Vestibular schwannomas(VS)is a benign tumor originating from Schwann cells in the vestibular nerve sheath of the internal auditory canal,often causing symptoms such as hearing loss and tinnitus in patients.With the significant increase in early detection rate of tumors,treatment goals are shifting towards preserving and rebuilding neurological function,improving patients'quality of life,and individualized treatment strategies.For those with functional hearing,hearing preservation surgery(HPS)is preferred.For patients who failed to do HPS or have no functional hearing before surgery,auditory restoration can be performed simultaneously or later.In terms of restoration approaches,for individuals whose cochlear nerve can be preserved,cochlear implant(CI)may be a viable option.CI not only provides good auditory perception but also effectively suppresses tinnitus.When the cochlear nerve cannot be preserved,BoneBridge implantation are considered.Although BoneBridge implantation cannot completely restore normal auditory function,it significantly improves speech recognition and quality of life.For patients with bilateral acoustic neuromas(as seen in neurofibromatosis type 2),although the effect of artificial auditory brainstem implantation(ABI)on auditory reconstruction varies greatly,it can help most patients regain auditory perception and improve communication skills,which is of great significance.Further research is needed in the future to investigate the influencing factors of auditory preservation and reconstruction effects,in order to maximize the hearing recovery and quality of life improvement for each patient.

Vestibular schwannomas(VS)is a benign tumor originating from Schwann cells in the vestibular nerve sheath of the internal auditory canal,often causing symptoms such as hearing loss and tinnitus in patients.With the significant increase in early detection rate of tumors,treatment goals are shifting towards preserving and rebuilding neurological function,improving patients'quality of life,and individualized treatment strategies.For those with functional hearing,hearing preservation surgery(HPS)is preferred.For patients who failed to do HPS or have no functional hearing before surgery,auditory restoration can be performed simultaneously or later.In terms of restoration approaches,for individuals whose cochlear nerve can be preserved,cochlear implant(CI)may be a viable option.CI not only provides good auditory perception but also effectively suppresses tinnitus.When the cochlear nerve cannot be preserved,BoneBridge implantation are considered.Although BoneBridge implantation cannot completely restore normal auditory function,it significantly improves speech recognition and quality of life.For patients with bilateral acoustic neuromas(as seen in neurofibromatosis type 2),although the effect of artificial auditory brainstem implantation(ABI)on auditory reconstruction varies greatly,it can help most patients regain auditory perception and improve communication skills,which is of great significance.Further research is needed in the future to investigate the influencing factors of auditory preservation and reconstruction effects,in order to maximize the hearing recovery and quality of life improvement for each patient.

