Hearing loss is the most prevalent disabling disease. Cochlear implantation（CI） serves as the primary intervention for severe to profound hearing loss. This consensus systematically explores the value of genetic diagnosis in the pre-operative assessment and efficacy prognosis for CI. Drawing upon domestic and international research and clinical experience, it proposes an evidence-based medicine three-tiered prognostic classification system（Favorable, Marginal, Poor）. The consensus focuses on common hereditary non-syndromic hearing loss（such as that caused by mutations in genes like GJB2, SLC26A4, OTOF, LOXHD1） and syndromic hereditary hearing loss（such as Jervell & Lange-Nielsen syndrome and Waardenburg syndrome）, which are closely associated with congenital hearing loss, analyzing the impact of their pathological mechanisms on CI outcomes. The consensus provides recommendations based on multiple round of expert discussion and voting. It emphasizes that genetic diagnosis can optimize patient selection, predict prognosis, guide post-operative rehabilitation, offer stratified management strategies for patients with different genotypes, and advance the application of precision medicine in the field of CI.
Humans ; Cochlear Implantation ; Prognosis ; Hearing Loss/surgery* ; Consensus ; Connexin 26 ; Mutation ; Sulfate Transporters ; Connexins/genetics*

Objective: To summarize and discuss the characteristics of endoscopic approach to manage the middle ear cholesteatoma, and to evaluate the operative safety and outcomes based on the data from the multicenter study. Methods: The data of 242 cases diagnosed with the middle ear cholesteatoma and received operation through endoscopic approach between June 2016 and June 2017 in six tertiary hospitals in China were analyzed in this work. There were 130 males and 112 females, with the age ranging from 3 to 72 years old. We evaluated the strategy about how to manage the cholesteatoma, discussed the detailed techniques about how to remove the cholesteatoma and to improve the efficiency under endoscopic visualization. Meanwhile, the recurrence rate and residual rate of cholesteatoma as well as the complications in endoscopic approach were summarized. Results: A total of 158 cases were operated in exclusively endoscopic transcanal approach, 72 cases operated in combined approach, and 12 cases operated majorly under microscope and minorly under endoscope. 219 cases were operated in one stage surgery, 23 cases received second look. In the second look, 3 cases were detected with residual cholesteatomas. Among them, 2 cases were found by MRI-DWI examination after the first-stage operation. With endoscopic examination after operation, 17 cases showed retracted pocket recurrence (7%,17/242). With introduction of endoscope in cholesteatoma, 153 cases were achieved canal wall-up operation (63%, 153/242). The complications in endoscopic approach included chord tympani never injury in 27 cases, skin injury of ear canal in 11 cases, tinnitus in 13 cases, vertigo in 7 cases, external ear canal stenosis in 1 case. Conclusions Using otoendoscope in cholesteatoma surgery would help keeping the normal structures of middle ear as much as possible, benefit to remove the hiding pathologies, help reducing residual cholesteatoma and lowering the rate of canal wall-down operation as well. This study showed good safety of otoendoscopic cholesteatoma surgery, however, strict evaluation of indication and quite good surgical techniques and skills are necessary for avoiding unexpected complication.

Objective: To analyze the safety of endoscopic stapes surgery, and to compare the results with stapes surgery under microscopic approach. Methods: This was a retrospective study. One hundred and thirty seven patients from Eye Ear Nose and Throat Hospital of Fudan University and other seven hospitals were enrolled in this study. Eighty eight patients, in whom 29 were male, and 59 were female, aged from 29 to 66 years old, with an average of 40.1±10.7, underwent endoscopic stapedotomy and 49 patients, in whom 17 were male, and 33 were female, aged from 32 to 64 yeas old, with an arerage of 38.7±9.2, underwent microscopic stapedotomy for otosclerosis. Interventions included endoscopic and microscopic stapes surgeries. Main outcome measures consisted of operating time, preoperative and postoperative hearing, intraoperative findings, and postoperative complications. SPSS 16.0 software was used to analyzed the date (t test and χ² test) . Results: Patients in the group who underwent endoscopic stapes surgery showed a mean operative time of (74.1±26.0) min. Patients in the group treated by microscopic approach had a mean operative time (66.5±15.9) min. Statistical difference was evident (t=1.279, P<0.05) . The average operative time of endoscopic surgery became shorter as the cases increased. The average duration of the last 10 cases was shorter than that of the first 10 cases in both groups. The differences were significant (t value was 3.028, 3.610, both P<0.05). No statistical difference was found in air conduction threshold improvement (t=1.074, P=0.289) , air-bone gap closure (t=-0.135, P=0.893) and bone conduction improvement (t=1.222, P=0.228) between the two groups. No difference regarding the incidence of the postoperative complications (chorda tympanum damage: 6 cases vs 2 cases, χ²=0.08,P>0.05; vertigo:18 cases vs 9 cases,χ²=0.09, P>0.05; facial paralysis: 0 case vs 0 case) between the two groups was found. Conclusion Audiological outcomes achieved by endoscopic surgery are similar to the results obtained through a microscopic approach. Endoscopic stapes surgery is safe.

