Contralateral Vestibular Schwannomas Research Articles

반대측 신경초종이 동반된 내시경 귀수술로 제거한 고실 사구종

반대측 신경초종이 동반된 내시경 귀수술로 제거한 고실 사구종

Management of Neurofibromatosis Type 2 Associated Vestibular Schwannomas

Treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannomas (VS) can prove challenging because of the various choices, from observation to surgery and radiosurgery with medical treatment opportunities, sometimes within clinical trials. Some factors direct the treatment decision tree such as size of VS and hearing level. Others factors have to be considered such as patient clinical status, inherited situation, and contralateral VS characteristics. All these factors are detailed, discussed, and weighted in this review. Another essential part of the clinical management of patients with NF2 includes the hearing rehabilitation process using either cochlear or auditory brainstem implants. The management of VS in patients with NF2 has become difficult, owing to a multitude of solution options, and should be performed in reference NF2 centers.

Current Management of Large Vestibular Schwannomas for NF2 Patients in a National Reference Center.

Recently, treatment decision making for large vestibular schwannomas (VS) in patients with neurofibromatosis type 2 (NF2) has become increasingly challenging due to the availability of multiple therapeutic options including surgery, bevacizumab (an anti-VEGF), radiosurgery, and observation; and it often remains an arbitrary decision based on local practices without firm recommendations. Our objective is to discuss the multimodal treatment options for Koos IV VS in a national reference center for NF2. Single-institution retrospective cohort study. All NF2 patients with Koos IV VS who visited our center, the National Reference Center for NF2 Rare Disease in Pitié-Salpétrière Hospital of Paris, between January 2016 and December 2018 were included. Clinical charts, radiology, operative reports, and audiograms were reviewed. Among 54 NF2 patients with Koos IV VS (mean maximum extrameatal diameter: 34 mm; range:17-62 mm), 27 were operated on for 28 VS; 21 were treated with bevacizumab; and six were observed. In the surgical group, VS resections were gross total, near-total, subtotal, or partial in 32%, 25%, 32%, and 11%, respectively; and a good (House-Brackmann grades I-II) facial nerve function was achieved in 81.5% at 1 year. Hearing was preserved in 14%, 78%, and 66% of the surgical (n = 7), bevacizumab (n = 9), and observation (n = 3) patients, respectively. All therapeutic options, including surgery and/or bevacizumab and occasionally observation, should be proposed to NF2 patients with large VS in the setting of dedicated centers. A decision-making tree is proposed for Koos IV VS management based on tumor evolution, hearing and clinical status of the patient, and contralateral VS size. 4, case series study, historically controlled study Laryngoscope, 131:E98-E107, 2021.

Hemifacial Spasm as Rare Clinical Presentation of Vestibular Schwannomas

Although classical hemifacial spasm (HFS) has been attributed to an atraumatic pulsatile vascular compression around the root exit zone (REZ) of the facial nerve, rare tumor-related HFS associated with meningiomas, epidermoid tumors, lipomas, and schwannomas in the cerebellopontine angle have been reported. The exact mechanism and the necessity of microvascular decompression for tumor-induced HFS is not clear, especially for vestibular schwannomas. We retrospectively analyzed 10 patients with vestibular schwannomas out of 5218 cases of hemifacial spasm between 2004 and2014. Hemifacial spasm occurred ipsilateral to the vestibular schwannoma in 9 patients and contralateral to the lesion in 1 patient. The mean follow-up period was 86 months (range, 22-140 months). All patients underwent surgery for resection of the vestibular schwannoma. Following the principle of neurovascular compression, offending vessels were found in 7 patients, no offending vessels in 2 patients, and a tumor with the displacement of brain stem contributing to contralateral facial nerve compression in 1 patient. HFS was relieved immediately postoperatively in 9 patients, whereas it improved gradually and then resolved after one month in one patient with a contralateral vestibular schwannoma. For HFS induced by vestibular schwannomas in our study, the majority of cases are caused by a combination of tumor and vascular co-compression at the REZ. Surgical intervention resulted in resolution of symptoms. For HFS with ipsilateral vestibular schwannoma, exploration of the facial nerve root for vascular compression should be performed routinely after tumor resection. It is critical to check that no vessel is contact with the entire nerve root.