Objective:To investigate the clinical characteristics, differential diagnosis, treatments and prognosis of facial nerve hemangioma and schwannoma at genicular ganglion, so as to provide reference for clinical diagnosis and treatments of facial nerve tumor at genicular ganglion.Methods:Clinical data of 13 patients with facial nerve tumors at genicular ganglion confirmed by postoperative pathology in the Ninth People′s Hospital affiliated to Shanghai Jiaotong University School of Medicine from March 2018 to April 2020 were retrospectively analyzed, including seven cases of hemangioma and six cases of schwannoma. There were eight males and five females. Their ages ranged from 20 to 65, with an average age of 40. The course of disease ranged from 3 to 118 months, with an average of 52 months. All the patients underwent preoperative HRCT of the temporal bone and facial nerve dynamic contrast-enhanced(DCE) MRI examinations. All the patients had detailed surgical procedures and at least one-year postoperative follow-up.Results:On HRCT of the temporal bone, (4/7) hemangioma at geniculate ganglion showed characteristic honeycomb appearance, while 6/6 schwannoma and 3/7 hemangiomas showed expansive bone changes. On DCE-MRI, geniculate ganglion hemangioma (7/7) showed characteristic "point-to-surface" enhancement, and schwannoma (6/6) showed characteristic "face-to-surface" enhancement. For five hemangioma-patients with HB-Ⅱ-Ⅳ before surgery, the facial nerve anatomy was completely preserved through transcanal endoscopic approach(TEA), and the facial nerve function improved one year after surgery (two cases of HB-I, two cases of HB-Ⅱ, and one case of HB-Ⅲ). For two patients, with preoperative facial nerve function HB-Ⅴ-Ⅵ, since their tumors was inseparable from the nerves, they were performed with facial nerve anastomosis during the surgery, and the facial nerve function was improved to HB-Ⅳ level one year after surgery. For six patients with meningioma whose facial nerve function was greater than or equal to HB-Ⅲ, based on the preoperative hearing level, the involved segments, and duration of facial paralysis, three of them were conducted surgeries through middle cranial fossa approach, one by translabyrinthine approach, and one via mastoid approach. Two patients among them with complete facial paralysis over three years preoperatively were not performed facial nerve anastomosis after total resections of the tumors, and there was no improvement in facial nerve function one year after surgery. Three patients underwent facial nerve anastomosis after total tumor resections, and their facial nerve function was HB-Ⅲ in one patient, HB-Ⅳ in two patients one year after surgery. One patient (preoperative HB-Ⅲ) had a normal hearing level preoperatively, and the tumor involved the labyrinth segment. To protect the hearing, partial tumor was resected through the middle cranial fossa approach, and facial nerve function improved to HB-Ⅱ one year after surgery.Conclusions:Temporal bone HRCT combined with DCE-MRI are useful for the differential diagnosis of hemangioma and schwannoma at geniculate ganglion and provide references for preoperative clinical decision makings. It is extremely necessary to select the appropriate surgical approach based on the patient′s hearing and involved segments. For geniculate ganglion hemangioma, early surgery can improve the possibilities of anatomical integrity of facial nerve, thereby improving facial nerve function postoperatively.TEA is a kind of surgical method worth consideration, with the characteristics of minimally invasive, favorable postoperative features, and so on. For schwannoma, one-stage functional reconstruction of the facial nerve is recommended during the resection of the tumors because of the inevitable damage to the anatomical integrity of the facial nerve.

Objective:To analyze imaging features on high resolution CT (HRCT) and MRI of facial nerve venous malformation (FNVM).Methods:A total of 23 patients with pathologically confirmed FNVM were retrospectively included from January 2016 to December 2020 in the Shanghai Ninth People′s Hospital, Shanghai Jiao Tong University School of Medicine. All patients underwent preoperative temporal bone HRCT, non-contrast MRI, diffusion weighted imaging (DWI) and dynamic contrast enhanced MRI (DCE-MRI). The location, morphology, and signal intensity of the lesions, as well as the enhancement pattern on DCE-MRI were reviewed. The time-intensity curve (TIC) was analyzed and the apparent diffusion coefficient (ADC) was measured.Results:The locations of FNVM lesions were geniculate ganglion ( n=19), mastoid segment ( n=1) and internal auditory canal ( n=3). Among 19 cases in geniculate ganglion, HRCT showed honeycomb-like bone change ( n=13) or expansive bone destruction with sharp border ( n=6). The case located in mastoid segment also presented as honeycomb-like changes. The other 3 cases in internal auditory canal presented with patchy hyperdensity bone change ( n=2) or resorptive bone destruction ( n=1). All 23 lesions demonstrated as irregular nodules with obviously heterogeneous enhancement on MRI. The lesions showed isointensity ( n=14), hypointensity ( n=8) or hyperintensity ( n=1) on T 1WI, and hyperintensity ( n=15) or isointensity ( n=8) on fat-suppression T 2WI. The ADC values of the lesions were (1.18±0.20)×10 -3 mm 2/s. A characteristic "point-to-plane" progressive enhancement pattern displayed on DCE-MRI, with type Ⅰ TIC (persistent pattern) presented. Conclusion:The imaging characteristics of FNVM are honeycomb-like bone changes on HRCT and"point-to-plane"progressive enhancement pattern on DCE-MRI.

