathogenic variant between the 2 tumors (\"first hit\") but distinct \"second hit\" NF2 variants, including mosaic loss of chromosome 22 in the IAC tumor seen only with OGM, consistent with mosaic NF2-related schwannomatosis.
CONCLUSIONS: Multimodality sequencing, including NGS, MLPA, and OGM, was required to ensure appropriate diagnosis of mosaic NF2-related schwannomatosis in this patient. A similar approach can be used for other patients with multiple ipsilateral tumors and suspected tumor predisposition.

Vestibular Schwannoma (VS) is a benign nerve sheath tumors comprised of Schwann cells. This tumor is encapsulated, slow-growing, and originates from the internal auditory canal, extending into the cerebellopontine angle (CPA). The incidence in individuals aged 20-44 is 0.75 per 100,000 cases, with bilateral VS incidence of 0.8 per 50,000 cases. Tumors in CPA are the most common type in the posterior fossa and cause serious neurological symptoms or become life-threatening when tumors enlarge and compress the brainstem. The majority of tumors are VS (acoustic neuromas), accounting for 80%-90% of cases. Common clinical symptoms include hearing loss, tinnitus, and vertigo. Additionally, these tumors cause compression of the trigeminal and facial nerves. Advances in rapidly evolving imaging technology and surgical methods have improved diagnosis and management. A 24-year-old male complained of hearing impairment for the past 3 years alongside headaches, and dizziness leading to a feeling of imbalance, double, and blurry vision, as well as a sensation of facial thickness on the left side. Neurological examination showed cranial nerve abnormalities, including bilateral paresis of cranial nerves III, IV, VI, left cranial nerves V and VII, bilateral cranial nerve VIII, right cranial nerve XII, and cerebellar abnormalities such as intention tremor, dysmetria, dysdiadokokinesia, wide-based gait, and falling to the right during Romberg testing with both eyes open and closed. The patient underwent a contrast-enhanced MRI of the head, followed by a right CPA tumors excision through craniotomy. A detailed understanding of the medical history, physical examination, and radiological proved to be crucial in establishing an accurate diagnosis and appropriate management. This was considered essential to minimize a worse prognosis.

OBJECTIVE: Intraoperative ultrasonography (ioUS) is an established tool for the real-time intraoperative orientation and resection control in intra-axial oncological neurosurgery. Conversely, reports about its implementation in the resection of vestibular schwannomas (VS) are scarce. The aim of this study is to describe the role of ioUS in microsurgical resection of VS.
METHODS: ioUS (Craniotomy Transducer N13C5, BK5000, B Freq 8 MHz, BK Medical, Burlington, MA, USA) is integrated into the surgical workflow according to a 4-step protocol (transdural preresection, intradural debulking control, intradural resection control, transdural postclosure). Illustrative cases of patients undergoing VS resection through a retrosigmoid approach with the use of ioUS are showed to illustrate advantages and pitfalls of the technique.
RESULTS: ioUS allows clear transdural identification of the VS and its relationships with surgically relevant structures of the posterior fossa and of the cerebellopontine cistern prior to dural opening. Intradural ioUS reliably estimates the extent of tumor debulking, thereby helping in the choice of the right moment to start peripheral preparation and in the optimization of the extent of resection in those cases where subtotal resection is the ultimate goal of surgery. Transdural postclosure ioUS accurately depicts surgical situs.
CONCLUSIONS: ioUS is a cost-effective, safe, and easy-to-use intraoperative adjunctive tool that can provide a significant assistance during VS surgery. It can potentially improve patient safety and reduce complication rates. Its efficacy on clinical outcomes, operative time, and complication rate should be validated in further studies.

Optimizing the extent of resection (EOR) and facial nerve outcomes (FNO) remain a challenge in medium to large vestibular schwannomas (VS). Currently, tumor size has been the only consistently reported factor predicting FNO and EOR. Here, we sought to evaluate whether the degree of the tumor\'s compression on the middle cerebellar peduncle (PC) influences FNO and EOR in medium to large VS.
This retrospective case series included 99 patients who underwent surgical resection of their VSs from 2014 to 2022. Preoperative MR imaging was used to measure the degree of PC. Patient medical records were queried to determine the EOR and FNO.
Patients with unfavorable FNO (HB 3 +) immediately post-op had significantly greater PC than those with favorable FNO (19.9 vs. 15.4 mm, P = .047). This significance was not observed at the last follow-up but there was a trend. When medium-sized tumors (15-30 mm) were analyzed separately, patients with unfavorable FNO immediate post-op and at last follow-up had significantly greater PC than their favorable counterparts (14.1 vs 8.7 mm). Significantly greater PC was also observed in patients who underwent subtotal resection (20.7 mm) compared to near (14.3 mm) and gross total resection (10.8 mm). Multivariate analyses confirmed these findings in medium-sized tumors, but not large-sized tumors.
The degree of PC as measured on preoperative imaging can predict FNO and EOR in medium-sized vestibular schwannomas. Medium-sized tumors with > 15 mm of PC likely will have worse FNO and lower EOR.

In this case report, the authors reviewed a rare case of a vestibular schwannoma manifesting as trigeminal neuralgia (TN). Intracranial tumors can have a variety of orofacial pain symptoms. Among benign cerebellopontine angle tumors, vestibular schwannoma is the most common cause of a TN-like manifestation. Although the most common symptoms of a vestibular schwannoma are hearing loss and vestibulopathy, the unique feature of this case was the manifestation of symptoms consistent with TN.
The patient had right-sided episodic facial pain that was short in duration and severe in intensity. The initial differential diagnoses included short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing and TN. As part of the routine evaluation, the patient was referred for brain magnetic resonance imaging, which revealed a right-sided vestibular schwannoma. The patient was prescribed 200 mg of gabapentin 3 times daily and was referred to neurosurgery for excision of the schwannoma. Surgical excision resulted in complete resolution of pain.
This case illustrates the importance of interdisciplinary treatment and how it can lead to an optimal outcome for a patient with complex orofacial pain symptoms.

Introduction  Vestibular schwannomas (VSs) are treated with microsurgery and/or radiosurgery. Repeat resection is rare, and few studies have reported postoperative outcomes. The objective of this study was to describe clinical characteristics and outcomes in patients undergoing repeat surgery for VS. Methods  All adult (≥ 18 years) patients undergoing VS resection between 2003 and 2022 at our institution were retrospectively reviewed to identify patients who underwent repeat surgery of an ipsilateral VS following prior gross-total (GTR) or subtotal resection. Patient, radiographic, and clinical characteristics were reviewed. Primary outcomes were postoperative tumor volume, extent of resection, postoperative cranial nerve deficits, and time to further tumor progression. Results  Of 102 patients undergoing VS resection, 6 (5.9%) had undergone repeat surgery. Median (range) follow-up was 20 (5-117) months. Three patients were female. Median age was 56 (36-60) years. Median pre- and postoperative tumor volumes were 8.2 (1.8-28.2) cm 3 and 0.4 (0-3.8) cm 3 . GTR was achieved in two patients. Four patients had higher House-Brackmann scores at last follow-up, but none had tumor progression. Conclusion  In this small cohort of patients, repeat resection of recurrent or progressive VS can effectively reduce tumor volume with acceptable perioperative outcomes.