The study of clinical factors influencing the growth rate of vestibular schwannoma in neurofibromatosis type II

Objective To assess the clinical factors influencing the growth rate of neurofibromatosis type 2 (NF2)-related vestibular schwannomas (VSs). Methods The medical records of 66 patients with NF2 treated in Department of Neurosurgery of Beijing Tiantan Hospital from January 2003 to October 2016 were analyzed retrospectively. The tumors were measured by the software of Image J. The tumor growth rate was estimated by linear regression analysis of the changes in VS volumes over time. The clinical characteristics of all patients were documented. The relationship between the tumor growth rate and clinical factors was investigated. Linear regression, Pearson's correlation and t-test were conducted using the SPSS 19.0 statistical package. Results The median follow-up duration was 58.8±10.4 months. The average growth rate was 2.6±1.2 cm3/year. The VS growth rate was positively correlated with the initial volume of VS (r=0.97, P<0.01). However, it was negatively correlated with the age at symptom onset (r=-0.41, P<0.01). The VS growth rate in patients who underwent resection of a contralateral VS was slightly higher than that of patients with untreated VSs (P<0.01). The average VS growth rate in patients with spinal tumors was higher than that in those without spinal tumors. Conclusions The potential clinical factors influencing the VS growth rate include the age at symptom onset, initial tumor volume, resection of contralateral VS and presence of spinal tumors, which could provide reference for clinical treatment. Key words: Neurofibromatosis 2; Neuroma, acoustic; Tumour growth rate

Internal Auditory Canal Decompression for Hearing Maintenance in Neurofibromatosis Type 2 Patients.

In neurofibromatosis type 2 (NF2), multiple therapeutic options are available to prevent bilateral hearing loss that significantly affects the quality of life of patients. To evaluate the morbidity and functional results of internal auditory canal (IAC) decompression in NF2 patients with an only hearing ear. Twenty-one NF2 patients operated on for IAC decompression in a 3-year period with a minimum follow-up of 1 year were included in this retrospective study. They presented unilateral deafness due to previous contralateral vestibular schwannoma removal in 16 patients or contralateral hearing loss due to the tumor in 5 patients. Hearing level was of class A (American Academy of Otolaryngology-Head and Neck Surgery classification) in 7 patients, B in 8 patients, C in 1 patient, and D in 5 patients. Pure-tone average and speech discrimination score evaluations were performed at 6 days, 1 year, and during the follow-up. Eight patients had postoperative chemotherapy. No case of facial nerve palsy was observed. In the early postoperative period; all patients maintained the hearing class of the preoperative period. At 1-year follow-up, all but 3 patients maintained their hearing scores; at last follow-up (mean follow-up, 23 ± 8 months; range, 12-44 months), hearing classes remained stable with only 1 patient worsening from class B to C and 1 patient improving from class D to B. Decompression of IAC seems to be a useful procedure for hearing maintenance in NF2 patients, with very low morbidity. Ideal timing and association with chemotherapy should be evaluated in the future. FN, facial nerveIAC, internal auditory canalNF2, neurofibromatosis type 2PTA, pure tone averageSDS, speech discrimination scoreVS, vestibular schwannoma.

Factors influencing the growth rate of vestibular schwannoma in patients with neurofibromatosis type 2.

