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Objectives: Cerebellopontine angle (CPA) tumors are a common cause of secondary trigeminal neuralgia (TN), characterized by their concealed location, slow progression, and difficulty in early detection. This study aims to explore the clinicopathological characteristics of patients with secondary TN due to CPA tumors to enhance understanding and management of secondary TN., Methods: A retrospective analysis was conducted on clinical data and pathological results of 116 patients with CPA tumor-related TN treated at Xiangya Hospital of Central South University from January 1, 2017 to December 31, 2022. The study analyzed the relationship of tumor pathological types with clinical manifestations, tumor location, surgical methods, and treatment outcomes., Results: Among the cases, 95.7% (111/116) were benign tumors, 3.4% (4/116) were malignant tumors, and 0.9% (1/116) were borderline tumors. Benign tumors were predominantly acoustic neuromas, meningiomas, and schwannomas. Among the patients, 46.6% (54/116) presented with isolated TN, while 53.4% (62/116) exhibited other associated symptoms depending on factors such as tumor growth location and rate. The complete resection rate in this group was over 90%, with 41.4% (48/116) of patients undergoing concurrent microvascular decompression after tumor resection, predominantly for schwannomas. The overall effective rate of surgical treatment reached 93.9%, with schwannomas showing higher efficacy rates compared with acoustic neuromas and meningiomas ( P <0.05). The recurrence rate of acoustic neuromas was significantly higher than that of meningiomas and schwannomas ( P <0.05)., Conclusions: CPA tumors are a major cause of secondary TN, predominantly benign, with occasional underdiagnosed malignant tumors. Early diagnosis and treatment significantly impact prognosis. Different tumor types vary in clinical symptoms, surgical approaches, and treatment efficacy. Surgical strategies should balance tumor resection extent and neural function preservation, with microvascular decompression as necessary.

Objective: This study was conducted to evaluate the applications of vestibular function tests in diagnosis, identifying tumor origins and prognosis of vestibular rehabilitation of patients with acoustic neuroma. Methods: This research is a single-center cross-sectional clinical study, which retrospectively analyzed the data of 335 patients with acoustic neuroma from March 2013 to March 2020 in the Eye and ENT Hospital of Fudan University. The study included caloric test, cervical and ocular vestibular evoked myogenic potentials（cVEMP, oVEMP）, video head impulse test（vHIT） and sensory organization test（SOT）. Firstly, the sensitivity, specificity, and Yoden index of each test were calculated. Secondly, the internal relevance of these tests was studied for application in judging the origins of the tumor. Results: The abnormal rates of caloric test, cVEMP, oVEMP, vHIT and SOT was 85.3%, 86.1%, 85.5%, 55.6% and 67.7% in these participants. Among all the vestibular function tests included, the caloric test showed the best sensitivity（0.855）, specificity（0.981）, and Yoden index（0.836）. The study found that the higher the Koos grades, the higher the abnormal rates of the caloric test, vHIT, and oVEMP（Cochran-Armitage test, P <0.05）. There was no significant relationship between the combination of abnormal vestibular function tests and tumor origin nerves（ P >0.05）. Conclusion: Majorlty of the participants in this study with acoustic neuroma showed abnormal results in SOT related to poor balance control. More than half of the patients had at least two abnormal result of the battery of vestibular function tests, among which the caloric test was proved to have better sensitivity and specificity. The higher the Koos grades of the tumor, the higher the abnormal rates of the caloric test, vHIT, and oVEMP., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objective: To analyze the clinical characteristics of unilateral acoustic neuroma（AN） with normal hearing, so as to provide evidence for early identification AN. Methods: Clinical datas from 73 patients of unilateral AN with normal hearing of Otorhinolaryngology Head and Neck Surgery of Beijing Tiantan Hospital affiliated of Capital Medical University from August 2019 to April 2022 admitted to department were retrospectively analyzed. All patients underwent pure tone audiometry（PTA）, speech discrimination score（SDS）, auditory brainstem response（ABR）, distortion product otoacoustic emission（DPOAE） and head enhanced MRI. Results: The incidence of normal hearing among patients with AN was 10.7%. Male∶female=1∶2.2; the mean age of the patients was（37.3±9.4） years; the mean tumor size was（24.2±11.2） mm. Tinnitus was the most common reason for visit; the patients who had headache and dizziness had larger tumors. Surgery was the main treatment, and the patients who underwent surgery had larger tumors than those of follow-up. Heterogeneous tumors were the most common type of MRI, homogeneous tumors were smaller than heterogeneous and cystic tumors. The sensitivity of ABR in the diagnosis of AN with normal hearing was 95.9%, and that of ≥20 mm tumors was 100%; prolonged Ⅴ-waves were the most common, patients with Ⅴ-wave deletion had larger tumors than those with normal or prolonged Ⅴ-waves. Patients who had the longer the Ⅴ-wave and the longer difference between Ⅰ-Ⅴ wave had larger tumors. DPOAE was not elicited at full frequency in 11 patients. There was no statistically significant difference in age among patients with different symptoms, treatments, types of MRI, ABR and DPOAE. Conclusion: AN of normal hearing was most common in 30-39 years old women. Patients had different symptoms, phenotypes of MRI and ABR. Patients with normal hearing who had tinnitus, dizziness, headache, facial paraesthesia, and recovery after sudden haring loss can be further examination of ABR and DPOAE for early identification AN. The sensitivity of ABR in diagnosis of hearing normal AN was 95.9%, and the abnormal type of Ⅴ-wave is related to tumor size., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