OBJECTIVE To explor e the characteristics of spectral oscillatory power of resting-state electroencephalography(EEG) in subjective tinnitus patients and to lay the foundation for study of central mechanism of tinnitus.METHODS 31 subjects(15 subjects with subjective tinnitus and 16 age matching healthy subjects) underwent a 128-channel resting-state EEG analyses. After a series of preprocessing, data were segmented into 8 frequency bands, including δ(0.5～3.5 Hz), θ(4～7.5 Hz), α1(8～10 Hz), α2(10～12 Hz), β1(13～18 Hz), β2(18.5～21 Hz), β3(21.5～30 Hz) and γ(30.5～44 Hz). The group differences of spectral power were analyzed by independent t test. Correlation between spectral power of each frequency band and tinnitus subjective symptoms were also analyzed. RESULTS Significant higher spectral power of the α1, β and γ bandwere found in the left and right temporal areas of tinnitus group compared with that of normal group.Tinnitus subjects also had higher spectral powerof the δ and θ band in temporo-parietal areas than that of the normal group. There was no significant difference of spectral power in other frequency bands. Moreover, signif icant positive cor relation were found between tinnitus loudness and spectralpower of right anterior lateral(R=0.66, P =0.007) and right anterior medial(R=0.58, P =0.031) areas. CONCLUSION Tinnitus subjects have higher spectral oscillatory power on right and left temporal lobe and temporo-parietal area. A positive correlation exsit between tinnitus loudness andspectral power of right anterior lateral and medial areas in tinnitus subjects, whichindicate that central reorganizationexsit in tinnitus reorganization andγ band maybe considered as a possible biomarkerforthe tinnitus subjective symptom.

OBJECTIVE To evaluate the short-term efficacy of ear molding in the treatment of congenital auricular deformation. METHODS Twenty-four infants(twenty-eight ears) were treated with ear molding devices(EarWell Infant Ear Correction System). Doctors and parents were surveyed 1 months after treatment. RESULTS All cases were treated successfully without severe complications. 25 ears(89％) and 26(92％) were rated as very satisfied or satisfied by doctors and parents, respectively. CONCLUSION Ear molding is a noninvasive treatment, and effectively corrects congenital auricular deformation.

Objective To explore the Efficacy and safety of intravenous thrombolysis with different doses of rt-PA in the treatment of acute anterior circulation cerebral infarction with atrial fibrillation.Methods Retrospective analysis of 61 cases of patients with anterior circulation of cerebral infarction with atrial fibrillation from October 2009 to October 2014 in the First Affiliated Hospital of Xiamen University, the incidence within 4.5 hours of intravenous thrombolysis,and divided into two groups by rt-PA usage,19 cases in adequate group,received 0.9 mg/kg rt-PA intravenous thrombolytic therapy,42 cases in low dose group, received 0.6 mg/kg rt-PA intravenous thrombolysis.Before and after thrombolysis 1,7 and 30 days,NIHSS score was measured, the indexes of coagulation were observed at before thrombolysis and 1,7 days after thrombolysis,,CT scans were performed at 1, 7, and 14 days after thrombolysis,and Rankin (MRS) scores were compared at 90 days after thrombolysis.Results NIHSS 1,7,30 days scores of 2 groups were significantly decreased after thrombolysis(P<0.05),there was no statistically significant at at each time point after thrombolysis.Plasma prothrombin time increased significantly at 1 day and 7 days after thrombolysis,fibrinogen was significantly lower,compared with the low dose group, the difference was significant (P<0.05).There was no significant difference between the two groups in clinical outcome and mortality.The rate of mucosal bleeding in low dose group was lower than that in adequate group (P<0.05).Conclusion Low-dose rt-PA group intravenous thrombolysis with anterior circulation of atrial fibrillation is more safe,can reduce the risk of bleeding, reduce neurological deficits and improve the quality of life of patients.

Objective To investigate effect of butylphthalide on serum sex hormone,5-HT and sleep quality in patients with stroke sleep disorder. Methods 240 patients with stroke sleep disorder in our hospital were selected,according to clinical medication were divided into 2 groups.Control group (n=120) were treated by routine treatment, experimental group (n=120) was treated on the base of the control group with butylphthalide.Serum sex hormones, 5-HT and PSQI levels were detected after the treatment.Results Compared with the control group,LH, FSH levels of the experiment were higher(P<0.05),E2 level was lower(P<0.05),serum 5-TH level was higher(P<0.05),PSQI score was lower(P<0.05),the difference was statistically significant.Conclusion Butylphthalide can improve stroke sleep disorder serum sex hormone and 5-HT levels, improve sleep quality, has guiding significance for the treatment.

