Objective To study the clinical features and treatment outcomes of otitis media caused by antineu-trophil cytoplasmic antibody ANCA-associated vasculitis[otitis media with AAV(OMAAV)].Methods A total of 19 cases of OMAAV diagnosed between September 2015 to September 2022 were studied.OMAAV was defined using the criteria introduced by Japan Society of Otology.Results Median age of all patients was 61.8 years old,predominantly proteinase 3(PR3)positive(47.3％).Averaged score of vasculitis activity was 6.6.Disease also in-volved lung(84.2％),nose(73.7％)and facial nerve(21.0％).Pathological biopsy was performed in nine pa-tients,and two reports indicated ANCA-related disease.Fourteen affected ears of eight patients showed mixed hear-ing loss.The administration of corticosteroid together with an immunosuppressant significantly improved hearing,from averaged air conduction of 75.1±23.2 dB HL to 50.5±22.1 dB HL after treatment(P＜0.05).Conclusion Otologists shall be aware of OMAAV when handling intractable otitis media in elderly patients if bone conduction hearing gets worse and there are other affected organs.Immunological serum test is an important diagnostic basis.Most patients were misdiagnosed for certain time,and hearing level might not be improved unless early diagnose and proper treatment.

Objective To establish normal reference values for the sensory organization test(SOT)for flight crews of different ages from 20 to 50 years old,and to investigate the effect of age on the results of the SOT.Meth-ods A total of 122 flight crew members were divided into three groups of 20～29(n=57),30～39(n=44),and 40～50 years old(n=21).The EquiTest Dynamic Balance System was applied to test the SOT of 122 flight crew members.Equilibrium score,composite equilibrium score,the sensory weighting score of somatosensory,visual,vestibular,and visual preference,the strategy analysis scores,and the center of gravity alignment under six condi-tions were analyzed.Results The equilibrium scores for conditions 1 to 6 were 94.41±1.47,93.27±1.96,92.57±2.74,85.01±5.43,70.62±7.23,and 69.08±7.84,respectively,and the composite equilibrium score was 81.31±3.38.The sensory weighting scores of somatosensory,visual,vestibular,and visual preference were 98.97±2.08,89.57±5.14,73.89±7.95,and 98.84±5.78,respectively.The strategy analysis scores for conditions 1 to 6 were 95.57±1.23,94.87±1.58,93.93±5.12,88.21±4.01,79.49±7.90,and 80.55±6.57,respectively.There were significant differences in equilibrium score and somatosensory sensory weighting scores for condition 2 across age groups in this study,and the differences in other parameters between age groups were not statistically significant.Conclusion This study establishes normal reference values of SOT for flight crews of different ages to provide an objective reference for the selection of flight cadets and the medical evaluation of flight crews'sensory organization ability of balance function.

Objective To investigate extended high-frequency hearing in young adults and to analyse its char-acteristics.Methods A total of 230 freshmen(101 males and 129 females,aged 17-19 years old)from the class of 2023 at the Air Force Medical University underwent audiometric tests,including acoustic impedance,conventional frequency and extended high-frequency audiometry,and distortion product otoacoustic emissions(DPOAE).Ac-cording to the results of extended high-frequency audiometry,the students were divided into normal and abnormal groups,and the hearing thresholds at conventional frequencies were compared between the two groups.Results Of the 230 students,47.83％(110/230)showed abnormal extended high-frequency hearing.The hearing thresholds of the right ear were 1 to 3 dB higher than those of the left ear at most frequencies.The hearing thresholds of the ab-normal group were higher than those of the normal group in the conventional frequencies(P＜0.05).The difference in extended high-frequency thresholds between the two groups increased with higher frequency.Conclusion Ex-tended high-frequency hearing loss occurs earlier,and has a higher prevalence in young adults,and right ear hearing is worse than that of left ear.Extended high-frequency audiometry can be used as a predictive tool for detecting con-ventional frequency hearing loss.

Objective To investigate extended high-frequency hearing in young adults and to analyse its char-acteristics.Methods A total of 230 freshmen(101 males and 129 females,aged 17-19 years old)from the class of 2023 at the Air Force Medical University underwent audiometric tests,including acoustic impedance,conventional frequency and extended high-frequency audiometry,and distortion product otoacoustic emissions(DPOAE).Ac-cording to the results of extended high-frequency audiometry,the students were divided into normal and abnormal groups,and the hearing thresholds at conventional frequencies were compared between the two groups.Results Of the 230 students,47.83％(110/230)showed abnormal extended high-frequency hearing.The hearing thresholds of the right ear were 1 to 3 dB higher than those of the left ear at most frequencies.The hearing thresholds of the ab-normal group were higher than those of the normal group in the conventional frequencies(P＜0.05).The difference in extended high-frequency thresholds between the two groups increased with higher frequency.Conclusion Ex-tended high-frequency hearing loss occurs earlier,and has a higher prevalence in young adults,and right ear hearing is worse than that of left ear.Extended high-frequency audiometry can be used as a predictive tool for detecting con-ventional frequency hearing loss.