At present, the main treatment for vestibular schwannomas is surgery. Considering the risk of multiple complications from surgery and the subjective and objective conditions of patients, a non-surgical treatment modality, namely stereotactic radiotherapy, has gradually been included in the treatment of vestibular schwannomas. Studies have shown that Gamma Knife therapy has a more prominent therapeutic effect on smaller tumors and can alleviate facial nerve disorders caused by space occupying of tumor mass. Cyberknife not only has a better effect on tumor control, but also has an ideal retention rate for patients' auditory function. Proton beam therapy has also been gradually applied to the treatment of vestibular schwannomas, but the effect of treatment remains to be further studied. Drug therapy includes a variety of target inhibitors and anti-angiogenic drugs. At present, drug treatment focuses more on preclinical research. This article reviews the clinical research of various radiotherapy and the progress of drug treatment., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objective: To explore the clinical features and treatment strategy of rare tumor in the internal auditory canal(IAC). Methods: A retrospective study was carried out in 213 patients with lesion of ICA form January 2010 to December 2020. According to imaging features, surgical findings, and pathological diagnosis, there were 7 cases of non-sporadic acoustic neuroma, including 2 cases of cavernous hemangioma, 2 cases of aneurysm, 1 case of intralabyrinthical schwannoma, 1 case of meningioma, and 1 case of unilateral neurofibromatosis type 2 (NF2). The clinical manifestations, imaging data and intraoperative conditions as well as the formulation of individualized treatment strategies and prognosis were comprehensively analyzed. Results: In addition to hearing loss, cavernous hemangioma early appeared damage of facial nerve. CT showed expansion and calcification of IAC. Patients with aneurysm appeared tinnitus and vertigo early. CT showed enlargement of ampulla shape of IAC. DSA or MRA can help confirm the diagnosis. Patients with intralabyrinthine schwannoma early appeared refractory vertigo. High resolution MRI was helpful for diagnosis. "Dural tail sign" can be seen on enhanced MRI of meningeoma. Neurofibromatosis type 2 usually presented as bilateral vestibular schwannomas,but a few patients presented only with unilateral vestibular schwannomas.. All patients underwent labyrinth approach resection except one patient with NF2 for followed-up. Their postoperative symptoms were relieved. No tumor recurrence was observed during 6-3 years of follow-up. Conclusions: The clinical and imaging manifestations of rare tumors of the internal auditory canal are different. The principle of treatment is also different. It is helpful to avoid the serious consequences caused by blind operation to confirm diagnosis before operation.

