Objective To investigate the relationship between postoperative cochlear implant electrade inser-tion depth,the scalar localization of cochlear electrode,cochlear size and coiling pattern,and the factors influencing the postoperative electrode implantation depth and the reasons of dislocation.Methods A total of 41 cases of pa-tients implanted with electrode of SONATA ti100 Standard and 30 cases of patients with CI24RE(CA)modilar elec-trode were studied.Postoperative insertion length,insertion depth angle of cochlear electrode,and the scalar locali-zation of cochlear electrode were measured.The relationships of the above factors and the parameters of cochlear size,cochlear length,tilt angles collectal via CT scan preoperatively were analyzed.Results ① The measurement results showed the insertion depth angle and liner insertion length varied greatly among individuals.The mean inser-tion depth angle was 702±53 degrees and the mean liner insertion length was 30.02±1.29 mm in the group of SO-NATA ti100 Standard.The mean insertion depth angle was 441±45 degrees and the mean liner insertion length was 18.4±1.0 mm in the group of CI24RE(CA).② The length and width of cochlear base both had negative corre-lations with the insertion depth angle in the two groups[SONATA ti100 Standard:r=-0.768,P＜0.001 & r=-0.678,P＜0.001;CI24RE(CA):r=-0.467,P=0.008 & r=-0.471,P=0.008].The liner insertion length of the electrode had a positive correlation with the insertion depth angle in the two groups[SONATA ti100 Standard:r=0.578,P＜0.001;CI24RE(CA):r=0.748,P＜0.001].③ 6 cases of modiolar electrode were dislo-cated and the site of the dislocation was at 180 degrees of cochlea.The tilt angle within the first turn of cochlea and angle between the first and second turn of cochlea had significant differences between the group of patients with dis-location and without dislocation(10.28 degrees vs 8.75 degrees,P=0.006;15.25 degrees vs 14.00 degrees,P=0.033).Conclusion The insertion depth angle and the insertion length of electrode varied greatly among individu-als.These differences are related to the cochlear size.The difference in cochlear coiling pattern is one of the reasons for dislocation of electrode.

Objective:To report the experience of using CT-guided cochlear implant surgery in difficult cases such as severe inner ear deformities and anatomical abnormalities, and to discuss the application value of intraoperative CT-assisted localization in difficult cases of cochlear implant surgery. Methods:Retrospectively analyzed the clinical data of 23 cases of difficult cochlear implant surgery cases completed by our team with the assistance of intraoperative CT, and collected their medical data, including preoperative imaging manifestations, surgical conditions, and intraoperative imaging images for evaluation. Results:During the study period, 23 difficult cases（27 ears） underwent cochlear implantation under the guidance of intraoperative CT, and 4 cases were bilaterally implanted. Including 6 cases of incomplete segmentation type Ⅰ（IP-Ⅰ）, 1 case of incomplete segmentation type Ⅱ（IP-Ⅱ）, 10 cases of incomplete segmentation type Ⅲ（IP-Ⅲ）, 3 cases of common cavity deformity（CC） and 3 cases of cochlear ossification after meningitis. Facial nerve anatomy was abnormal in 9 cases, cerebrospinal fluid "blowout" was serious in 14 cases, electrode position was abnormal in 3 cases requiring intraoperative adjustment of electrode position, anatomical difficulties required intraoperative CT to assist in finding anatomical landmarks in 2 cases, and electrodes were not fully implanted in 3 cases. Conclusion:When faced with difficult cases with challenging and complex temporal bone anatomy, intraoperative CT can accurately evaluate the electrode position and provide intraoperative anatomical details, allowing immediate adjustment of the electrode position if necessary, providing safety guarantee for difficult cases of cochlear implant surgery and ensure accurate implantation of electrodes.
Humans ; Cochlear Implantation/methods* ; Retrospective Studies ; Tomography, X-Ray Computed/methods* ; Cochlea ; Cochlear Implants

