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 investigate the risk factors of wet ear status and its impact on the efficacy of endoscopic type Ⅰ tympanoplasty. Methods:A retrospective analysis was conducted at the Department of Otolaryngology Head and Neck Surgery, the First Affiliated Hospital of Chinese People's Liberation Army（PLA）Air Force Medical University, on 160 ears that underwent endoscopic type Ⅰ tympanoplasty; these were assigned to a dry-ear group （n= 118） and a wet-ear group （n= 42）.Univariate analysis and binary logistic regression were used to identify risk factors for wet ear status. Postoperative outcomes, including tympanic meoombrane healing rate and hearing improvement across frequencies, were compared between groups. Results:①Significant intergroup differences were observed in age, residual tympanic membrane status, external auditory canal condition, mastoid pneumatization（MC0）, and middle ear ventilation dysfunction（P<0.05）; ②The degree of mastoid pneumatization being MC0 is an independent risk factor for wet ear（P<0.05）; ③No significant difference in tympanic membrane healing rates was found（P>0.05）; ④The wet ear group showed significantly higher pre-and postoperative air-conduction（AC） and bone-conduction（BC） thresholds at 2 kHz and 4 kHz compared to the dry ear group（P<0.05）, though the postoperative air-bone gap（ABG） improvement was comparable. Conclusion:Poor mastoid pneumatization is a risk factor for wet ears. The wet ear state has no effect on tympanic membrane healing and air-bone conduction gap, but patients in the wet ear group may have more severe inner ear or auditory nerve pathway damage.
Humans ; Retrospective Studies ; Tympanoplasty/methods* ; Adult ; Risk Factors ; Male ; Female ; Young Adult ; Endoscopy ; Adolescent ; Middle Aged ; Treatment Outcome ; Child ; Logistic Models ; Tympanic Membrane/surgery*

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.

Objective To evaluate the reliability and validity of the Chinese version of the hyperacusis scale and estimate its applicability in the Chinese population.Methods The patients admitted to the Department of Oto-laryngology of Xijing Hospital from June 2017 to December 2017 were surveyed.A total of 300 questionnaires were sent out and 293 valid questionnaires were collected with effective response rate of 97.67％.All participants comple-ted a basic information survey,pure tone audiometry and fill in the Chinese version of the hyperacusis scale.The re-liability and validity of this scale were evaluated.Results Among the 293 respondents,243(82.93％)were compli-cated with tinnitus.A total of 181 cases(61.77％)were subjectively diagnosed with hyperacusis through question-naire survey.According to the scale evaluation,174 cases(59.39％)were diagnosed with hyperacusis,and the Kappa value of conformity test of the two methods was 0.81(P＜0.001).Reliability analysis indicated a Cronbach'sα coefficient of 0.93 for the entire scale.Each dimension had a Cronbach's α coefficient＞0.60.The split-half relia-bility coefficient for the entire scale was 0.86,and for each dimension,it was＞0.60.Exploratory factor analysis of validity suggested that 25 items could be grouped under 4 factors,such as functional part,social part,emotional part and harmonic sensitivity part.These factors had eigenvalues greater than 1,accounting for 59.42％of the total variance.Post-rotation,the 25 items were distributed across the 4 components,with each item having a factor load-ing＞0.40.Confirmatory factor analysis of validity showed model fit parameters as:x2/df=1.98,GFI=0.91,AGFI=0.83,CFI=0.92,TLI=0.89,RMSEA=0.08.Each item had a standardized factor loading＞0.40.Con-clusion The Chinese version of the hyperacusis scale demonstrated strong reliability and validity,making it suitable for use among the Chinese population.

