ImportanceOpening the cochlear‒carotid recesses through the endoscope-assisted modified translabyrinthine approach to treat lesions in the petrous apex has been demonstrated to preserve the cochlea and hearing. The objective parameters of recesses and their relationships with vital structures were fundamental to the effective application of this technique.ObjectiveHerein, we present a quantitative analysis of cochlear‒carotid recesses on high-resolution computed tomography (HRCT) images and discuss their implications for hearing preservation in the surgical management of massive petrous bone cholesteatomas (PBCs).DesignObservational study.ParticipantsTwenty samples (40 sides) obtained between April and June 2021 radiographically via HRCT.MethodsHRCT images of 40 ears were acquired at 0.60 mm thickness and processed via Mimics. The intrinsic structures of the temporal bone were reconstructed in a 3D view. The morphological data were precisely measured on multiplanar reconstructed images. In addition, clinical implications were demonstrated in one patient with massive temporal bone cholesteatoma.ResultsA 3D model of the cochlear‒carotid recesses was reconstructed. The recesses were essentially divided into 2 parts, namely, the triangle anterior and superior to the cochlea (TASC) and the triangle anterior and inferior to the cochlea (TAIC). The size of the cochlear‒carotid recesses varied widely among the samples. The TASC was accessible in 80% of the samples via a conventional ear endoscope with a diameter of 3 mm, while the applicability of the TAIC was 62.5%. We further discovered that the low position of the trigeminal nerve impression and the small distance between the cochlea and the jugular bulb suggested narrow recesses. The thickness of the compact bone shell of the cochlear lumen acted as a safe drilling divider.ConclusionsHRCT and 3D reconstruction clearly displayed cochlear‒carotid recesses. For patients with massive PBC, the use of an endoscope to open recesses is feasible in the majority of cases and increases the possibility of preserving the structure and function of the cochlea. Individualized preoperative measurements of cochlear‒carotid recesses via HRCT could help surgeons perform safe and accurate dissection.

OBJECTIVE:To investigate the audiological characteristics of vestibular schwannoma (VS) patients with normal pure-tone audiometry (PTA) results. STUDY DESIGN:A retrospective study. SETTING:Forty-two VS patients with normal PTA results from October 2016 to October 2022 were included. METHODS:Normal PTA was defined when the hearing threshold is ≤25 dB hearing loss (HL) in each test frequency and the PTA is ≤25 dB HL. Results of multiple audiological tests such as the auditory brainstem response (ABR), distortion product otoacoustic emission (DPOAE), multiple auditory steady-state responses threshold (ASSR), and speech discrimination score were retrospectively reviewed. Demographic data of these patients were also been collected. RESULTS:According to our results, the ABR and average ASSR threshold of the affected side were statistically significantly higher in VS patients with normal PTA. ABR waveforms on the affected side also showed more abnormalities. The DPOAE pass rates of the affected side were lower than the unaffected side while the amplitude and signal-to-noise ratio rate was also lower. In addition, we used magnetic resonance imaging 3-dimensional reconstruction images to measure the volume of tumors in these patients. We also found that higher ABR threshold means lager tumor size in patients with normal PTA. CONCLUSION:VS patients with normal PTA result cannot be assumed to have no impairment of hearing function. ABR, DPOAE, and ASSR results showed the characteristic changes in the affect ear. ABR threshold has the highest sensitivity for hearing abnormalities and is strong relative with tumor size in patients with normal PTA.

Objective: Hearing performance after myringoplasty with full-thickness tragal cartilage (FTTC) has been questioned because a considerable difference exists between the graft and natural tympanum. This study aimed to analyze the air-bone gap (ABG) trends before and after myringoplasty and its interaction with risk factors in the FTTC group; further, it compared postoperative ABG in the FTTC group to that in the temporalis fascia (TF) and partial-thickness tragal cartilage (PTTC) groups. Design: A retrospective cohort study Setting: Tertiary care University Hospital Methods: The general linear model repeated measures was used to analyze ABG trends in the FTTC group and its influencing factors. Non-parametric tests were used to compare ABGs in the FTTC group and the other two groups. Propensity score matching was done to balance baseline characteristics between the TF and FTTC group. Results: The mean postoperative ABG in the FTTC group was 12.17±6.58 dB, and 90.9% of the patients had a mean ABG within 20 dB, which improved significantly after surgery (F(1,108)=38.707, P<.001). The tympanic perforation size and malleus handle exposure status, which significantly affected the preoperative ABG, did not affect postoperative hearing (P>.05). In the FTTC group, the mean ABG was comparable to that in the other two groups (P>.05). For all except 250 Hz, a similar audiological performance was observed in the FTTC and PTTC groups; FTTC had a better performance at 250 Hz (Z=-2.277, P=.023). Conclusions: Good hearing performance was achieved after myringoplasty with FTTC, irrespective of the preoperative perforation size and malleus handle exposure status.