[ABSTRACT]OBJECTIVETo evaluate the short-term efficacy of Eustachian tube balloon dilation (ETBD) in the treatment of symptomatic Eustachian tube dysfunction (SETD) by subjective and objective analysis.METHODS Forty patients who underwent ETBD were included. Subjects’ inclusion criteria were as followed: symptoms of ETD (aural fullness predominantly, with or without otalgia, muffle hearing and tinnitus), normal tympanic membrane, type A or C tympanograms, and without a history of any middle ear diseases. Main outcomes including subjective improvement, otoscopy, pure-tone audiometry, impedance audiometry, R-value in tubomanometry (TMM) and Eustachian Tube Score (ETS) were assessed 1 week and 6 months postoperatively.RESULTSAll cases were dilated successfully. A significant effect of treatment was documented when measuring subjective improvement, impedance audiometry, R-value in TMM and ETS 1 week and 6 month postoperatively. Subjective symptoms were not relieved only in one patient. The overall success rate for all patients was 98%.CONCLUSIONETBD can provide short-term benefits to those who are diagnosed with SETD and refractory to medical management. SETD might be an optimal indication for ETBD in the treatment of ETD.

Objective To summarize the condition of surgical treatment and prognosis of 54 cases of jugular foramen tumors in the past 5 years.Methods All 54 cases were treated with operation by Fisch approach.Thirty-eight cases underwent total or near total resection of the tumor.Most resection of the tumor was underwent in 16 cases,in which,7 cases underwent postoperative radiotherapy or gamma knife therapy.The facial nerve monitoring was used in all cases.The facial nerve was anatomized and shifted in 35 cases,was just anatomized like a bridge in 10 cases,and was excised partly in 9 cases,in which,4 cases underwent facial-hypoglossal nerve anastomosis and 3 cases underwent transplantation of facial nerve and great auricular nerve.Results The external auditory canal was closed in 41 cases.The near pedicled temporalis muscle flap was obliterated in the operating cavity in 35 cases.Six cases underwent fat filling in the operating cavity.Eighteen patients showed facial palsy in varying degrees after operation.Among them,14 cases improved to different extents in 2 weeks to 9 months and 4 cases did not improve.One case showed recurrent laryngeal nerve paralysis in the same side before operation and improved in half a year after operation.Postoperative subcutaneous hematoma occurred in 2 cases,wound infection in 1 case.All the cases improved by debridement suture and anti-infective therapy.The cavity filling necrosis occurred in 2 cases,which recovered after debridement and dressing.For half a year after operation,except 9 cases of residual or recurrent,and the rest showed no recurrence.Conclusion The Fisch approach of surgical treatment of jugular foramen tumors can provid good exposure,clear vision,facilitate hemostasis.The skills of intraoperative facial nerve monitoring,facial nerve anatomy like a bridge or anatomy and shift when necessary are beneficial to the total or nearly total resection of tumor and reduce the injury of facial nerve.The operating cavity filling and selective external auditory canal closure can effectively reduce the surgical cavity effusion and the incidence of postoperative infection.

OBJECTIVE: To investigate the effect of Eustachian tube balloon dilation (ETBD) in treatment of eustachian tube related diseases. METHOD: Fifteen cases (20 ears) of otitis media with effusion and 22 cases (30 ears) of symptomatic Eustachian tube dysfunction were recruited. Technique of tubomanometry (TMM) showed obstructive Eustachian tube dysfunction in all patients. All the patients were received ETBD and followed up with VAS evaluation of ear fullness, muffled hearing, poping sound in the ear and tinnitus. And also the TMM change and middle ear effusion. RESULT: Ear fullness, muffled hearing released with 1 week (ear fullness: 8.2 ± 1.4 vs. 2.0 ± 1.2, P < 0.05, muffled hearing: 6.2 ± 1.2 vs. 3.1 ± 0.8, P < 0.05). No recurrence was seemed within 6 months. The eustachian function test turned better. Symptomatic Eustachian tube dysfunction had an effective rate of 96.6% while otitis media with effusion was 95.0%. CONCLUSION ETBD have good short-term effect in obstructive eustachian tube dysfunction related middle ear dysfunction, which might provide a good way to solve the eustachian tube related diseases.
Catheterization ; Ear Diseases ; Ear, Middle ; Eustachian Tube ; physiopathology ; Humans ; Otitis Media ; Otitis Media with Effusion ; Recurrence