Objective: To evaluate the effectiveness and safety of the endoscope combined with microscope for the microvascular decompression in hemifacial spasm. Methods: A total of 26 patients underwent endoscope combined with microscopic facial nerve microvascular decompression through retrolabyrinthine approach from January 2013 to December 2016 were retrospectively reviewed in Ear Institute, Shanghai Jiaotong University School of Medicine. Among them, 9 were male and 17 were female, with a mean age of (51.9±11.4) years;15 cases of left side and 11 of right side patients were followed up for 1-3 years. The pre-and post-operative Cohen Classification was used for hemifacial spasm, House-Brackmann Grade for facial nerve function, hearing level and complication rates were reviewed. SPSS 19.0 software was used to analyze the data. Results: All 26 patients were operated successfully. No recurrence was seen during 1-3 year follow-up. Post-operative Cohen Grade were as follows: 25 cases with Cohen Grade I and 1 case with Cohen Grade II. The difference in Cohen grade between pre-and post-operative was statistically significant (Z=-4.87, P<0.01). Post-operative facial nerve function was satisfactory in all patients (House-Brackmann Grade I-II in all patients). No hearing loss was observed. No facial paralysis and other lower cranial nerve dysfunction were observed. No postoperative complications such as cerebrospinal fluid leakage occurred. Conclusions Using an angled endoscope combined with microscope in microvascular decompression in hemifacial spasmis is safe and effective.

Objective To investigate the diagnostic value of endotoscopy,tympanometry,pure tone audiometry (PTA) and high resolution temporal bone CT in children with secretory otitis media (SOM).Methods The study comprised 414 ears in 207 children with adenoid hypertrophy.Endotoscopy,tympanometry,PTA and high resolution temporal bone CT were conducted to evaluate the function of middle ear.Based on the imaging findings of tympanic cavity fluid by high resolution temporal bone CT,the diagnostic accordance rate of endotoscopy,tympanograms and PTA for SOM were studied.Results The diagnostic accordance rate of endotoscopy,tympanometry and PTA for SOM were 98.79％(409/414),98.07％ (406/414) and 88.41％ (366/414),respectively.The sensitivity of the three examinations were 99.20％(124/125),100％ (125/125),63.20％ (79/125),respectively.The specificity of the three examinations were 98.62％ (285/289),97.23％ (281/289),99.31％ (287/289),respectively.The diagnostic accordance rate of endotoscopy combined with tympanometry for SOM was significantly higher than any single examination or any combined examinations.Conclusion Endotoscopy combined with tympanometry shows a great value in diagnosing SOM,which is accurate and safe.

OBJECTIVE To explore the significance of intraoperative auditory monitoring(IAMA) in surgery of acoustic neuroma and to compare the value of auditory brainstem response(ABR) and cochlear nerve action potential(CNAP) in auditory monitoring.METHODS Retrospective analysis of 12 cases of acoustic neuroma from January 2016 to December 2016 was performed.All patients have a practical hearing(AAO-HNS,grade class a,b),the ABR waveform can be elicited,wave v differentiation,All tumors were removed via posterior sigmoid sinus approach.RESULTS ABR waveform of all patients were prolonged with different degrees of change(0.68±0.41) ms compared with the preoperative data.Amplitude of CNAP diverse in different individuals,with an average prolong compared to the data before operation(0.25±0.16) ms.In all 12 cases,8 (66.7％) patients remained usable hearing after the operation,4 cases(33.3％) failed to have a usable hearing.Among these 4 patients,3 showed disappearance of wave v,1 patient showed wave v latency prolong in the ABR,meanwhile,2 patients showed P1 dissapear,2 patients showed P1 latency prolong in CNAP.The intraoperative auditory monitoring could play a role in preventing the hearing damage in the procedure.Drilling,noise,surgical nerve stretch or thermal injury may cause the hearing damage.A 5 minutes pause could get some degree of regain,with the amplitude rise again.CONCLUSION A combination use of the ABR and CNAP monitoring has a certain significance in surgery of acoustic neuroma.ABR waveform is stable and reliable,but costs longer time;CNAP stack quickly and improve monitoring sensitivity,but waveform varies.Vibration and noise caused by drilling,nerve stretch during operation and heat damage can be monitored timely.Combined use of ABR and CNAP monitoring can enhance the auditory preservation rate during acoustic neuroma surgery.

