[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.

[Analysis of clinical features and surgical outcomes of petrous bone cholesteatomas].

Objective: To analyze the clinical features and surgical outcomes of petrous bone cholesteatomas (PBCs). Methods: Data from 39 PBCs patients treated in the Department of Otorhinolaryngology, Xijing Hospital from September 2011 to December 2017 were reviewed retrospectively, including 23 males, 16 femals, aged 12-71 years old, with the median age of 37. Clinical classifications, surgical methods, facial and hearing function, and intraoperative and postoperative complications were made summary analysis. Results: In this study, five patients were congenital PBCs and 34 patients were acquired PBCs. The common clinical symptoms were hearing loss (100%, 39/39), ear discharge/pus (89.7%, 35/39) and facial paralysis (46.2%, 18/39). According to Sanna's classification, 14 cases were supralabyrinthine, including three cases underwent transcochlear (TC) approach, six cases underwent transotic (TO) approach and five underwent translabyrinthine (TL) approach. 10 cases were infralabyrinthine, including eight cases underwent subtotal petrosectomy, one case underwent TO approach and one underwent TL approach.10 cases were massive, including seven cases underwent TC approach, three cases underwent TO approach. Five cases were infralabyrinthine-apical, including two cases underwent TC approach, two cases underwent TO approach, and one case underwent endoscope assisted infratemporal fossa type B. The degree of facial nerve (FN) dysfunction from high to low was massive (6/10), supralabyrinthine (8/14), infralabyrinthine-apical (2/5) and infralabyrinthine (2/10). 19 cases involved in facial nerve operation, three cases underwent FN decompression, four cases underwent FN rerouting, four cases underwent nerve grafting, and one case underwent facial-hypoglossal anastomosis. Preoperative FN involvement in 18 cases, and the FN function was improved in 14 cases after surgery. The improved rate of postoperative FN function was 77.8%. The bone conducted hearing retained 50.0% (14/28) postoperatively. Five cases with cerebrospinal fluid leak were managed by inserting free muscle plugs and cavity obliteration. Two cases with the cholesteatomas matrix involved the sigmoid sinus and the jugular bulb, and occlusion of the sigmoid sinus was performed. Postoperatively, two patients presented with synkinesis. The patients were followed up for 40 to 115 months, and there was no recurrence. Conclusions: There are no specific clinical manifestations for PBCs, thus, it is difficult in early diagnosis and treatment. According to Sanna's classification, preoperative FN and hearing function, the best surgical approach should be selected with minimal recurrences and perioperative morbidity.

MicroRNA-29 targets FGF2 and inhibits the proliferation, migration and invasion of nasopharyngeal carcinoma cells via PI3K/AKT signaling pathway.

The article "MicroRNA-29 targets FGF2 and inhibits the proliferation, migration and invasion of nasopharyngeal carcinoma cells via PI3K/AKT signaling pathway, by M. Xu, G.-L. Tian, C.-C. Hao, M. Shi, D.-J. Zha, K. Liang, published in Eur Rev Med Pharmacol Sci 2019; 23 (12): 5215-5222. DOI: 10.26355/eurrev_201906_18186. PMID: 31298371" has been withdrawn from the authors. The Publisher apologizes for any inconvenience this may cause. https://www.europeanreview.org/article/18186.

[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.

[Crouzon syndrome with secretory otitis media: a case report and literature review].

Summary Clinical data from a case of Crouzon syndrome with secretory otitis media in our department was collected and the related literatures were reviewed. Whole exome sequecing and Sanger sequencing were performed to analyze genetic cause. The 6-year old patient with Crouzon syndrome had snoring and mouth breathing during sleep for 2 years, and was found hearing loss for 2 weeks. The results of endoscopy showed adenoid hypertrophy and secretory otitis media of both ears. And CT scan proved chronic rhinosinusitis. Myringotomy and adenoidectomy were done under general anesthesia. The follow-up at 6 months showed normal sleep and hearing level. A heterozygous fibroblast growth factor receptor 2 missense mutation（c.1061C>G, p.S354C） in exon 8 was identified in this patient.

[Analysis of predictors for Frey syndrome after parotidectomy].