Objective: To investigate the relationship between the amplitude change of supramaximal facial nerve stimulation during acoustic neuroma removal and facial function outcome, and discuss its influencing factors., Methods: Retrospective study of 34 acoustic neuroma patients. Intraoperative amplitude by supramaximal stimulation, facial function outcome, tumor size, surgical approach, facial nerve course pattern, and tumor cystic status, as well as tumor adhesion degree were collected and analyzed by SPSS 17.0., Results: Acoustic neurnomas were removed in all 34 patients without intra- and postoperative complications, the facial nerve were all anatomically preserved. Two weeks later, 5 cases showed facial function HB Grade Ⅰ, 13 HB Ⅱ, 11 HB Ⅲ and 5 HB Ⅳ. The amplitude changes under supramaximal stimulation after tumor removal were 12.9%-100%, average (63.9±25.7)%. The facial function outcomes were poor (HB Ⅳ) in the cases whose intraoperative amplitude decreases were more than 70%. Different postoperative facial function group showed significantly different intraoperative amplitude changes (F=7.585, P=0.001), except HB Ⅰ group and HB Ⅱ group. The existence of cystic degeneration showed strong correlation (r=-0.635) to the amplitude changes, the level of tumor adhesion showed moderately negative correlation (r=-0.455), and tumor size showed weak negative correlation (r=-0.292), surgical approach and the facial nerve course pattern were not statistically relevant., Conclusions: The amplitude change of facial nerve supramaxial stimulation can predict the postoperative facial function. The amplitude change is valuable for early treatment of facial nerve damage.

Current treatment options of vestibular schwannomas should not be limited to conventional surgery because of the variability of tumor growth. Recent studies of vestibular schwannomas suggested that the development of the tumor tended to depend on the shuttle between the nucleus and cytoplasm, and the expression patterns of merlin protein. New drug targets for schwannoma therapies might be identified through future in-depth investigations of the function of merlin, thus contributing to tumor control.

Objective: To discuss the surgical technique and common complications from the microsurgical treatment of giant intracranial vestibular schwannoma via suboccipital retrosigmoid approach and to propose strategies for minimizing such complications., Methods: Surgical outcomes and complications were evaluated in a consecutive series of 657 unilateral giant vestibular schwannomas treated in Shanghai Huashan Hospital via suboccipital retrosigmoid approach from 1999 to 2014. According to the international classification of vestibular schwannoma, giant tumor means tumor's size over 4 cm in diameter. Clinical status and complications were assessed postoperatively within 14 days and at follow-ups (range, 6-191 months; mean, 59.6 months)., Results: Follow-up data were available for 566 of the 657 patients (86.1%). The most frequent clinical symptoms were hearing loss in different levels (100%), deafness (36.4%), facial numbness (68.8%). Total tumor resection was achieved in 556 patients (84.6%), subtotal resection in 99 patients (15.1%), and partial resection in 2 patients (0.3%). The common postoperative complications included new deafness (49.6%), intracranial infection (7.6%), low cranial nerve defect (7.5%) and pneumonia (6.2%). The facial nerve was preserved anatomically in 589 cases (89.6%) after operation, and the functional valuation of facial nerve according to postoperative House-Brackmann showed 216 patients (32.9%) in grade Ⅰ-Ⅱ, 308 cases (46.9%) in grade Ⅲ, 133 patients (20.2%) in grade Ⅳ-Ⅵ. Long-term followed-up results showed 428 patients (75.6%) in grade Ⅰ-Ⅲ one year after surgical treatment., Conclusions: Many of these complications are avoidable. Surgical experiences and the clinical anatomy of the approach, accompany with using intraoperative nerve monitoring, preoperatively study the individual imaging and clinical data and multidisciplinary cooperation are the key points to avoid the complications of giant intracranial vestibular schwannoma via suboccipital retrosigmoid approach.

