Combined microscope-endoscopy resection of petrous bone cholesteatoma with temporary facial nerve transposition versus nontransposition.

PURPOSE: The narrow supralabyrinthine space affects surgical procedures. To study the effect of temporary transposition of geniculate ganglion of facial nerve versus nontransposition on lesion recurrence and facial nerve function in patients with petrous bone cholesteatoma. METHODS: A total of 18 patients with petrous bone cholesteatoma involving the facial nerve were treated in our hospital from November 2016 to March 2023. The main surgical method is the extended supralabyrinthine approach assisted by a microscope and an endoscope. We collected and retrospectively analyzed their medical records. RESULTS: Temporary facial nerve transposition was performed in five patients, and nontransposition was performed in 13 patients. Cholesteatoma recurred in three patients with facial nerve nontransposition, whereas none in patients with facial nerve transposition. In this study, except for one case with a second operation, postoperative facial paralysis in other cases was improved to varying degrees, and there was no significant difference between the two groups. CONCLUSION: Temporary transposition of geniculate ganglion of facial nerve will not affect the postoperative nerve function of patients and can reduce the possibility of cholesteatoma recurrence of the petrous bone.

Drug screening identifies aldose reductase as a novel target for treating cisplatin-induced hearing loss.

Cisplatin is a frequently used chemotherapeutic medicine for cancer treatment. Permanent hearing loss is one of the most serious side effects of cisplatin, but there are few FDA-approved medicines to prevent it. We applied high-through screening and target fishing and identified aldose reductase, a key enzyme of the polyol pathway, as a novel target for treating cisplatin ototoxicity. Cisplatin treatment significantly increased the expression level and enzyme activity of aldose reductase in the cochlear sensory epithelium. Genetic knockdown or pharmacological inhibition of aldose reductase showed a significant protective effect on cochlear hair cells. Cisplatin-induced overactivation of aldose reductase led to the decrease of NADPH/NADP+ and GSH/GSSG ratios, as well as the increase of oxidative stress, and contributed to hair cell death. Results of target prediction, molecular docking, and enzyme activity detection further identified that Tiliroside was an effective inhibitor of aldose reductase. Tiliroside was proven to inhibit the enzymatic activity of aldose reductase via competitively interfering with the substrate-binding region. Both Tiliroside and another clinically approved aldose reductase inhibitor, Epalrestat, inhibited cisplatin-induced oxidative stress and subsequent cell death and thus protected hearing function. These findings discovered the role of aldose reductase in the pathogenesis of cisplatin-induced deafness and identified aldose reductase as a new target for the prevention and treatment of hearing loss.

Clinical correlation research of 3D reconstruction of retrolabyrinthine space based on HRCT of temporal bone.

Background: The retrolabyrinthine approach helps clinicians perform complex surgeries such as vestibular neurectomy, resection of petrous apex cholesteatoma, or use this space to complete endoscopic combined with microscope surgical operations in a relatively safe buffer space. Some of our current studies using 3D reconstruction in the clinic have also helped us perform some complex surgical procedures.Objective: This study aims to reveal the relationship between important structures in retrolabyrinthine space through objective parameters. These measurement data help clinicians locate intraoperatively and provide a reference for clinical surgery. Also, we are intended to help improve surgical techniques and expand the operating space to increase reachable anatomic structure.Material and Methods: The inner structures of the temporal bone from HRCT (High-resolution computed tomography) images which were taken at the Eye & ENT Hospital of Fudan University were reconstructed. Precise measurement of the structures was accomplished by using the software 3D-Slicer (3D Slicer, https://www.slicer.org/; version 4.8.0, Massachusetts, USA).Results: 3D model of temporal bone structures, including the cochlea, semicircular canals (SCCs), the internal auditory canal (IAC), facial nerve (FN), jugular bulb(JB), and carotid artery was reconstructed. The combination of HRCT and 3D models is utilized to analyze the Quantitative data of the retrolabyrinthine space and its adjacent structures.Conclusions and Significance: 3D reconstruction of CT images clearly displayed the detailed structures of the temporal bone. Surgical adaptability of the retrolabyrinthine approach can be assessed preoperatively by image and other methods, and anatomical parameters play an important role in the retrolabyrinthine space. Therefore, this study helps to skeleton the bone as much as possible to expand the surgical space, so that the surgeon can contact the anatomical structure more diversified to expand the surgical indications.

Surgical Techniques and Functional Reconstruction for Complex Tenosynovial Giant Cell Tumor of Temporal Bone and Middle Skull Base.

Objectives: Temporal tenosynovial giant cell tumors (TGCTs) are often large and have invaded the middle skull base. It is difficult to protect the important neurovascular structures around the tumor and perform functional reconstruction on the basis of complete tumor resection. This study aimed at analyzing the surgical techniques and functional reconstruction during the operation of complex TGCT of temporal bone and middle skull base. Methods: Five patients with pathologically confirmed TGCT of different complex types in the temporal bone and middle skull base were treated in our hospital from December 2020 to February 2023. We collected and retrospectively analyzed their medical records, including medical imaging, surgical procedures, and follow-up data. Results: The tumors invaded beyond the temporal bone and destroyed the middle skull base in all cases, involving the intracranial space and other important neurovascular structures. The internal carotid artery, infratemporal fossa, pterygopalatine fossa, and parapharyngeal space were also involved in 1 case. All the patients' tumors were completely removed, and the operations were performed mainly via modified infratemporal fossa approach, or combined with expanded middle cranial fossa approach. All cases with temporal and skull base destruction were repaired using the temporalis muscle flap with no occurrence of intracranial complications. The dura mater, condyle of temporomandibular joint, facial nerve, and internal carotid artery were fully preserved. Normal maxillofacial morphology was also preserved. The air conductive hearing of 2 patients was preserved. Conclusions: We found and summarized some surgical techniques that can help safeguard the important structures around massive TGCTs of temporal bone and middle skull base, and reconstruct the defects after tumor resection. The techniques are effective and feasible.