Objective:To analysis the data of the patients with parotid tumors, clarify the contributing factors of Frey syndrome, and to evaluate the role of soft tissues membrane SIS in prevention of Frey syndrome after parotidectomy. Method:The data of 95 patients who suffered from parotid tumors and underwent parotidectomy were included in this study. The relationship between the patients' age, sex, tumor location ,tumor size, disease pathology, type of resection, SIS application and the incidence of Frey syndrome were statistically analyzed . Result:The incidence of Frey syndrome after parotidectomy for 95 patients was 25.3%. Age, sex, tumor location and size, disease pathology, type of resection did not appear to be associated with development of Frey syndrome（P>0.05）. SIS application was the only statistically significant factor（P<0.01）, and SIS could prevent Frey syndrome after parotidectomy. Conclusion:Frey syndrome is one of the common complicationsafter parotidectomy. Implantation of SIS is an effective method for prevention of Frey syndrome after parotidectomy.

[Screening and antibiotic resistance analysis of nasal colonized bacteria in patients with chronic rhinosinusitis].

Objective:Since bacterial infection is one of the common pathogenic factors lead to chronic rhinosinusitis （CRS）, our study is designed to investigate the prevalence and antibiotic resistance of nasal colonized bacteria in patients with CRS, and to analyze the correlation between nasal colonized bacteria and pathogenesis of CRS. Method:Nasal vestibule samples collected from 200 patients with CRS were automatically inoculated on to various bacterial culture medium, cultured by WaspLab microbiology automated system, potential pathogenic bacterial colonies grew on agar plates were then examined, identified by matrix-assisted laser desorption ionization-time of flight mass spectrometry, and analyzed their antibiotic-resistant phenotype by antibiotic susceptibility testing. Result:Two hundred and seventy-five bacterial strains of nasal colonization were isolated, including coagulase-negative Staphylococci, Staphylococcus aureus, Streptococcus pneumonia, Moraxella catarrhalis, Haemophilus influenza, Pseudomonas aeruginosa, and Enterobacteriaceae such as Escherichia coli and Klebsiella pneumoniae. All strains displayed various degrees of antimicrobial resistance among children and adult patients, or among patients with and without endoscopic sinus surgery. Conclusion:The constitution of nasal colonized commensal bacteria in patients with CRS was partially similar to that of pathogenic bacteria causing CRS. They may form a reservoir of antimicrobial resistant pathogens, which would transform into pathogenic bacteria causing CRS under certain conditions. Screening of nasal colonized bacteria may represent an alternative approach for diagnosis and treatment of CRS.

MicroRNA-29 targets FGF2 and inhibits the proliferation, migration and invasion of nasopharyngeal carcinoma cells via PI3K/AKT signaling pathway.

OBJECTIVE: Studies have indicated that miRNAs may prove essential therapeutic targets for the treatment of cancer. The study was designed to investigate the role and therapeutic potential of miR-29 in nasopharyngeal cancer. MATERIALS AND METHODS: The quantitative Real-time polymerase chain reaction (qRT-PCR) was used for expression analysis. WST-1 assay was used for cell viability assessment. The 4',6-diamidino-2-phenylindole (DAPI) staining and electron microscopic analysis was used for the detection of apoptosis and autophagy, respectively. Transwell assays were used for cell migration and invasion assay. RESULTS: It was found that miR-29 is significantly downregulated in nasopharyngeal cancer cell lines. Overexpression of miR-29 causes decrease in the viability of CNE2 nasopharyngeal cancer cells via induction of apoptosis and autophagy. Bioinformatics analysis indicated FGF2 to be the target of miR-29 in CNE2 cells, which was also confirmed by luciferase reporter assay. The qRT-PCR results showed fibroblast growth factor 2 (FGF2) to be significantly upregulated in the nasopharyngeal cancer cell lines. However, miR-29 overexpression in CNE2 cells resulted in post-transcriptional suppression of FGF2 expression. Nonetheless, silencing of FGF2 also caused inhibition of CNE2 cell proliferation via induction of apoptosis and autophagy. Overexpression of FGF2 could reverse the effects of miR-29 overexpression on the proliferation of CNE2 cells. Moreover, overexpression of miR-29 causes significant decline in the phosphorylation of PI3K and AKT expression cells and inhibits their migration and invasion of the CNE2 cells. Finally, miR-29 overexpression could also suppress the subcutaneous xenografted tumor growth. CONCLUSIONS: The findings of the present study indicate the therapeutic implications of miR-29 in nasopharyngeal carcinoma.