Neurofibromatosis type 2 (NF2) is a devastating disease with no well-accepted management guidelines. Better understanding of the disease process provides the basis for how or when to initiate treatment. Only few studies have addressed the factors influencing the growth rate of NF2-related vestibular schwannomas (VSs), and these studies have reported variable results. This study aimed to assess the clinical factors influencing the growth rate of NF2-related VSs. The medical records of 66 patients (totalling 74 VSs) were retrospectively analysed. The tumours were measured according to a two-component box model. The tumour growth rate was estimated by linear regression analysis of the changes in VS volumes over time. The clinical characteristics of all the patients were recorded. The relationship between the tumour growth rate and clinical factors were analysed. Linear regression, Pearson's correlation and Student's t-test were conducted using the SPSS 19.0 statistical package. The median follow-up duration was 4.9 years. The VSs growth rate was highly correlated with the initial VS volume (r = 0.97, p < 0.01). However, it was inversely correlated with the age at symptom onset (r = -0.41, p < 0.01). The average VS growth rate in patients with spinal tumours was 13.18 cm(3)/year compared with 0.19 cm(3)/year in patients without spinal tumours (p < 0.01). The VS growth rate in patients who had resection of a contralateral VS was slightly higher than that of patients with untreated VSs (p < 0.01). Other factors including sex, vestibular symptom, presence of other cranial schwannomas, family history and dermal abnormalities did not affect the VS growth rate. The potential clinical factors influencing the VS growth rate are the age at symptom onset, initial tumour volume and presence of spinal tumours. Surgical resection of VSs might accelerate the growth rate of contralateral tumours. The effects of these factors require further experimental confirmation.

Recurrent Contralateral Hearing Loss After 2 Craniotomies for Vestibular Schwannoma

To describe recurrent sudden sensorineural hearing loss after contralateral vestibular schwannoma resection and re-resection. Clinical capsule report. Tertiary academic referral hospital. A patient who underwent 2 craniotomies for vestibular schwannoma. In 2003, a patient experienced contralateral low-frequency sensorineural hearing loss after undergoing translabyrinthine resection of a vestibular schwannoma. This resolved after a course of oral steroids. Seven years later, in 2010, the patient developed tumor recurrence. After retrosigmoid resection, the patient experienced a similar episode of transient, contralateral, low-frequency predominant sensorineural hearing loss. The recurrence of contralateral hearing loss after craniotomies years apart suggests that patient specific anatomic risk factors predispose an individual to hearing loss after contralateral cerebellopontine angle surgery. Patients with previous history of contralateral hearing loss should be counseled that they may be at increased risk for recurrent loss in the setting of re-resection.

Pediatric cerebellopontine angle and internal auditory canal tumors

The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed. Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9-18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening. Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.

Increased growth rate of vestibular schwannoma after resection of contralateral tumor in neurofibromatosis type 2

Surgical management of bilateral vestibular schwannomas (VS) in neurofibromatosis type 2 (NF2) is often difficult, especially when both tumors threaten the brainstem. When the largest tumor has been removed, the management of the contralateral VS may become puzzling. To give new insights into the growth pattern of these tumors and to determine the best time point for treatment (surgery or medical treatment), we studied radiological growth in 11 VS (11 patients with NF2) over a long period (mean duration, 7.6 years), before and after removal of the contralateral tumor while both were threatening the brainstem. We used a quantitative approach of the radiological velocity of diametric expansion (VDE) on consecutive magnetic resonance images. Before first surgery, growth patterns of both tumors were similar in 9 of 11 cases. After the first surgery, VDE of the remaining VS was significantly elevated, compared with the preoperative period (2.5 ± 2.2 vs 4.4 ± 3.4 mm/year; P = .01, by Wilcoxon test). Decrease in hearing function was associated with increased postoperative growth in 3 cases. Growth pattern of coexisting intracranial meningiomas was not modified by VS surgery on the first side. In conclusion, removal of a large VS in a patient with NF2 might induce an increase in the growth rate of the contralateral medium or large VS. This possibility should be integrated in NF2 patient management to adequately treat the second VS.

Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2

The authors report on 2 patients with bilateral vestibular schwannomas (VSs) who underwent unilateral surgical tumor removal. One patient was followed up for 4 years, the other for 9; in both cases, the contralateral VS regressed markedly without any additional treatment during the follow-up period. Serial MR imaging was performed to monitor the untreated tumor, which in both cases involved the only hearing ear. The tumors were assessed volumetrically. The contralateral tumors appeared to enlarge mildly at initial follow-up and then, with no treatment, regressed (to 23% of the original maximum volume in Case 1 and to 15% of the original maximum in Case 2). The largest posterior fossa diameter decreased from 30.1 mm to 18.6 mm in Case 1 over 4 years and from 27 mm to 16 mm over 8 years in Case 2. Hearing declined only mildly during follow-up in both patients. These cases demonstrate the first well-documented, long-term, spontaneous VS regressions in patients with neurofibromatosis Type 2. They underline the importance of careful observation of VS involving the only hearing ear in the management of bilateral VS to determine the natural growth pattern of the tumors. The mechanism of the dramatic spontaneous tumor regression is uncertain.

Spontaneous Regression of Contralateral Vestibular Schwannomas after Resection of an Ipsilateral Tumor in Neurofibromatosis Type II

Spontaneous Regression of Contralateral Vestibular Schwannomas after Resection of an Ipsilateral Tumor in Neurofibromatosis Type II

Ipsilateral and contralateral vestibular schwannomas in unilateral long-term deaf ears

In the past decade, we have encountered two patients with unilateral long-term deaf ear having vestibular schwannoma. One was on the deaf ear in a 53 years' woman and the other was on the contralateral hearing ear in a 62 years' woman. These two patients had total deafness on the right ear since childhood, associated with absent auditory brainstem response (ABR) and caloric responses, whereas normal ABR and caloric responses were shown on the left ear. Although similar physiological findings were disclosed on the deaf ears, MRI scan demonstrated vestibular schwannoma on the different side, that is, one in deaf ear and the other in hearing ear. For the former, absent ABR and caloric results indicate that the tumor originates from the superior vestibular nerve. In contrast with the latter, normal ABR and caloric responses, but absent vestibular-evoked myogenic potentials on the hearing ear imply that the tumor originates from the inferior vestibular nerve. The ipsilateral tumor on the deaf ear underwent intracranial surgical excision, followed by radiosurgery due to tumor recurrence 10 years later, while the contralateral one on the only hearing ear received radiosurgery only. Both patients were rather well, 3 years after last treatment.

Unilateral vestibular schwannoma with other neurofibromatosis Type 2–related tumors: clinical and molecular study of a unique phenotype

Although the manifestations of neurofibromatosis Type 2 (NF2) vary, the hallmark is bilateral vestibular schwannomas (VSs). The authors studied the clinical course and genetic basis of unilateral VSs associated with other NF2-related tumors. Forty-four adults presenting with unilateral VSs and other NF2-related tumors were identified. A comprehensive review of patient records and cranial imaging was conducted. Molecular analysis of the NF2 locus was performed in available tumors and paired blood specimens. Patient age at symptomatic onset ranged from 11 to 63 years (mean 32 years). Twenty-two patients (50%) presented with eighth cranial nerve dysfunction. Twenty-six presented with multiple lesions. Thirty-eight harbored other intracranial tumors and 27 had spinal tumors, with most lesions situated ipsilateral to the VS. No patient had a relative with NF2, although two of 63 offspring had isolated NF2-related findings. A contralateral VS developed in four patients 3 to 46 years after the symptomatic onset of a unilateral VS, and two of these patients experienced rapid progression to total deafness. Results of a Kaplan-Meier analysis identified actuarial chances of developing contralateral VS: 2.9% (3-17 years after the VS symptomatic onset of unilateral VS), 11% (18-24 years), and 28.8% (25-40 years). Mosaicism for the NF2 gene mutation was proven in eight patients. The authors describe the clinical features of this unique phenotype--unilateral VS with other NF2-related tumors. Persons with this phenotype should undergo evaluation and monitoring similar to that conducted in patients with NF2, and the possibility of aggressive contralateral VS formation should be considered in their treatment. Molecular genetic analysis is best performed using resected tumor specimens and will enable future studies to determine the genetic risks of individuals with mosaicism.

Molecular genetic analysis of the NF2 gene in young patients with unilateral vestibular schwannomas

Neurofibromatosis type 2 (NF2) must be suspected in patients presenting with a unilateral vestibular schwannoma at a young age who are therefore at theoretical risk of developing bilateral disease. We...