Long-Term Hearing Stability at Different Frequencies after Lower Intact Bridge Tympanomastoidectomy and Possible Theoretical Basis.

Objective: We aimed to review the long-term hearing outcomes of intact bridge tympanomastoidectomy (IBM) to discuss hearing preservation and combine the theory of "modified miniature lung," middle ear ventilation, and gas pressure balance theory to explore the possible reasons for long-term stable hearing. Study Design: We designed a collection of patients with chronic suppurative otitis media (CSOM) who underwent IBM and divided them into 2 groups to compare long-term and short-term hearing level. Setting: From April 2007 to July 2017, 102 patients received IBM for CSOM treatment in Eye & ENT Hospital of Fudan University. Methods: We divided the patients into 2 groups according to whether the follow-up period was longer than 6 months. We used the numerical value of the air-bone gap difference as an index to evaluate the degree of hearing recovery. Results: We found that 99/102 (97.1%) of the hearings were improved to more than 3 frequencies. There was no significant difference between long-term hearing level and short-term hearing level, which means IBM could get long-term hearing stability. Conclusion: Intact bridge tympanomastoidectomy could provide significant hearing recovery and long-term hearing stability.

Reconstruction of an orbital defect in rabbits using a silk fibroin-bone microparticle complex.

OBJECTIVE: To construct a silk fibroin-bone microparticle composite based on a porous silk fibroin membrane and to study its feasibility as a material to reconstruct an orbital bone defect. METHODS: A 3D porous silk fibroin membrane scaffold was constructed with a defined pore size and incorporated with bone microparticles from a New Zealand rabbit orbital bone defect. The silk fibroin-bone microparticle composite was then implanted into the orbital bone defect to promote osteogenesis along the surface of the porous silk fibroin membrane. The feasibility of constructing an ideal orbital defect repair material and the silk fibroin-bone micronucleus complex was evaluated by animal experiments, molecular biology, histomorphology, imaging, raw molecular mechanisms, and the biological behavior of the material in vivo. RESULTS: The silk fibroin-bone microparticle composite promotes angiogenesis and osteogenesis to repair bone defects in vivo. Moreover, SF (silk fibroin)/BD (bone dust) complex promotes osteogenesis and angiogenesis by activating FGF2 (Fibroblast Growth Factor 2) and SF scaffolds can bind and restore FGF2. CONCLUSION: Silk fibroin is biocompatible and the silk fibroin-bone microparticle complex successfully repaired orbital bone defects. Additionally, fibroblast growth factor expression around or within the remaining incompletely degraded silk fibroin materials was observed in vivo.

Descending Necrotizing Mediastinitis: Analysis of 9 Cases in Our Hospital.

OBJECTIVES: Descending necrotizing mediastinitis (DNM) is a serious and progressive infection involving the neck and chest and with high mortality if not treated quickly and properly. The aim of this study is to share our practices for managing this condition. METHODS: We retrospectively evaluated 9 patients diagnosed with DNM in our hospital between January 2006 and October 2019. Age, gender, origin of infection, length of hospital stay, microorganisms present, type of surgical treatment, and clinical outcomes were reviewed. RESULTS: All patients underwent surgery to drain neck and mediastinal secretions and collections. Three (33.3%) patients were treated with transcervical drainage alone, and 6 (66.7%) patients were treated with combined transcervical and transthoracic drainage. Reoperations were reported in 3 (33.3%) cases. The average length of hospital stay was 22.78 ± 10.05 days (range: 9-40 days). The average length of intensive care unit stay was 6.44 ± 10.10 days (range: 0-25 days). There were no in-hospital deaths, and all patients were discharged home with good outcomes. CONCLUSIONS: To improve the prognosis of DNM, we suggest early and adequate debridement of all affected areas along with the proper use of antibiotics. A multidisciplinary approach involving both cardiothoracic and ENT surgeons is also required.

Spectrum of SLC20A2, PDGFRB, PDGFB, and XPR1 mutations in a large cohort of patients with primary familial brain calcification.

Primary familial brain calcification (PFBC) is a rare neurodegenerative disorder with four causative genes (SLC20A2, PDGFRB, PDGFB, and XPR1) that have been identified. Here, we aim to describe the mutational spectrum of four causative genes in a series of 226 unrelated Chinese PFBC patients. Mutations in four causative genes were detected in 16.8% (38/226) of PFBC patients. SLC20A2 mutations accounted for 14.2% (32/226) of all patients. Mutations in the other three genes were relatively rare, accounting for 0.9% (2/226) of all patients, respectively. Clinically, 44.8% of genetically confirmed patients (probands and relatives) were considered symptomatic. The most frequent symptoms were chronic headache, followed by movement disorders and vertigo. Moreover, the total calcification score was significantly higher in the symptomatic group compared to the asymptomatic group. Functionally, we observed impaired phosphate transport induced by seven novel missense mutations in SLC20A2 and two novel mutations in XPR1. The mutation p.D164Y in XPR1 might result in low protein expression through an enhanced proteasome pathway. In conclusion, our study further confirms that mutations in SLC20A2 are the major cause of PFBC and provides additional evidence for the crucial roles of phosphate transport impairment in the pathogenies of PFBC.