[Comparison of the treatment effect for stage â epitympanic cholesteatoma with atticotomy and Bondy modified radical mastoidectomy].

Objective:To compare the efficacy and safety between Bondy modified radical mastoidectomy (BMRM) and atticotomy for stage Ⅰ epitympanic cholesteatoma. Method:A retrospective analysis of 36 cases with stage Ⅰ epitympanic cholesteatoma was performed. According to different surgery methods, the cases were divided into the atticotomy group (21 cases) and the BMRM group (15 cases). Hearing function, the dry ear ratio after two months of the surgery, and the epithelial time were observed. The complications for the retraction of tympanic membrane, residual or recurrence of cholesteatoma, and otorrhea were analyzed. Result:There was no significant difference between the two groups in average air-bone gaps (ABG) and ABG gain before and after surgery (P>0.05). The preoperative hearing was preserved both in the atticotomy group and the BMRM group. However, more surgery time, lower ratio of the dry ear after two months of the surgery, and longer epithelial time were observed in the BMRM group than those in the atticotomy group(P<0.05). No cholesteatoma residual or recurrence was observed in both groups. Conclusion:For stage Ⅰ epitympanic cholesteatoma lateral to an intact ossicular chain, atticotomy has the advantage of complete removal of the cholesteatoma matrix, avoiding big mastoidal cavity, keeping normal anatomical structure of external auditory canal, better hearing preservation, and faster healing. Therefore, atticotomy has better clinical and practical value.

[Surgical management of petrous bone cholesteatoma and facial nerve protection].

Objective: To discuss the therapeutic scheme of petrous bone cholesteatoma(PBC) and the technique of facial nerve reconstruction. Method: The data of 28 patients who underwent surgery for PBC in our center were analyzed retrospectively. All patients were diagnosed radiologically with PBCs and reconfirmed pathologically after surgery. The surgical approach was discussed basing Sanna's classification of PBCs, and the facial nerve outcomes were analyzed moreover. Result: PBC cases 11 were supralabyrinthine, 4 infralabyrinthine, 3 infralabyrinthine-apical, 10 massive and none apical. The facial nerve was involved in 50% of the cases. The translabyrinthine approach were used in 3 cases. The transotic approach was used in 7 cases in this series.The transcochlear approach type was applied in 12 cases. The Infratemporal fossa type B approach and subtotal petrosectomy were employed in 2 cases and 4 cases respectively. Active management of the nerve(rerouting, anastomosis, or grafting) was required in 14 cases, postoperative facial nerve function were inproved in 10 cases(71.43%). Conclusion: The appropriate surgery approach was vitally important to radical disease clearance in PBCs. The facial nerve preservation was preceded hearing preservation. Active facial nerve management were beneficial to facial nerve recovery..

[The study for the characteristics of secondary acquired cholesteatoma].