Objective To study the clinical characteristics and related factors affecting the severity of acute tinnitus in patients.Methods A retrospective analysis was conducted on the data of 319 patients with acute tinni-tus.All patients had detailed case history,including basic patient information,tinnitus location,course of disease,acoustic characteristics of tinnitus,hearing loss,combined headache,dizziness,aural fallness,earache and other symptoms,as well as accompanying clinical diseases.All patients completed the tinnitus handicap inventory(THI),generalized anxiety disorder(GAD-7),patient health questionnaire(PHQ-9),pittsburgh sleep quality index(PSQI),hyperacusis questionnaire(HQ),type D personality scale-14(DS-14),etc.The mild tinnitus group was determined based on a THI score of ≤36.The moderate tinnitus group was determined based on a THI score of 38～56.The severe to extremely severe tinnitus group was determinded based on THI 56～100.THI grouping was used as the dependent variable and the above factors as independent variables.The correlation between each factor and the severity of acute tinnitus was analyzed using ordered multinomial logistic regression.Results Among 319 patients with acute tinnitus,158(49.5％)were in the mild tinnitus group,and 72(22.6％)were in the moderate tinnitus group,and 89(27.9％)were in severe to extremely severe tinnitus group.Ordered multinomial logistic re-gression analysis found that hyperacusis(OR=3.921),anxiety(OR=2.495),depression(OR=2.921),and D-type personality(OR=0.349)were associated with the severity of acute tinnitus(P＜0.05),with more serere tin-nitus in these patients.Conclusion Hyperacusis,anxiety,depression,and D-type personality may be factors that affect the severity of acute tinnitus,so high attention should be paid when treating patients with acute tinnitus.

Objective:Retrospective analysis of clinical data of 123 patients with atticotomy, exploring the clinical characteristics of patients undergoing atticotomy and the efficacy of hearing reconstruction methods. Methods:123 patients with atticotomy were divided into three groups according to the ossicular chain treatment method: preservation of the ossicular chain group（37 cases）, cartilage elevation of stapes group（49 cases）, and PORP group（37 cases）. The clinical characteristics of patients with atticotomy, preoperative and postoperative hearing levels of the three groups of patients, and postoperative complications were analyzed. Results:①89.43%（110/123） of patients who underwent atticotomy were pars flaccida cholesteatomas, while 10.57%（13/123） of patients were secondary cholesteatoma, adhesive otitis media, pars tensa cholesteatomas, congenital cholesteatoma, and external auditory canal cholesteatoma; ②The group with preserved ossicular chain had a shorter medical history compared to the other two groups, and the difference was statistically significant（P<0.001）. The group with preserved ossicular chain had smaller inter group ABG values and average ABG values at frequencies of 500 Hz, 1 000 Hz, 2 000 Hz and 4 000 Hz before surgery compared to the other two groups, and the difference was statistically significant（P<0.001）; The differences in ABG frequencies and average ABG between the three groups of patients before and after surgery were statistically significant（P<0.05）; Postoperative ABG: The group preserving the ossicular chain had a smaller difference compared to the other two groups, with a statistically significant difference（P<0.05）. There was no statistically significant difference between the cartilage plus high stapes group and the PORP group（P>0.05）; ③At 3 months post surgery, all patients achieved ear dryness. Two patients experienced delayed facial paralysis after conservative treatment, and all recovered. One patient had a slight decrease in bone conductivity due to the influence of grinding during hammer anvil fixation surgery, and one patient experienced a recurrence after 4 years due to residual surgery. 81 patients（65.85%） experienced non cartilage repair area invagination during postoperative follow-up, of which 5 patients（4.07%, 5/123） underwent a second surgery. Although the rest of the patients had local invagination, they could still self clean and did not form a cholesteatoma. The total recurrence rate was 4.88%（6/123）, with an average recurrence time of 4 years. Conclusion:Atticotomy surgery is most commonly used for pars flaccida cholesteatomas with limited scope. The incidence of postoperative retraction is high, and regular follow-up is necessary; When conditions permit during surgery, priority can be given to preserving the ossicular chain for better postoperative hearing. Both cartilage elevation of stapes and PORP implantation can effectively improve hearing, and there is no difference in postoperative hearing between the two methods. However, there is a risk of detachment and high cost after PORP surgery, and cartilage elevation of stapes is limited by insufficient height and stapes head erosion. Therefore, it is necessary to choose a comprehensive hearing reconstruction method based on the patient's condition.
Humans ; Retrospective Studies ; Female ; Male ; Adult ; Middle Aged ; Plastic Surgery Procedures/methods* ; Hearing ; Middle Ear Ventilation/methods* ; Treatment Outcome ; Adolescent ; Child ; Ossicular Replacement/methods* ; Ear Ossicles/surgery* ; Young Adult ; Tympanoplasty/methods*

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.