OBJECTIVE To evaluate the application ofretrolabyrinthine approach in cerebellopontine angel(CPA) region surgeries.METHODS A total of 42 patients underwent microscopic-endoscopic cooperative surgeries for CPA lesions via retrolabytinthine approach were reviewed.They were hospitalized from January 1st 2011 to January 1st 2016 in our institution.The microscopic-endoscopic technique was applied in all surgeries.RESULTS Complete removal was obtained in all tumor resections and satisfactory symptom-relief was achieved in all microvascular decompressions and neurectomies.In cases with acoustic neuroma and cholesteatoma,useful hearing (AAO-HNS classes A,B and C) was obtained in 66.7％(6/9) patients.No facial paralysis and postoperative complications such as cerebrospinal fluid leakage occurred during follow-up.In cranial nerve rhizopathies cases,complete relief was achieved in all patients.No facial paralysis and other lower cranial nerve dysfunction occurred.CONCLUSION With the preservation of labyrinthine structures,the microscopic-endoscopic cooperative surgeries for CPA lesions via retrolabytinthine approach could increase the hearing preservation level without facial nerve injury.Retrolabyrinthine approach is a good option for cranial nerve rhizopathies and tumors smaller than 15 mm in diameter without involvement of the fundus of internal auditory canal in the CPA region.

OBJECTIVE To analyse the diagnosis of non-acoustic lesions in internal auditory canal(IAC) and cerebellopontine angle(CPA),and discuss the effects of imaging examinationon differential diagnosis.METHODS A retrospective study was carried out in 185 patients with non-acoustic lesions of IAC and CPA from January 2001 to December 2016,in which lower cranial schwannoma,facial nerve tumor,meningioma,cholesteatoma,malignant tumor,trigeminal schwannoma,cavernous hemangioma,arachnoid cyst,lipoma were diagnosed in 65,55,25,9,9,8,6,5 and 3 patients,respectively.All patients were performed surgeries,and preoperative diagnosis and surgical approach were decided according to clinical manifestation,CT and MRI findings.Postoperative diagnosis were confirmed by pathological examination.RESULTS Total removal of lesion was achieved in 185 patients [96 men and 89 women,11-77 years old,mean age (46.4±25.6) years] and mean follow-up time was (5.1 ±3.2) years.Postoperative complication was leakage of cerebrospinal (4 patients,2.2％).4 patients with malignancy were dead and 1 patient with cholesteatoma was found recurrent during the follow-up.All kinds of non-acoustic lesions in IAC and CPA had its characteristic imaging findings.Preoperative diagnostic accuracy rate was 93.5％(173/185).CONCLUSION Accurate preoperative diagnosis has great significance in developing treatment strategy for IAC and CPA lesions;Preoperative diagnosis should be achieved according to clinical manifestation,CT and MRI findings,which can significantly improve the accuracy rate of diagnosis.

Objective To investigate the clinical and genetic characteristics of a large family with late-onset,progressive autosomal dominant non-syndromic hearing loss.Methods Collections of detail history hereditary features,physical and audiological examination were performed.After mutation screening of GJB2,SLC26A4,MTRNR1 (12SrRNA) genes by Sanger sequencing,the proband was investigated by targeted next-generation sequencing of 79 deafness genes.Results This family included seven generations and 73 members.Eleven persons with hearing loss and 11 normal-hearing persons participated in this study.All affected members but one exhibited late-onset,progressive non-syndromic sensorineural hearing loss; the ages of onset were between 9 and 30 years.Mutation screening by sanger-sequencing and targeted next-generation sequencing excluded the possibility of pathogenic mutations within known deafness gene.Conclusions A Chinese family with late-onset progressive non-syndromic sensorineural hearing loss was investigated clinically and genetically.By candidate gene approach and targeted next-generation sequencing,this family was preliminary proved to be caused by unknown deafness gene.