Objective:To retrospectively analyze the effectiveness of transcranial facial nerve bridging in the treatment of facial nerve dysfunction. Methods:A retrospective analysis was conducted on 27 patients with facial nerve dysfunction who underwent transcranial facial nerve bridging at the Eye, Ear, Nose, and Throat Hospital affiliated with Fudan University from 2017 to 2022. The main collected data includes the patient's age, gender, primary lesion, damaged location, interval from facial paralysis to surgery, and preoperative and postoperative House-Brackmann（HB） scale for facial nerve function. Statistical comparisons were made between the average HB level of patients before and after surgery. Results:A total of 27 patients included 17 males and 10 females. The average age of patients during surgery is（42.50±3.38） years old. Primary lateral skull base diseases include trauma（n=3）, tumors（n=22）, and infections（n=2）. The duration of facial paralysis varies from 6 months to 5 years. Statistics analysis has found that the average postoperative HB score of patients who underwent transcranial facial nerve bridging was significantly lower at（3.750 ± 0.183） compared to preoperative（4.875±0.168）. The proportion of patients with good facial nerve function increased significantly from 7.4% before surgery to 42.9% after surgery. Conclusion:Transcranial facial nerve bridging surgery with interpositional graft has a significant effect on improving facial nerve function in patients with facial nerve injury. Further research is still needed to evaluate the long-term effectiveness of this surgery, to determine the optimal patient selection criteria and postoperative rehabilitation strategies.

Background Treacher Collins syndrome (TCS; OMIM 154500) is a craniofacial developmental disorder. Methods To investigate the genetic features of a four-generation Chinese family with TCS, clinical examinations, hearing tests, computed tomography, whole-exome sequencing (WES), Sanger sequencing, reverse transcription (RT)-PCR, and the Minigene assay were performed. Results The probands, an 11-year-old male and his cousin exhibited typical clinical manifestations of TCS including conductive hearing loss, downward slanting palpebral fissures, and mandibular hypoplasia. Computed tomography revealed bilateral fusion of the anterior and posterior stapedial crura and malformation of the long crura of the incus. WES of both patients revealed a novel heterozygous intronic variant, i.e., c.4342 + 5_4342 + 8delGTGA (NM_001371623.1) in TCOF1 . Minigene expression analysis revealed that the c.4342 + 5_4342 + 8delGTGA variant in TCOF1 caused a partial deletion of exon 24 (c.4115_4342del: p.Gly1373_Arg1448del), which was predicted to yield a truncated protein. The deletion was further confirmed via RT-PCR and sequencing of DNA from proband blood cells. A heterozygous variant in the POLR1C gene (NM_203290; exon6; c.525delG) was found almost co-segregated with the TCOF1 pathogenic variant. Conclusions In conclusion, we identified a heterozygous TCOF1 splicing variant c.4342 + 5_4342 + 8delGTGA (splicing) in a Chinese TSC family with ossicular chain malformations and facial anomalies. Our findings broadened the spectrum of TCS variants and will facilitate diagnostics and prognostic predictions.

Purpose: Glomus tympanicum tumors are benign primary tumors of the middle ear that can be completely removed using modern surgery. We compared endoscopic ear surgery (EES) to traditional microscopic ear surgery (MES) in terms of the removal of early-stage glomus tympanicum tumors. Methods: We retrospectively reviewed 25 cases treated from 2003 to 2021 that were of Grade I or II based on the Glasscock–Jackson classification system. Overall, 18 cases underwent MES: 8 via trans-tympanic bone and 10 via canal-wall-down or canal-wall-up tympanomastoidectomy (CWDT or CWUT) and 7 underwent EES. We compared surgery durations, the lengths and costs of hospitalization, postoperative complications, and relapse rates between the two groups and among the three specific operation ways. Results: The postoperative follow-up period ranged from 1 to 19 years. There was no between-group difference in operative time or the length or cost of hospitalization. Operative time and cost of hospitalization did not show a statistically significant correlation to the three surgical procedures, whereas it was found that the group of MES via the trans-tympanic bone had shorter length of hospitalization when compared with CWUT or CWDT group. All tumors were completely resected; pulsatile tinnitus improved in all patients, and there was no major complication. Two patients who underwent CWUT or CWDT (one each) relapsed; no patient relapsed in the EES group. Conclusion: MES via the trans-tympanic bone and EES via the ear canal safely and reliably remove early-stage tumors without excessive patient discomfort.

