[Evaluation of the outcomes of cochlear implant in children with auditory neuropathy].

Objective: To investigate the auditory and speech abilities of children with congenital auditory neuropathy (AN) after cochlear implant (CI), and to analyze the role of genetic testing in predicting the postoperative outcomes of CI in AN patients. Methods: Fourteen children diagnosed with AN by audiological battery test and underwent CI surgery in Xijing Hospital of the Air Force Medical University from 2002 to 2021 were included in this study (9 males and 5 females), with an implantation age of (3.1±1.7) years (mean±standard deviation, the same as follows). The preoperative audiological results and deafness gene results were analyzed. Another 52 children with ordinary sensorineural hearing loss (SNHL) were selected as the control group (30 males and 22 females), with an implantation age of (2.2±0.9) years. The demographic factors such as age and gender were matched with those of the AN group. The modified Category Auditory Performance (CAP-Ⅱ) and Speech Intelligence Rate (SIR) were used to evaluate the development of postoperative auditory and speech abilities in two groups. The Mandarin Speech Test System was used to test the speech recognition rate of monosyllabic and disyllabic words and sentences. Matlab 2022 software was used to analyze the data. Results: The results of gene in 14 children with AN showed that 6 cases had OTOF gene mutations, 2 cases (siblings) were confirmed to have TNN gene mutations through whole exome sequencing, and the remaining 6 cases were not find any clear pathogenic gene mutations. All subjects underwent CI surgery with electrodes implanted into the cochlea smoothly, and there were no postoperative complications. After surgery, all AN children had improved auditory and speech abilities, but only 64% (9/14) of AN children with CI had auditory ability scores comparable to the control group of SNHL children (including 2 children with TNN gene mutations), and 36% (5/14) of AN children had lower scores than the control group of SNHL children.The average speech recognition rate of two children with TNN gene mutations was 86.5%, and of two children with OTOF gene mutations was 83.2%. Conclusions: AN children achieved varying degrees of auditory and speech abilities after CI, but the postoperative effects varied greatly. Some children achieved similar results as ordinary SNHL children, but there were still some children whose effects were worse than those of ordinary SNHL children. The postoperative efficacy of CI in two children with AN caused by TNN pathogenic genes were comparable to that of ordinary SNHL in children. Genetic testing had certain reference value for predicting the postoperative effect of CI in AN children.

[HedgeHog inhibitor targeted therapy for periocular basal cell carcinoma: a case report].

A female patient, 87 years old, presented with an irregular swelling in the left medial canthus for 7 years. Due to the patient's poor general condition, radical surgery was not considered appropriate. Surgical biopsy confirmed periocular basal cell carcinoma of the left medial canthus. The patient was administered oral HedgeHog inhibitor targeted therapy for 6 months, resulting in approximately 90% reduction in tumor size. The patient's condition improved, and the tumor remained stable during the course of follow-up.

[A summary of oculoplastic and orbital surgery in China in the past 70 years].

Oculoplastic and orbital surgery is concerned with the occurrence, manifestation, diagnosis, treatment and prevention of the eyelid, orbit and lacrimal diseases, as well as the repair and reconstruction of the related deformities and defects. Since the beginning of the new century, the number of patients with eyelid and orbital diseases has increased continuously, and the team of specialists has expanded rapidly. The development of oculoplastic and orbital surgery in China has entered an all-round new stage, especially with the establishment of Chinese Society of Oculoplastic Surgery and Orbital Disease in 2013. Oculoplastic and orbital surgery in China has made outstanding achievements in strengthening the training of professional talents, promoting interdisciplinary and international exchanges, improving the academic level of basic research, and emphasizing on the research of translational medicine. The technique of ocular plastic surgery has been progressing continuously. Cosmetic surgery of the eye has gained widespread popularity and standardization. Orbital surgery has recorded an innovative, interdisciplinary and rapid growth. International exchanges have helped to expand the international influence and competitiveness. This article is written to celebrate the 70th anniversary of Chinese Journal of Ophthalmology. (Chin J Ophthalmol, 2020, 56: 805-810).

[Seventy years of development and prospects of the ocular oncology specialty in China].