Objective:Retrospctive analysed the Characteristics and outcomes of surgical treatment of 18 patients who were diagnosed as secondary acquired cholesteatoma (SAC).Method:Patients with SAC accepted operations were enrolled in this study. Then the factors such as sex, age, cource of history, otorrhea before operation, the size of perforation of tympanic membrane, entry site of epithelium, extension direction, ossicular destruction, tympanosclerosis, tympanum tympani tendon involvement, stage of cholesteatoma（JOS, 2015, Japan），degree of gasification of mastoid（JOS，2015，Japan），air conductive threshold, bone conductive threshold, air-bone gap, the healing of tympanic membrane and the auditory improvement were analyzed.Result:Eighteen patients were enrolled in this study, with course of history range from 2 months to 50 years (average: 20.20±16.31) years. There were 14 cases with wet ear before operation. All patients were conformed with perforation of membranal tensa by otoendoscopic photography before operation, with nearly total in 5 ears,large size in 10 ears,medium size in 2 ears and small size in 1 ear. Ossicular erosion were found in 13 patients (malleus involved in 6, incus involved in 12 and stapes involved in 4). Thirteen patients with tendon of tensor tympani involved and 9 patients with tympanosclerosis were conformed during operation. The epithelium entered through the malleus manubrium to the promontory in 13 cases, through the edge of the perforation in 3 cases and through the incus long process to around stapes in 1 case. Cholesteatoma invasion extend to anterosuperior area in 3 cases, posterosuperior area in 3 cases,both in anterosuperior and posterosuperior area in 12 cases. The cholesteatomas classified: stage Ⅰ in 11 cases, stage Ⅱ in 7 cases. Mastoid gasification classified MC0 in 6 cases, MC1 in 10 cases and MC2 in 2 cases. The average air conductive threshold was (56.32±10.15) dB, bone conductive threshold was (20.76±6.22) dB and air-bone gap (35.56±9.84) dB.Tympanic membrane healed in all patients during following up, without recurrent of cholesteatoma, and the post-operative air conductive threshold (43.02±14.96) dB and air-bone gap (21.04±12.90)dB were improved significantly(P<0.05).Conclusion:Most of SAC were secondary to nearly total or large perforation of membranal tensa (83.33%), with relative long history of chronic otitis media (average 20.20 ± 16.31) years and otorrhea before operation. The epithelium entered mainly through the malleus manubrium to the promontory，then through edge of the perforation，by extending anterosuperior and posterosuperior area and usually accompanied with tendon of tensor tympani involved, ossicular destruction, and poor mastoid gasification and tympanosclerosis. The characteristics of SAC were different from other type of cholesteatoma which need further researches.

[Goldenhar syndrome].

Summary Goldenhar syndrome is a condition characterized by alterations involving the development of the structures of the first and second branchial arches. The abnormalities primarily affect the face, the eyes, the spine, and the ears, and the auricular abnormalities are associated with possible hearing loss. Here, we present a review of the literature on Goldenhar syndrome, discussing what is known about the clinical phenotype , the aetiology, audiological findings and treatment,in order to provide certain help for people about disease diagnosis, genetic counseling and hearing intervention.

[Etiology analysis and vestibular assessment of bilateral vestibular vestibulopathy].

Objective:To define clinical and laboratory characteristics of bilateral vestibulopathy(BVP) and to propose diagnostic criteria of this disorder based on clinical and laboratory vestibular function test findings.Method:Forty-two case series with a clinical suspicion of BVP were retrospectively analyzed, in an attempt to determine etiology. Presenting auditory-vestibular symptoms, bedside dynamic visual acuity tests and laboratory test were reviewed, including bithermal caloric test, rotatory chair tests, video head impulse test (vHIT), vestibular-evoked myogenic potentials (VEMP).Result:Among these 42 patients, dizziness was seen in 42 cases(100%), oscillopsia was seen in 21 cases(50%), hearing loss was seen in 30(71.4%). Eight cases(19%) had tinnitus. Twenty-five cases showed vestibular loss in dynamic visual acuity test (69.4%). Definite diagnosis of complete BVP was made in 36 patients when the patients showed abnormal findings on caloric test, rotatory chair test and vHIT in addition to the symptoms. Whereas probable diagnosis of partial BVP was obtained in 6 patients with abnormal caloric test and rotatory chair test but no pathological vHIT. VEMP (ocular or cervical) could be recorded in 20 patients. Fourteen cases were caused by ototoxic drugs while no causes could be determined in 6 cases among these 42 cases.Conclusion:The diagnosis of BVP is a challenge. Vestibular laboratory test battery which reflect full frequency function of VOR has great value to confirming the diagnosis and differentiate complete BVP to partial BVP. Diagnosis standard shall be made combining clinical history, characteristic symptoms and the results of auditory-vestibular function testing. Ototoxic drugs contribute most considering etiology.

[A retrospective study of 54 cases with chronic otitis media prepared for staging tympanoplasty].