-OBJECTIVE: To report the long-term outcome of delayed facial nerve paralysis (DFNP) after surgical resection of vestibular schwannoma and evaluate the influence of various factors on the prognosis of facial nerve function.-METHODS: Of 265 patients who underwent surgical excision of VS through a retrosigmoid approach between April 2019 and October 2021, 15 (5.7%) developed DFNP and were retrospectively studied. Preoperative and post-operative data were collected and analyzed. -RESULTS: The mean age of patients with DFNP was 42.6 years (range, 27-63 years), and 11 (73.3%) were male. Tumor size ranged from 12 to 37 mm (mean 24 mm) in largest dimension. All patients had normal (House-Brackmann [HB] I) facial nerve function preoperatively. Immediate postoperative facial nerve function was HB I in 12 patients (80%) and HB II in 3 patients (20%). The mean severity of DFNP onset was HB 4.7 (range, HB III -V). The average day of onset was postoperative day 12.6 (range, day 5-28). At 1-year follow-up, 12 patients (80%) were HB I, 1 patient (6.7%) was HB III, and 2 patients (13.3%) were HB IV. All patients who were HB III and IV at the last follow-up had immediate postoperative facial nerve function of HB II. -CONCLUSIONS: Most patients who develop DFNP have a favorable prognosis. However, a small proportion of pa-tients with deteriorated facial nerve function immediately after surgery have poor long-term outcomes, despite confirmation of their facial nerve integrity anatomically and by electrical stimulation.

Background: Vestibular schwannomas (VSs) typically originate in the superior vestibular nerve or inferior vestibular nerve. Given the potential impact on a patient's hearing and associated surgical risks, preoperative differentiation of a tumor's origin between the inferior and superior vestibular nerves can influence treatment strategies and optimal therapeutic and counseling approaches.Methods: 120 patients diagnosed with VS at the Eye and ENT Hospital of Fudan University were enrolled and randomly assigned to a training set or a validation set. T-tests and chi-square tests selected the variables. The variables were further screened using a bidirectional stepwise regression model based on the Akaike information criterion and the Least Absolute Shrinkage and Selection Operator (LASSO) regression. Sensitivity, specificity, predictive values, and likelihood ratios assessed model accuracy. Receiver operating characteristic curve (ROC) analysis maximized the Youden index to determine the optimal cutoff value. Decision curve analysis calculated clinical utility.Findings: We selected the model obtained by LASSO regression after accuracy testing and external validation. The variables included dural fullness, hearing loss, pure tone audiometry (PTA), and auditory steady-state evoked response (ASSR). The best cutoff value for this model was 0.333, and the area under the ROC curve was 0.839. The sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios were 0.800, 0.846, 0.839, 0.809, 5.20, and 0.236, respectively. Interpretatio : In this study, we built a clinical model for predicting VS patients’ neural origin. Our model has significant advantages compared to vestibular-evoked myogenic potentials (VEMP), the traditional predictor of neural origin.Funding: National Natural Science Foundation of Shanghai (21ZR1411800), and Excellent Doctors- Excellent Clinical Researchers Program (SYB202008).Declaration of Interest: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.Ethical Approval: The ethics committee of the EYE & ENT Hospital of Fudan University approved this study, and all participants signed an informed consent form.

Objective: To investigate the technique and efficacy of fully endoscope resection of intralabyrinthine schwannomas (ILS) by transcanal transpromontorial endoscopic approach (TTEA). Study Design: Retrospective case review. Setting: Hospital. Patients: All patients who were affected by ILS, without extension to the internal auditory canal and underwent surgery with TTEA in our hospital in 2020. Intervention(s): Therapeutic. Main Outcome Measure(s): Recovery status, postoperative complications and remaining symptoms after surgery. Results: Three patients were included, all of which underwent gross total resections. The follow-up period was from 10 months to 2 years. No intraoperative and postoperative major complications were observed. There was no facial paralysis or cerebrospinal fluid leakage postoperatively. The hospitalization time of TTEA was 5 days. Three patients' vertigo was relieved after 1 week without receiving vestibular therapy. Only 1 patient complained of transient episodes of vertigo when climbing or holding heavy objects. Conclusions: TTEA has the advantages of clear vision to identify the anatomical structure, enabling complete tumor resection, reduced operation time, and faster postoperative recovery.Level of Evidence: IV