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 identify the pathogenic gene and variant for a family with branchio-otic syndrome.Methods The clinical data of this family were collected,and the peripheral blood was extracted for deafness gene NGS panel analysis.Pathogenic variation detected was verified by Sanger sequencing.Results The family contained 17 members in three-generations,3 of whom exhibited autosomal dominant,hearing loss,preauricular fistula and branchial cleft fistula,which were in accordance with the clinical diagnosis criteria of branchio-otic syndrome.A no-vel heterozygous variant c.963dupT(p.E322X)in EYA1 gene was identified,which co-segregated with the branchi-o-otic syndrome phenotype in the family.The variant was a nonsense variant resulting in the premature appearance of the stop codon.According to the American College of Medical Genetics and Genomics(ACMG)guidelines and the criteria,the variant was classified as pathogenic.Conclusion We identified a novel pathogenic variant EYA1:c.963dupT(p.E322X)in a family with branchio-otic syndrome.

Objective To identify the pathogenic gene and variant for a family with branchio-otic syndrome.Methods The clinical data of this family were collected,and the peripheral blood was extracted for deafness gene NGS panel analysis.Pathogenic variation detected was verified by Sanger sequencing.Results The family contained 17 members in three-generations,3 of whom exhibited autosomal dominant,hearing loss,preauricular fistula and branchial cleft fistula,which were in accordance with the clinical diagnosis criteria of branchio-otic syndrome.A no-vel heterozygous variant c.963dupT(p.E322X)in EYA1 gene was identified,which co-segregated with the branchi-o-otic syndrome phenotype in the family.The variant was a nonsense variant resulting in the premature appearance of the stop codon.According to the American College of Medical Genetics and Genomics(ACMG)guidelines and the criteria,the variant was classified as pathogenic.Conclusion We identified a novel pathogenic variant EYA1:c.963dupT(p.E322X)in a family with branchio-otic syndrome.

Objective To study the clinical features and treatment outcomes of otitis media caused by antineu-trophil cytoplasmic antibody ANCA-associated vasculitis[otitis media with AAV(OMAAV)].Methods A total of 19 cases of OMAAV diagnosed between September 2015 to September 2022 were studied.OMAAV was defined using the criteria introduced by Japan Society of Otology.Results Median age of all patients was 61.8 years old,predominantly proteinase 3(PR3)positive(47.3％).Averaged score of vasculitis activity was 6.6.Disease also in-volved lung(84.2％),nose(73.7％)and facial nerve(21.0％).Pathological biopsy was performed in nine pa-tients,and two reports indicated ANCA-related disease.Fourteen affected ears of eight patients showed mixed hear-ing loss.The administration of corticosteroid together with an immunosuppressant significantly improved hearing,from averaged air conduction of 75.1±23.2 dB HL to 50.5±22.1 dB HL after treatment(P＜0.05).Conclusion Otologists shall be aware of OMAAV when handling intractable otitis media in elderly patients if bone conduction hearing gets worse and there are other affected organs.Immunological serum test is an important diagnostic basis.Most patients were misdiagnosed for certain time,and hearing level might not be improved unless early diagnose and proper treatment.

Objective To establish normal reference values for the sensory organization test(SOT)for flight crews of different ages from 20 to 50 years old,and to investigate the effect of age on the results of the SOT.Meth-ods A total of 122 flight crew members were divided into three groups of 20～29(n=57),30～39(n=44),and 40～50 years old(n=21).The EquiTest Dynamic Balance System was applied to test the SOT of 122 flight crew members.Equilibrium score,composite equilibrium score,the sensory weighting score of somatosensory,visual,vestibular,and visual preference,the strategy analysis scores,and the center of gravity alignment under six condi-tions were analyzed.Results The equilibrium scores for conditions 1 to 6 were 94.41±1.47,93.27±1.96,92.57±2.74,85.01±5.43,70.62±7.23,and 69.08±7.84,respectively,and the composite equilibrium score was 81.31±3.38.The sensory weighting scores of somatosensory,visual,vestibular,and visual preference were 98.97±2.08,89.57±5.14,73.89±7.95,and 98.84±5.78,respectively.The strategy analysis scores for conditions 1 to 6 were 95.57±1.23,94.87±1.58,93.93±5.12,88.21±4.01,79.49±7.90,and 80.55±6.57,respectively.There were significant differences in equilibrium score and somatosensory sensory weighting scores for condition 2 across age groups in this study,and the differences in other parameters between age groups were not statistically significant.Conclusion This study establishes normal reference values of SOT for flight crews of different ages to provide an objective reference for the selection of flight cadets and the medical evaluation of flight crews'sensory organization ability of balance function.