Objective:To analyze the genetic and clinical characteristics of MYO15A variants associated non-syndromic autosomal recessive deafness3 (DFNB3).Methods:The hearing test and high-throughput sequencing data of 108 families with non-syndromic hearing loss, who visited the Center of Genetics and Prenatal Diagnosis in the First Affiliated Hospital of Zhengzhou University from November 2016 to February 2019, were retrospectively analyzed to investigate the characteristics of MYO15A variation.Results:Compound heterozygous MYO15A variations were detected in nine patients from eight families, accounting for 7.4% of all 108 families. The variants were c.5910+1G>A/c.9417_9418insTA, c.4234T>G/c.8324G>T, c.3926A>T/c.5002delC, c.9690+1G>A/c.10257_10259delCTT, c.8324G>T/c.10419_10423delCAGCT, c.4519C>T/c.6454G>C, c.6177+1G>T/c.10257_10259delCTT and c.5692C>T/c.7396-1G>A. All patients had severe to profound hearing loss. Among the 14 variations, 12 variations were located in the main structural domains, including 5 in motor domain, 3 in FERM domain, 3 in MyTH4 domain and 1 in IQ motif. The c.3926A>T, c.4234T>G, c.4519C>T, c.5002delC, c.6454G>C, c.8324G>T, c.9417_9418insTA and c.10419_10423delCAGCT had not been reported in the Human Gene Mutation Database up to February 2020. According to the guidelines of the American College of Medical Genetics and Genomics (ACMG), 6 reported variants and the first reported c.4519C>T, c.5002delC, c.9417_9418insTA and c.10419_10423delCAGCT were identified as pathogenic variants, while c.8324G>T was likely pathogenic variant, and c.3926A>T, c.4234T>G and c.6454G>C were variants of uncertain significance.Conclusions:The variations of MYO15A in patients with DFNB3 are mainly complex heterozygous. The clinical phenotypes are mostly severe to profound hearing loss, and the mutation loci are mainly in the motor, FERM and MyTH4 domains.

Objective To study the age, the degree of hearing loss and the characteristics of inner ear imaging in children with GJB2 and SLC26A4 gene mutation-related deafness.Methods A total of 218 children with GJB2 and SLC26A4 gene mutations were enrolled in this study.Among them, with the combined test of deafness gene chip and DNA sequencing, 123 patients were diagnosed with GJB2 homozygous or complex mutations, and 95 patients were diagnosed with SLC26A4 homozygous or complex mutations.The age of the onset, the degrees of hearing loss and CT features of the temporal bone in children with GJB2 and SLC26A4 mutations were studied.Results The incidence of GJB2 and SLC26A4 gene mutations was 43.09%, 37.40%, 14.63%, 4.88% and 24.2%, 44.21%, 18.95% and 12.63% in the periods of infancy,early childhood,preschool and shoolage,respectively.The age composition of onset in the two groups showed statistical significance(P=0.014).The constituent ratio of children with moderate, severe and extremely severe degrees of hearing loss in the two groups with GJB2 and SLC26A4 gene mutations were 8.94%, 17.89%, 73.17% and 9.47%, 34.74% and 55.79%, respectively.Most of the group with GJB2 gene mutation had profound hearing loss, and the composition ratio of hearing loss degree in SLC26A4 group was statistically significant(P=0.014).99.19% of the children with GJB2 gene mutation group had normal structures of the inner ears.Only one case of CT showed bilateral internal auditory canal stenosis.For 95.79% of the children with SLC26A4 gene mutation, the CT results of the temporal bone were associated with the vestibular aqueduct expansion.Conclusion The onset age of GJB2 gene mutation children is concentrated in the infancy.Most of them are with very severe sensorineural deafness, not associated with the inner ear malformation.The onset age of SLC26A4 gene deafness children is concentrated in the early childhood.Most of them are with severe and extremely sever sensorineural deafness, closely related to vestibular aqueduct expansion and inner ear malformations.