Objective Papillomas originating from the Schneiderian epithelium within the middle ear are extremely rare and may be associated with a high rate of recurrence and malignant transformation. Oncocytic papillomas represent the rarest pathological subtype of such tumors. The current investigation aimed to determine whether there exists a distinct mechanism underlying the incidence of oncocytic papillomas arising primarily within the middle ear, and to explore potential treatment strategies to ensure complete removal and prevent recurrence. Study Design Search of the English literature for cases of middle ear papilloma and RNA sequencing analysis of three samples from one new case presenting at the Eye and ENT Hospital, Fudan University (Shanghai, China), with recurrent middle ear oncocytic papilloma, along with two normal mucosal samples. Setting Academic, tertiary referral hospital. Patient and interventions The patient underwent open mastoidectomy and endoscopic tympanoplasty twice in 6 years. Histopathology confirmed oncocytic papilloma in middle ear. The patient has been free of the disease at 18 months of follow-up without radiation, whereas the RNA-seq analysis of the samples in endoscopic operations remained nonmalignant. Results Only four cases of primary middle ear oncocytic papillomas have been reported. Recurrent masses usually originate from around the eustachian tube, which may explain the pathogenesis of this lesion. RNA-seq analysis was used to identify 1,317 (UP, 239; DOWN, 1078) differentially expressed genes between papillomas and normal mucosa. The involvement of some hub proteins (e.g., FN1, CXCL8, L10, JUN, and FOS) in the pathogenesis of primary middle ear papillomas was found to align with the observed clinical features. Conclusion The middle ear oncocytic papillomas were extremely rare and remained incompletely understood. The findings of this first RNA-seq analysis of this rare tumor may serve to enhance comprehension of and aid in the management of middle ear papillomas.

Tumors originating from the facial nerve are relatively rare and primarily include three types: facial nerve schwannoma, facial nerve neurofibroma, and facial nerve hemangioma. The Facial Nerve Tumor Diagnosis and Treatment Consensus Writing Group of China has established the current consensus after multiple discussions, based on a review of domestic and international literature and taking into account the domestic situation. This consensus systematically elucidates the epidemiology, pathological characteristics, and main clinical manifestations of facial nerve tumors. It establishes diagnostic criteria for facial nerve tumors, clarifies the anatomical classification of facial nerve tumors, and outlines its main differential diagnoses. Furthermore, the consensus clearly defines the principles of applying different treatment methods for facial nerve tumors and outlines a concise workflow for the diagnosis and treatment of facial nerve tumors.

髓鞘是高等脊椎动物中实现快速、精准神经信号传递的重要结构,是形成和维持神经元网络之间快速、协调交流的关键组成部分,其对于听觉神经功能的正常运行起到了重要作用.近些年来,学术界越来越意识到,髓鞘的动态变化是听觉神经元系统病理和可塑性机制中重要环节.本文旨在通过回顾中枢和周围听觉系统中的髓鞘相关病理机制,同时综述听觉活动、脑源性神经营养因子BDNF、ERK1/2信号通路、髓鞘相关抑制因子No-go-A等因素与听觉神经系统髓鞘可塑性之间的关系.

Background Hereditary hearing loss is a heterogeneous class of disorders that exhibits various patterns of inheritance and involves many genes. Variants in the EYA4 gene in DFNA10 are known to lead to postlingual, progressive, autosomal dominant nonsyndromic hereditary hearing loss. Patients and methods We collected a four-generation Chinese family with autosomal-dominant nonsyndromic hearing loss (ADNSHL). We applied targeted next-generation sequencing (TNGS) in three patients of this pedigree and whole-genome sequencing (WGS) in the proband. The intrafamilial cosegregation of the variant and the deafness phenotype were confirmed by PCR, gap-PCR and Sanger sequencing. Results A novel CNV deletion at 6q23 in exons 8–11 of the EYA4 gene with a 10 bp insertion was identified by TNGS and WGS and segregated with the ADNSHL phenotypes . Conclusions Our results expanded the variant spectrum and genotype‒phenotype correlation of the EYA4 gene and autosomal dominant nonsyndromic hereditary hearing loss in Chinese Han individuals. WGS is an accurate and effective method for verifying the genomic features of CNVs.