Objective: To analyze the surgical outcomes and share experience in the surgical management of acoustic neuroma(AN)., Methods: A retrospective review was performed in 134 patients with sporadic large ANs operated from Jan. 2001 to Dec. 2012. The patients' information, tumor size, tumor cystic degeneration, surgical approach, intraoperative anatomical facial nerve integrity rate, postoperative facial nerve function and complications were recorded., Results: There were one hundred and twenty-nine patients (96.3%) received surgeries within one month after first diagnosis of vestibular schwannoma(VS) or definite diagnosis of recurrence.Five patients (3.7%) hesitated to be operated until tumor was found to be growing on MRI during the follow-up. The average tumor diameter was about (40.9 ± 6.8) mm (31.0-70.0 mm). The cystic tumor percentage arrived at 35.1% (47/134). The surgical approaches included 118(88.1%) translabyrinthine approaches, comprising 16(11.9%) modified enlarged translabyrinthine approaches, and 16 (11.9%) transaortic approaches. Total, near total, and subtotal tumor removal rate was 91.0% (122/134), 6.7% (9/134) and 2.2% (3/134), respectively. The anatomical facial nerve integrity was preserved in 88.8% (19/134) of all patients. And it was significantly lower in cystic VS (80.9% vs 93.1%, P = 0.044). There were 32.3% (43/133) and 36.8% (49/133) of patients had a good postoperative facial nerve function in short-term (discharge from hospital) and long-term (1 year) follow-up, respectively.General rate of complications was 17.9% (24/134). CSF leakage occurred in 7.5% of patients. Mortality rate was 0.7% (1/134). Follow-up time ranged from 1 to 5 years, average time was(2.7 ± 0.4)years.Four cases occurred recurrence., Conclusions: Major management strategy of large AN is surgical resection.Even for those who had temporary antagonism for surgery, the wait and scan policy is necessary. Although the tumor removal is safe and accompanies extremely low mortality and incidence of complication, the patient with large AN must be informed in detail about the possible surgical risks. The tumor cystic degeneration is an important crucial factor which influences the surgical outcomes of AN, especially in postoperative facial nerve function.

Objective: By detecting the myelin structure in acoustic tumor tissues, the cell origin and state of acoustic tumor tissues were investigated., Method: Immunofluorescence labeling, immunoblot analysis and electron microscopic study were performed to identify myelin structure and myelin protein in acoustic tumor tissues., Result: In this work, we found some early stage of myelin forming in acoustic tumor tissues, but there were no axon nor compact myelin formed and the myelin basic protein whose expression indicates the beginning of myelination was negative detected. We also found that the cell of acoustic tumor express p75,a marker for immature Schwann cells and mature non-myelin-forming Schwann cells., Conclusion: The date shown in this experiment indicates that the cell of acoustic tumor is in a remyelinating state.

Objective: To investigate the impact of S518 phosphorylation in Merlin on the interaction with CD44 in vestibular schwannoma and the tumor growth., Methods: Thirty-five samples of vestibular schwannoma were identified by pathology. Immunohistopathology and western blot were employed to analyze the expression and localization of S518 phosphorylated Merlin in the tumor tissues. Nerve tissues that were collected during other surgical operation were used as control. The expression level of S518 phosphorylated Merlin was compared with clinical stages, tumor size, clinical course and cystic degeneration. Immunoprecipitation was used to evaluate the impact of S518 phosphorylation in Merlin on the interaction with CD44., Results: In vestibular schwannoma, Merlin was phosphorylated at S518 and demonstrated perinuclear localization. The S518 phosphorylation level was much lower in the normal control nerve tissues than that in vestibular schwannoma tissues. There was no correlation between the phosphorylation level on Merlin and clinical stages, tumor size, clinical course and cystic degeneration. The S518 phosphorylated Merlin bound CD44 was higher than wild-type Merlin bound CD44 in vestibular schwannoma tissues., Conclusions: The affinity of Merlin to CD44 was increased after phosphorylation at S518. Different cellular biological results might be triggered through binding to wild type Merlin and S518 phosphorylated Merlin.