Objective To explore the expressions of annexin and epidermal growth factor receptor(EGFR) in pediatric middle ear cholesteatoma.Methods Twenty-three children with middle ear cholesteatoma and 26 children with normal skin of external auditory canal(control group) were selected from the children enrolled in the First Affiliated Hospital of Zhengzhou University from August 2014 to March 2016.The expressions of annexin A1 (AnxA1),AnxA2 and EGFR mRNA in cholesteatoma and normal tissues were analyzed by quantitative real-time PCR (qPCR).Protein expressions of AnxA1,AnxA2 and EGFR were evaluated by using Western blot and immunohistochemistry methods.Results The expressions of AnxA1,AnxA2 and EGFR mRNA were significantly increased in cholesteatoma compared with the control group (AnxA1:4.68 ± 1.77 vs.2.65 ± 0.96,U =111.5,P ＜ 0.001;AnxA2:3.89 ± 1.00 vs.2.4 7 ± 0.81,U =84.5,P ＜ 0.001;EG FR:4.97 ± 1.85 vs.3.50 ± 0.95,U =15 3.5,P =0.004).AnxA1 and AnxA2 mRNA expressions were positively correlated with EGFR mRNA (AnxA1 and EGFR:r2 =0.283 2,P =0.009;AnxA2 and EGFR:r2 =0.213 5,P =0.027).Compared with the control group,protein expressions of AnxA1,AnxA2 and EGFR were markedly enhanced (AnxA1:0.450 ±0.031 vs.0.320 ±0.026,U =102.4,P ＜0.001;AnxA2:0.568 ±0.024 vs.0.365 ±0.028,U =94.6,P ＜0.001;EGFR:0.397 ±0.021 vs.0.228 ±0.017,U =128.4,P ＜0.001).The results of immunohistochemistry analysis showed that the ratio of AnxA1,AnxA2 and EGFR positive cells were higher than those in the control group(AnxA1:65.22％ vs.38.46％,x2 =9.296,P =0.026;AnxA2:69.57％ vs.46.15％,x2 =8.378,P =0.039;EGFR:69.57％ vs.50.00％,x2 =10.574,P =0.014).Conclusions The expressions of AnxAl,AnxA2 and EGFR are upregulated in pediatric cholesteatoma,with AnxA1 and AnxA2 expressions positively correlated with EGFR.

Objective To study clinical characteristics and treatment effects on old patients with sudden sen-sorinerural hearing loss .Methods To compare clinical characteristics and effects of 357 cases of unilateral SSHL from Sep ,2013 to Mar ,2015 in the First Affiliated Hospital of Zhengzhou University .The patients were divided into 2 groups :the old group(66 cases)≥60 years ,and the control group(291 cases)<60 years .All cases were trea-ted with drugs to supply nutrition to the nerves and drugs to improve circulation and intratympanic injection of dexa-methasone .Results The genders ,ear sides ,concomitant symptoms and onset time ,degrees of hearing in 2 groups showed no significant difference (P>0 .05) ,but there were less patients with the type in low frequencies (1 .51% ) and more with the type in high frequencies (22 .73% ) and the type in total deafness (50 .0% ) than those of in the control group(14 .43% ,15 .44% ,39 .52% ) .The old group had more patients with systemic diseases (60 .61% ) and the hearing impairment of the other ear (57 .58% ,P<0 .05) .Total effective rate was 70 .03% ,which the old group and the control group were 60 .61% and 72 .16% ,respectively .The difference had no statistical significance (P>0 .05) .The old group's complete recovery rate was 10 .61% ,which was significantly lower than that in the control (P<0 .05) .Conclusion The old group has more patients with systemic diseases and the hearing impairment of the other ear .Their hearing impairment more easily occurs in the type in high frequencies and the type in total deafness . But the complete recovery rate is low ,the doctors and patients should have a reasonable expectation .