OBJECTIVE: We sought to report the outcome of resection of vestibular schwannomas (VSs) originating from the inferior vestibular nerve that extended into the fundus of the internal auditory canal through the middle cranial fossa (MCF) approach with endoscopic assistance. METHODS: Seven patients with VSs who underwent resection through MCF approach with endoscope assistance and 12 patients with conventional microsurgery in our department were enrolled in this study. These patients' characteristics were acquired, and the surgical outcomes, as well as postoperative complications, were evaluated. RESULTS: In the endoscope group, the VS was first removed by a conventional microscopic procedure and then an endoscope was used to find and remove any residue lesions. In 3 of 7 patients, residue tumor in the space below transverse crest was found and completely removed under endoscope. Six of 7 patients (85.7%) had House-Brackmann grade I at the time of last follow-up in the endoscope group compared with 10 of 12 patients (83.3%) in the conventional microsurgery group. Serviceable hearing was successfully maintained in 2 of 3 patients (66.7%) in the endoscope group compared with 6 of 10 patients (60`)/0) in the conventional microsurgery group. Total resections were achieved in all the patients (100%) in the endoscope group compared with 9 of 12 patients (75%) in the conventional microsurgery group. CONCLUSIONS: Using an endoscope in VS resection through the MCF approach could facilitate complete removal of the lesion while minimizing the risk of hearing loss and facial paralysis. The endoscope-assisted MCF approach is especially suitable for removing an intracanalicular VS with lateral extension involving the space below the transverse crest.

资料 患者男性,19岁.发现右颈部肿物3年.体格检查:右颈部肿块质韧、界清,不能推动,无压痛,大小约 3 cm×4 cm.无搏动性耳鸣,无面神经及后组脑神经受累征象,无其他阳性体征.颞骨磁共振成像:左侧颈静脉孔区及右侧颈动脉分叉处富血供肿块占位.颈动脉CT血管造影(CT angiography,CTA):右侧颈动脉体瘤(carotid body tumour, CBT),左侧颈静脉球体瘤可能性大(图1).综合上述症状及检查,术前诊断为右侧CBT、左侧颈静脉球体瘤.患者存在双侧病灶,考虑病情的轻重缓急,拟先行右侧CBT切除术.

BACKGROUND:Facial nerve schwannomas located at internal auditory canal and cerebellopontine angle (IAC/CPA FNS) were diagnosed intraoperatively, it poses a therapeutic dilemma to the surgeon.OBJECTIVE:To report our experience in managing IAC/CPA FNS and to propose a treatment strategy.METHODS:A total of 14 patients with IAC/CPA FNS who were diagnosed intraoperatively and treated by operation between 2015 and 2019 were retrospectively studied.RESULTS:Unilateral hearing loss was the most common symptom and all these patients had normal facial nerve function preoperatively. Surgical approaches used in these patients including translabyrinthine (2 cases), retrosigmoid (RS) (11 cases), and middle cranial fossa (MCF) approach (1 case). Eight patients underwent partial resection, three patients underwent subtotal resection and three patients had complete tumor removal with facial nerve reconstruction. All partial resection patients and two patients underwent subtotal resection achieved a long-term HB grade I facial nerve function. The long-term facial nerve function of patients underwent complete resection and nerve grafting was no better than HB grade III.1 of the eight patients underwent partial resection experienced tumor regrowth during the follow-up.CONCLUSIONS:Partial or subtotal resection for IAC/CPA FNS may provide an opportunity of retaining excellent facial nerve function. Regular postoperative imaging is helpful to monitor the recurrence.

Endoscopic middle ear surgery is a widely employed minimally invasive surgical technique to address middle ear and mastoid pathology. Bone drilling is the main technical challenge of endoscopic middle ear surgery. The accompanying video describes the detailed protocol of a constant-suction bone-drilling technique and the procedure of endaural exclusive endoscopic atticoantrotomy (retrograde mastoidectomy) using this technique. The main components of this bone-drilling technique include a soft and flexible suction tube, which is placed into the tympanic cavity to provide constant suction, and a soft sleeve, which is wrapped around the drill shaft to prevent the high-speed rotating shaft from damaging the lens of the endoscope. With these simple modifications, the traditional otological electrodrill can be used for drilling a tiny endaural incision in endoscopic middle ear surgery. Based on this bone-drilling technique, endaural endoscopic atticoantrotomy (retrograde mastoidectomy) can be successfully established for the removal of various amounts of bone, depending on the extent of the lesion. The short-term postoperative outcome seems promising.