Ocular tumors include intraocular tumors and tumors of the eyelid, orbit, conjunctiva and lacrimal apparatus. After seventy years of continued growth, ocular oncology in China, from small to large and from weak to strong, has made great achievements. Especially since the beginning of the new century, there has been all-round and rapid development. The mechanism of ocular tumorigenesis has been elucidated based on the biobank and animal models. New therapeutic techniques and treatment modalities have been established based on multi-center cohort studies. The team competence and the discipline level have been improved based on academic organization constructions and international exchanges. Looking into the future, ocular oncology in China will move on in gene detection and early diagnosis, basic research and drug targeting, interdisciplinary, intelligent diagnosis and treatment, clinical research and translational medicine, with the support of national strategies and scientific and technological innovation, to further improve the survival rate, the ocular salvage rate and the quality of life for patients with ocular tumors. This article is written to congratulate the Chinese Journal of Ophthalmology on the 70th anniversary of its publication. (Chin J Ophthalmol, 2020, 56: 646-652).

[Frequency properties of bone-conducted vibration and sound-induced ocular evoked myogenic potential].

Objective: The present study was to compare the frequency characteristics of bone- conducted vibration (BCV) and air-conducted sound-induced ocular vestibular evoked myogenic potential(oVEMP) with different stimulation settings, so as to select an optimal setting. Methods: Bruel and Kjaer artificial mastoid was used to calibrate bone vibrator, the mini-shaker. The outputspectrum and transient waveform were recorded when the input frequency was set at different frequencies and data were further analyzed to get the peake quivalent force level value of the output vibration. oVEMP was recorded as we previously reported with normal young adults. Response prevalence, latency, wave interval, amplitude, amplitude ratio, and bilateral amplitude asymmetry ratio were recorded and analyzed. SPSS 24.0 software was used for statistical analysis. Results: Output peak equivalent force level increased as the intensity of primary input stimulation increased at certain frequency,while it decreased as the frequency of primary input stimulation increased at certain intensity. Peak equivalent force level of 500 Hz tone burst (1-2-1) input stimulation was greatest as 139.8 dB when using mini-shaker for vibration stimulation,even greater when using bone conductor B-71.Twenty subjects were elicited with normal oVEMP with N1 and P1 waves in both ears using air-conducted stimulation or bone-conducted vibration.The response rate in these subjects was 95% and 100% respectively. The latency of N1 was (11.33±1.35) ms and (10.14±0.38) ms, latency of P1 was (16.24±1.56) ms and (15.65±1.19) ms, interval N1-P1 was (4.59±1.26) ms and (5.55±0.81) ms, symmetry coefficient was 12.22%[5.50%,21.85%] and 8.74%[3.37%,14.08%], amplitude was 3.07[2.05,4.43] µV and 3.07[2.05,4.43] µV in sound and bone conducted vibration group, respectively. The latency of N1, latency of P1, interval N1-P1, symmetry coefficient and n1-P1 amplitude of BC-oVEMP were different statistically (all P<0.05). Conclusions: 500 Hz 45 dB input stimulation setting is the optimal parameter for BC-oVEMP. BC-oVEMP can be recorded simply, safely and quickly in normal young people, thus it is a clinically practical way of measuring utricular function.

[Actively promoting the research and development of artificial intelligence diagnosis and treatment of orbital disease].

Medical artificial intelligence (AI) promotes technological revolution and industrial transformation in the medical field, and the medical level of orbital disease will also be improved with the in-depth development of AI diagnosis and treatment. The problems should be solved in the orbital disease AI research at the initial stage include: the complex knowledge system of orbital disease requires cross-disciplinary knowledge to improve the level of diagnosis and treatment; the early diagnosis and treatment of orbital disease is absent, and effective technical methods are needed to crack the screening dilemma; the operation of the disease is difficult, and special mechanical devices are needed to break through the boundaries of surgical technique; the medical team of orbital disease is scarce, and it is necessary to share the medical resources effectively. In order to solve the problems mentioned above, the following research directions of orbital disease AI diagnosis and treatment should be included: the orbital disease AI diagnosis system, the orbital disease AI screening platform, the orbital surgery robot and the orbital disease telemedicine. Through the development of multidisciplinary diagnosis and treatment, the in-depth exploration of interdisciplinary basic research, the extensive implementation of clinical research, and the extensive integration of industry and academic communication, the AI diagnosis and treatment of orbital disease is approaching a new era. (Chin J Ophthalmol, 2019, 55: 801-805).

[An update on the treatment of orbital venous malformation].