Objective: To evaluate the relationship between labeling index (LI) Ki-67, proliferating cell nuclear antigen (PCNA) and transforming growth factor-beta1 (TGF-beta1) with the clinical behavior of acoustic neuroma., Methods: Expression of Ki-67, PCNA and TGF-beta1 was detected by immunohistochemistry in 53 specimens of acoustic neuromas. The relationship among tumor proliferation, histological representation, size of tumor, clinical proliferation index of tumor and tumor proliferation activity were analyzed., Results: In all 53 cases, the positive rate of Ki-67 was 77.4% (41/53) but the positive rate of PCNA was 84.9% (45/53). There was significant difference between the proliferate index, clinic growth rate and course of disease (t = 2.14, t = 2.70; P < 0.05). The positive rate of TGF-beta1 was 83.0% (44/53). The correlation of TGF-beta1 with LI (Ki-67) was significant difference (r = 0.36, P < 0.05). Cystic degeneration often occurred in large-size tumor (Z = 4.44, P < 0.05). There was no significant relationship between the expression of LI (Ki-67), LI (PCNA) and TGF-beta1 and the course of disease as well as between the cystic degeneration and the non-cystic degeneration. Although clinic growth rate of cystic degeneration was bigger than that of non-cystic degeneration, there was not statistically significant., Conclusions: Ki-67 and PCNA are reflected proliferation activities of tumor cells in acoustic neuromas. Cell proliferation-labeling index LI (PCNA) was related with clinical growth rates. TGF-beta1 might participate in the biological behavior of acoustic neuroma. Cystic degeneration was one of special pattern of acoustic neuroma, however, tumor enlargement might due to the volume of the cystic but unrelated to fast proliferation of parenchyma cell.

Objective: To clarify the expression and subcellular localization of merlin in vestibular schwannoma., Methods: Fifty four paraffin embedded vestibular schwannoma samples confirmed by pathology after resection were included in the study. The expression of merlin in vestibular schwannoma was analyzed by immunohistochemistry. Nerve tissues that were resected during surgical treatment for trigeminal neuralgia and Meniere's disease were used as control. Western blotting was used to analyze the electrophoresis migration of merlin in the acoustic neuroma. Image analysis was used to calculate the positive expression percentage of merlin in each individual. The expression percentage of merlin in the tumor tissue was compared with age and gender of the patients, clinical course of the tumor, tumor growth index, tumor diameter and clinical stage., Results: Merlin was expressed in 0 to 87.5% of the cells in vestibular schwannoma tissue with a mean of (46.66 +/- 5.75)%. There was a negative correlation between merlin expression percentage and tumor growth index. There were no correlations between merlin expression percentage and the age, gender, tumor diameter and clinical stage. There exists a difference for the location of merlin, mainly in the nucleus and perinucleus. There was also a cytoplasmic location. Merlin in the tumor tissue was shown by western blot to be in 65000 and 125000 positions., Conclusions: Merlin was expressed in vestibular schwannoma tissue, with a different intra-cellular location. Merlin might also exist as a complex with other proteins in the tumor tissue.

Background and Objective: Acoustic neurinomas is one of the most common benign tumor in central nervous system, the main treatment is surgical resection. This study is to explore keyhole surgery with suboccipital retrosigmoid sinus approach for acoustic tumor resection and discuss how to improve the surgical skill and effect., Methods: Thirteen cases of acoustic neurinomas from Sept. 2000 to Dec. 2001 in our department were operated using keyhole craniotomy technique through unilateral modified suboccipital retrosigmoid approach with "[symbol: see text]" incision. All patient underwent suboccipital craniotomy in order to maintain anatomical replacement, and the tumors (2.0-4.4 cm in diameter) were removed under microscope., Results: Tumors were completed resected in 11 cases, subtotally removed in 2 cases. Eleven patients had obtained anatomic preservation of the facial nerves. Complete follow-up information was obtained in all patients for a period of 3-15 months after operation. House-Brackmann Score in 8 case were Grade I-II, 4 cases Grade III-IV, one case Grade V. Grades I and II facial nerve function were maintained in 61.5% of cases, measurable hearing was preserved in 53.8% of cases(7 cases), and 38.5% of cases maintained serviceable hearing. No severe permanent operative complications were found and no surgical mortality occurred., Conclusions: Microsurgery with keyhole craniotomy is a safe and effective method for treatment of patients with small and medium-size acoustic neuromas. The advantages of keyhole suboccipital craniotomy are anatomical replacement, less postoperative complications, and beneficial to patient's mental health.