Objective:To analyze the clinical effect of 54 cases with chronic otitis media prepared for staging tympanoplasty and 19 cases finished staging ossicular reconstruction surgery, evaluate the advantages of different surgery technique.Method:Fifty-four cases with chronic otitis media were planned for staging tympanoplasty surgery and had been received the first stage surgery. Silicagel plates were placed in the tympanic cavity in order to prevent adhesions. Among them, 19 cases had received the second stage ossicular reconstruction, including 10 cases with TORPs and 9 cases with PORPs. The average air bone gaps (ABG) were measured at four frequencies: 500, 1 000, 2 000 and 4 000 Hz.Result:Among the 54 cases, 45 cases had swollen mucosa in the tympanic cavity and eustachian tube, 18 cases had tympanic fibrous adhesions, and 12 cases had fixed or sclerotic stapes. The preoperative ABG of the 54 cases were (38.26±7.88)dB. As for the 19 cases finished the second stage surgery, their preoperative ABG in the first stage were (39.21±7.05)dB, the preoperative ABG in the second stage were (38.82±11.43)dB, and the postoperative ABG after the second stage were (21.77±11.92)dB. The hearing function after staging tympanoplasty was significantly improved compared with the preoperative hearing in the first and the second stage operation (P< 0.01). In addition, the second stage surgery of three cases was postponed because of a good postoperative hearing with (10.42±10.63)dB ABG after the first stage surgery. Up to now, none of the 54 cases suffered from facial palsy, infection and other complications after surgery.Conclusion:Staging tympanoplasty is an important technique to improving hearing and reducing the complications, especially for the cases with chronic otitis media unsuitable for one stage reconstruction of ossicular chain.

[Chronic subjective dizziness].

Chronic subjective dizziness(CSD) was defined as persistent nonvertiginous dizziness or subjective unsteadiness. Patients are hypersensitivity to motion stimuli, such as head movement and motion of objects in the visual surrounding, and have difficulty with precise visual tasks. The concept of CSD was first proposed by Staab and Ruckenstein, which indicates relationships between chronic dizziness and acute vestibular or psychiatric disorders, including neuro-otologic type, psychiatric type or interactive type. Treatment includes vestibular rehabilitation therapy, pharmacologic interventions and psycho-behavioral interventions(cognitive behavioral therapy).

[Horizontal semicircular canalvideo head impulse test in normaladults:normal value and age distribution].

Objective:To study the parameters of video head impulse test (vHIT) for horizontal semicircular canal and explore its application when evaluating angular vestibular-ocular reflex (VOR) in adults.Method:vHIT were applied to 80 adults without prior vertigo or dizziness history.Parameters provided by the software included instantaneous gain(40 ms,60 ms,80 ms),regression gain,asymmetry value,corrective saccades(latency,peak velocity,occurrence rate).Result:The mean horizontal VOR velocity gain of normal subjects was 0.877±0.171,0.944±0.133,0.967±0.130 at 40 ms,60 ms,80 ms respectively.Regression gain of horizontal VOR is 0.944±0.090.The lower limit of normal horizontal VOR velocity gain was 0.73 at 60 ms and 0.80 as regression gain.All velocity gains declined by age,although there were no statistically significant difference(P>0.05).Asymmetry value was 5.60±3.46.Corrective saccades occurred in 28±15 of normal subjects,while latency and peak velocity was (200.87±0.34)ms and (97.7±40.1)°/s.Conclusion:The study found that horizontal VOR instantaneous gain value and regression gain value decreases slightly with age,which should be considered in clinical application.

Malignant triton tumour of right paranasal sinuses: case report.

OBJECTIVE: We report an extremely rare case of malignant triton tumour. METHOD: Case report and review of the world literature concerning malignant triton tumour and heredity. RESULTS: We present the case of a 47-year-old woman who underwent a lateral rhinotomy surgical resection of a malignant triton tumour of the right paranasal sinuses, a rare location for this tumour. Thereafter, she received adjuvant radiotherapy. The prognosis for this group of tumours is poor. Radical surgical excision of the tumour followed by radiation therapy must be the treatment of choice. CONCLUSION: To our knowledge, this is a rare report in the world literature of malignant triton tumour. This case indicates that malignant triton tumour of the paranasal sinuses is a rare disease which otolaryngologists should be aware of, and one which should be included in the differential diagnosis of malignant lesions involving the sinonasal tract.