目的 探讨开放式乳突切开术联合鼓室成形术在慢性化脓性中耳炎及中耳胆脂瘤治疗中的应用及疗效.方法 选择江苏省昆山市第一人民医院耳鼻喉科于2017年1月～2019年1月期间行开放式乳突切开术联合鼓室成形术的慢性化脓性中耳炎及中耳胆脂瘤患者71例,统计术后干耳时间、听力情况,按照听力重建方式不同进行回顾性分析.结果 71例患者术后平均干耳时间为(27.96±5.97)d.其中行自体材料听力重建30例、人工钛听小骨听力重建41例.自体材料组术前平均听阈为(55.31±18.01)dB HL,术后6个月为(44.00±19.05)dB HL;人工钛听小骨组术前平均听阈为(55.44±21.97)dB HL,术后6个月为(40.42±20.23)dB HL,自体材料组及人工钛听小骨组重建听力前后的听阈改变差异均有统计学意义.结论 开放式乳突切开术联合鼓室成形术治疗慢性化脓性中耳炎及中耳胆脂瘤效果明显,乳突缩窄利于干耳,上鼓室加高可以提高上鼓室含气量,提高听力;另外,自体软骨取材方便,适用于经济困难的患者.

INTRODUCTION:Triaging patients into correct severity categories in an emergency department is an advanced skill that depends on a quick assessment after obtaining very little information. The purpose of this study was to assess specific risk factors associated with hospital admissions in the emergency department environment of the specialized Eye, Ear, Nose, and Throat hospital located in Shanghai, China.METHODS:This study was a retrospective cohort study. Patients visiting the emergency department in a tertiary hospital in eastern China from February 2008 to August 2015 were included. Univariate and multivariate analyses were used to identify the risk factors related to hospital admissions. Combining variables calculated from the regression equation of multivariate analysis (binary logistic regression analysis) enabled the risk factors quantification. The receiver operating characteristic analysis was used to identify the most informative cutoff point of the combining predictors.RESULTS:A total of 188715 patients were enrolled in the study. Of them, 8395 patients (4.4%) required hospital admission. Hour of visit, season, age, sex, chief complaint, anatomical location, and locale of patients were independent risk factors of hospital admission by univariate and multivariate analysis. Combining predictors were calculated from the equation of the multivariate logistic model. The area under the curve of the combining predictors was 0.949, and the 95% confidence interval was 0.947 to 0.951 (P <.001), with a sensitivity of 95.2% and a specificity of 85.6%. A cutoff score of less than -35.1975 was associated with hospital admission.DISCUSSION:This study provided a method to build a feasible predictive model of hospital admission during triage. Understanding risk factors is an important part of the triage process in order to correctly assign priorities to the patients served. The outcomes of this study would add additional information for the triage nurse to consider in assessing the patient and assigning acuity ratings. The model developed here requires validation in future research.

OBJECTIVES:To identify genes that are related to delayed endolymphatic hydrops (DEH) in patients by RNA-Seq analysis.DESIGN:Observational study.SETTING:Eye & ENT Hospital, Fudan University (Shanghai, China).PARTICIPANTS:We collected the entire vestibular system from four patients with DEH who underwent labyrinthectomy. Three control samples were collected from patients with acoustic neuroma or facial neuroma treated via the translabyrinthine approach. High-throughput RNA-Seq analysis was performed to investigate gene expression in the pathological vestibular system.MAIN OUTCOME MEASURES:Our bioinformatic analysis identified 17 genes that were upregulated and eight genes that were downregulated in patients with DEH compared with the controls.RESULTS:The altered gene expression profile suggested that DEH is closely related to neuropathy and autoimmune disease. In addition, many of the differentially regulated genes were involved in cell adhesion, suggesting a role of cell adhesion in DEH. Immunofluorescence analysis confirmed the expression of PMP2 and CLDN19 in the cytoplasm of hair cells and scattered expression of MPZ at cell junctions. The protein expression levels were higher in specimens from patients with Ménière's disease and DEH compared with controls.CONCLUSIONS:The protein expression profile of vestibular organs in patients with endolymphatic hydrops exhibited a degree of similarity to that of Ménière's disease. Endolymphatic hydrops is characterised by autoimmune abnormalities. DEH and Ménière's disease are likely to be different manifestations of the same disease, with disparate clinical symptoms. RNA-Seq is a useful analytical tool to characterise the vestibular pathology based on its transcriptome.