Objective To investigate the recurrent etiologic causes in otitis media with effusion(OME).Meth-ods The patients with OME had been conducted clinical evaluation and ventilation tube insertion.Among 255 cases observed,85 cases were recurrence within one year,while 170 cases were recovered.Factors including age,gender, course of disease,smoking environment,recurrent respiratory tract infection,chronic nasal sinusitis,nasal struc-tural abnormalities,adenoid hypertrophy/nasopharyneal lymphadenosis,and mastoid gasification condition,histo-ry of tube insertion,duration of ventilation tube were evaluated by univariate analysis and multivariate logistic re-gression analysis and compared among preschool children,school age children and adults.Results In univariate a-nalysis,it was strongly associated with age,higher prevalence of recurrent respiratory tract infection(χ2= 22.546,P= 0.000),chronic nasal sinusitis(χ2= 5.211,P= 0.023),adenoid hypertrophy/nasopharyneal lymphadenosis(χ2= 10.338,P= 0.002),mastoid pneumatization adverse(χ2= 15.196,P= 0.000)and duration of ventilation tubeand (χ2= 11.347,P= 0.001).In multivariate logistic regression model,five of these factors were found to be predictors of recurrent OME,which were younger age,recurrent respiratory tract infection (P= 0.001,OR= 2.992),adenoid hypertrophy/nasopharyneal lymphadenosis (P= 0.021,OR= 2.198),mastoid pneumatization adverse (P= 0.000, OR= 3.433)and duration of ventilation tube(3~6 m)(P= 0.010,OR= 2.237).When comparing the difference a-mong preschool children,school age children and adults,recurrent respiratory tract infections in preschool and school age children had the statistic significance(P<0.05),and adenoid hypertrophy in preschool had the statistic significance(P<0.05).Conclusion Younger age,recurrent respiratory tract infection,chronic nasal sinusitis,ade-noid hypertrophy/nasopharyneal lymphadenosis,mastoid pneumatization adverse and less time of ventilation tube are the risk factors causing recurrence of OME.When comparing the recurrent preschool children,school age chil-dren and adults cases,more recurrent respiratory tract infection in preschool and school age children have statistic significance,and adenoid hypertrophy in preschool has the statistic significance.

Objective To analyze the mutations ofPAX3 gene in two Waardenburg syndrome type Ⅰ (WS1) pedigrees and make prenatal diagnosis for the high-risk 18-week-old fetus.Methods PAX3 gene was first analyzed by Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) for detecting pathogenic mutation of the probands of the two pedigrees.The mutations were confirmed by MLPA and Sanger in parents and unrelated healthy individuals.Prenatal genetic diagnosis for the high-risk fetus was performed by amniotic fluid cell after genotyping.Results A heterozygousPAX3 gene gross deletion (E7 deletion) was identified in all patients from WS1-01 family,and not found in 20 healthy individuals.Prenatal diagnosis in WS1-01 family indicated that the fetus was normal.Molecular studies identified a novel deletion mutation c.1385_1386delCT within thePAX3 gene in all affected WS1-02 family members,but in none of the unaffected relatives and 200 healthy individuals.Conclusions PAX3 gene mutation is etiological for two WS1 families.Sanger sequencing plus MLPA is effective and accurate for making gene diagnosis and prenatal diagnosis.

Objective The aim of this study was to evaluate the outcomes of auditory perception skills after cochlear implantation in children with malformed inner ear and compare them with a group of congenitally deaf chil ‐dren implantees with a normal inner ear .Methods 21 children with inner ear malformation were retrospective stud‐ied .There were 9 cases with large vestibular aqueduct syndrome (LVAS) ,7 with Mondini abnormality ,5 with com‐mon cavity .The postoperative outcomes of these 21 cases were compared with 21 cases with normal inner ear struc‐ture .The outcomes of all the children after the surgery in 1 year were studied using the soundfield test in frequency ranging 0 .5 to 4 kHz and the auditory perception skills ,and the auditory perception skills consisted of natural envi‐ronmental sound recognition ,consonants recognition ,vowels recognition ,numeral recognition ,tone recognition , monosyllabe recognition ,disyllabe recognition ,trisyllabe recognition ,short sentences recognition ,selective hearing recognition .Results The results of soundfield test and auditory perception skills after cochlear implantation in 9 children with LVAS and 6 with Mondini abnormality had no significant difference (P > 0 .05) comparing with the control cases .Postoperative thresholds in soundfield test were 50 ~ 75 dB HL for 1 case with severe Mondini abnor‐mality ,the mean value of hearing ability score was 70 .5% ,and less than the results of control cases .Postoperative mean thresholds in soundfield test were 65 .26 ± 5 .13 dB HL for 5 cases with common cavity ,the hearing ability score was less than the results of control cases (P< 0 .05) .Conclusion The effect of rehabilitation had no difference between the children with LVAS and the cases with normal inner structure after cochlear implantation ,but was poo‐rer in children with severe Mondini abnormality and common cavity .It was necessary to evaluate the degree of mal‐formation of inner ear structure before cochlear implantation .