Objective: To study the anatomicopathological relation between facial nerve and large vestibular schwannoma., Methods: Operation by suboccipital retrosigmoid sinus approach was performed on 40 cases with large vestibular schwannoma, During the operation, the anatomicopathological relation between the facial nerve and the vestibular schwannoma was observed directly., Results: The facial nerve was found to be located ventrally (deep under the tumor), dorsally (over the tumor), at the upper pole of the tumor (near the tentorium cerebelli), at the lower pole of the tumor (near the rear group cranial nerves), or aberrant (unable to be identified because of infiltration of tumor). In 31 cases, mainly with parenchymatous tumor, the facial nerve was flat in shape. In 9 cases, mainly with cystic tumor, the facial nerve was bandlike., Conclusion: The facial nerve varies greatly in neuroanatomy among patients with large vestibular schwannoma. Strengthening of operative monitoring can increase the safety of operation.

Objective: To further understand the biological features of the human vestibular organ., Methods: Vestibular hair cells were isolated from patients with acoustic neuroma during the surgery and intracellular free calcium concentration of the cells was monitored with calcium-sensitive dye Fura-2 and digital imaging microscopy., Results: Isolated vestibular hair cells were mainly of type I and could survive about 2.5 hours in vitro. Perfusion with 150 mmol/L KCl, induced a depolarization, resulting in a temporary increase of 340 nm/380 nm ratio from 0.52 to 1.16 within 1 min. In Ca2+ free environment, 150 mmol/L KCl failed to induce intracellular calcium increase. In the presence of 2.5 mumol/L ionomycin, there was an irreversible increase in 340 nm/380 nm ratio., Conclusion: Human vestibular hair cell apparently possess voltage sensitive calcium channels. The method and observation aid in elucidating human vestibular biological features.

To improve the therapeutic effect of acoustic neuroma, 158 patients with acoustic neuroma treated in our hospital were retrospectively reviewed. Among them, 11 recurrent acoustic neuroma were found. The clinical analysis demonstrated that the recurrence of acoustic neuroma had close relationship with the size of the tumor, operative approach and extent of excision. All recurrent acoustic neuroma was large-sized when first operated. The more the tumor was excised, the lower the recurrent rate was. The recurrent rates were 19.4% for partial resection, 13.2% for near-total resection, 0% for total resection respectively. Retrolabyrinthine approach was more likely to recur than other approaches. The key to decrease recurrent rate was to excise as much tumor as possible. According to growth rate and double time of acoustic neuroma measured in this study, regular follow-up with CT or MRI at interval of 6 months was emphasized. X-knife was effective for early-diagnosed recurrent acoustic neuroma.

A case of small occult schwannoma in a 73-year-old man is reported. Histopathological sections of the temporal bone revealed a tumor in the left internal auditory meatus. The tumor measured about 5 x 6 mm in size. There were whorls of spindle shaped cells with the typical palisading characteristic of acoustic neurinoma-Antoni A type. The posterior wall of the meatus had been eroded but the nerve fibers were intact. It seems to have raised from the cochlear nerve trunk and was displaced and compressed. Slight degeneration of the spiral ganglion cells and the cochlear nerve fibers was found. There was a very fine granular eosinophilic precipitate throughout the perilymphatic space. This probably represents increased protein content in the perilymph. Thickening of the walls of the internal auditory artery and some vessels of striae vasculares was also noted. The organs of Corti, the maculae of the utricle and saccule and the cristae of semicircular canals appeared normal.