Orbital venous malformation (OVM) ranks as the most common form of vascular lesions in the orbit. OVM may cause functional impairment as well as disfigured appearance in orbital and periorbital regions. A variety of treatment methods has been used to deal with OVM, including sclerotherapy, laser therapy, embolization, surgical resection. The treatment scenario should be made based on hemodynamics, location and extent of lesions in order to achieve encouraging results. With increased understanding on hemodynamics of OVM and application of newly proposed techniques, a combined modality of multiple approaches with defined order has been advocated to be applied in the treatment of OVM. In this paper, the latest advancement in treatment of OVM is reviewed. (Chin J Ophthalmol, 2018, 54: 548-550).

[Pay attention to the application of the international intraocular retinoblastoma classification and sequential multiple modality treatment].

Retinoblastoma (RB) is the most common intraocular malignancy in childhood. It may seriously affect vision, and even threaten the life. The early diagnosis rate of RB in China remains low, and the majority of patients are at late phase with high rates of enucleation and mortality. The International Intraocular Retinoblastoma Classification and TNM staging system are guidances for therapeutic choices and bases for prognosis evaluation. Based on the sequential multi-method treatment modality, chemotherapy combined with local therapy is the mainstream in dealing with RB, which may maximize the results of eye saving and even vision retaining. New therapeutic techniques including supra-selective ophthalmic artery interventional chemotherapy and intravitreal chemotherapy can further improve the efficacy of treatment, especially the eye salvage rate. The overall level of RB treatment should be improved by promoting the international staging, new therapeutic techniques, and the sequential multiple modality treatment. (Chin J Ophthalmol, 2017, 53: 561-565).

[The progress in the diagnosis and management of orbital lymphoma].

Lymphoma is one of the most common malignant tumor of the orbit in adults. Orbital lymphoma mainly consists of extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue, follicular lymphoma, diffuse large B-cell lymphoma, mantle cell lymphoma, infrequent NK/T cell lymphoma and Burkitt lymphoma. Diagnosis on orbital lymphoma remains challenging in that it is difficult to distinguish it from benign lesions, especially for low degree lymphomas. Combined examinations of histological, cytological and molecular features are required to make final differentiation. There are many treatment options for diagnosis of orbital lymphoma, such as surgical resection, radiotherapy, chemotherapy, monoclonal antibody targeted therapy, immunotherapy. The appropriate scenario of treatment on orbital lymphoma should be made based on the Ann Arbor or TNM staging system to determine the stage of orbital lymphoma at the moment for maximizing the results. The latest concepts on diagnosis and management of orbital lymphoma are reviewed in this paper. (Chin J Ophthalmol, 2017, 53: 632-636).

VEGF-targeted RNA interference suppresses angiogenesis and tumor growth of retinoblastoma.

Vascular endothelial growth factor (VEGF) is one of the most important angiogenic growth factors for tumor angiogenesis which has been verified to be involved in neovascularization of retinoblastoma. Here, we sought to explore whether RNA interference (RNAi) targeting VEGF could inhibit retinoblastoma angiogenesis and tumor growth. Stable transfection of the two human retinoblastoma cell lines SO-RB50 and HXO-RB44 with VEGF-targeted small interfering RNA (siRNA) expression plasmid significantly inhibited VEGF expression determined by real-time polymerase chain reaction (PCR), enzyme-linked immunosorbent assay and Western blot, whereas the control transfection showed no effects. The chemically synthesized VEGF siRNA dramatically suppressed tumor angiogenesis (CD34 immunohistochemistry) and tumor growth in the SO-RB50 subcutaneous xenograft model. Significant downregulation of VEGF expression both on messenger RNA and protein levels in VEGF-siRNA-treated SO-RB50 subcutaneous xenograft was confirmed by real-time PCR and Western blot compared to control. Our data demonstrate the suppression function on angiogenesis and tumor growth of retinoblastoma by VEGF-targeted RNAi. This novel therapeutic strategy promises to play a part in the clinical management of retinoblastoma.

Apoptosis in oncology.

Apoptosis is a complex process involving a large array of genes and mutation of any of these genes may lead to malignancy formation. Re-acquirement of FasL by tumor cells may enable them to evade the surveillance of immune system and thus contributes to the growth of tumor. Apart from traditional therapies, inducing apoptosis of tumor cell by new methods employing death receptor ligands and making use of Fas counterattack is also being developed.