A retrospective analysis of audiologic outcome and graft take rate on post-tympanoplasty with different middle ear mucosal conditions in wet ear. According to the characteristics of middle ear mucosal condition and residual eardrum, 80 cases with wet ear of chronic suppurative otitis media were divided into the hydrocele group, the swelling group and the granulation group. The factors in different groups, including gender, age, disease course, sides, size and location of perforations, destruction of ossicular chain and reconstruction methods were analyzed. Moreover, postoperative hearing improvement and graft take rate were compared among the three groups. There was no significant difference in gender, age, disease course, sides, size and location of perforations among the hydrocele group, the swelling group and the granulation group （>0.05）. Overall, the postoperative average Air-Bone Gaps（ABG） were reduced in all wet ear patients after surgery （<0.01）. The ABG was decreased from （25.5 ± 10.8） dB to（15.4 ± 9.4） dB in the hydrocele group, and decreased from （27.6 ± 8.7） dB to （15.2 ± 9.6） dB in the swelling group, and from （29.5 ± 7.7） dB to （17.2 ± 17.2） dB in the granulation group. The graft take rates were 90.0% in totally. There were no significant difference in graft take rates among the three groups, and 84.6% in the hydrocele group, 93.3% in the swelling group and 100.0% in the swelling group（>0.05）. Wet ear is not an absolute contraindication of tympanoplasty for chronic suppurative otitis media. Whether there was effusion, swelling or granulomatous hyperplasia in the tympanoplasty, the patients'hearing improved significantly after tympanoplasty, and the healing rate of the tympanoplasty did not decrease. Further basic and clinical studies are needed to standardize the timing of wet ear surgery, clarify the operative contraindication and elucidate the pathophysiological mechanism of eardrum healing.

Objective: A retrospective analysis of audiologic outcome and graft take rate on post-tympanoplasty with different middle ear mucosal conditions in wet ear. Method: According to the characteristics of middle ear mucosal condition and residual eardrum, 80 cases with wet ear of chronic suppurative otitis media were divided into the hydrocele group, the swelling group and the granulation group. The factors in different groups, including gender, age, disease course, sides, size and location of perforations, destruction of ossicular chain and reconstruction methods were analyzed. Moreover, postoperative hearing improvement and graft take rate were compared among the three groups. Result: There was no significant difference in gender, age, disease course, sides, size and location of perforations among the hydrocele group, the swelling group and the granulation group （P>0.05）. Overall, the postoperative average Air-Bone Gaps（ABG） were reduced in all wet ear patients after surgery （P<0.01）. The ABG was decreased from （25.5 ± 10.8） dB to（15.4 ± 9.4） dB in the hydrocele group, and decreased from （27.6 ± 8.7） dB to （15.2 ± 9.6） dB in the swelling group, and from （29.5 ± 7.7） dB to （17.2 ± 17.2） dB in the granulation group. The graft take rates were 90.0% in totally. There were no significant difference in graft take rates among the three groups, and 84.6% in the hydrocele group, 93.3% in the swelling group and 100.0% in the swelling group（P>0.05）. Conclusion Wet ear is not an absolute contraindication of tympanoplasty for chronic suppurative otitis media. Whether there was effusion, swelling or granulomatous hyperplasia in the tympanoplasty, the patients'hearing improved significantly after tympanoplasty, and the healing rate of the tympanoplasty did not decrease. Further basic and clinical studies are needed to standardize the timing of wet ear surgery, clarify the operative contraindication and elucidate the pathophysiological mechanism of eardrum healing.