Clinical Epidemiological Study Of Patients With Congenital Malformation Of Middle And Outer Ears

Part oneThe general Clinical Epidemiological Study of Patients with Congenital Deformity of middle and Outer EarsObjective:To investigate the general clinical epidemiologic characteristics of congenital deformation of middle and outer ears(CDMUE).Methods:Total of 551 cases(650 ears)with CDMUE were analyzed,including inpatients 371 cases(448 ears)from 2006.4-2017.11 and outpatients 180 cases(202ears)from 2016.10-2017.11 in our hospital,in which 460cases(543ears)external auditory canals can be identified by physical examination,375 cases(485 ears)with photos for detecting deformity of the head,neck and limb,excluding other complications 223cases(264ears)as objects for analysis of auricle deformity and pure-tone threshold,185 cases(198 ears)undertaking the external auditory canal reconstruction and/or exploratory tympanotomy,80cases(88 ears)for studying ossicular development,40 cases with cardiac ultrasound and 36 cases with renal ultrasound.Results:Male 403 cases(73.1%)>females148(26.9%),right side 75 cases(49.9%),left side 177 cases(32.1%),and bilateral microtia 99 cases(18%).the grade III of microtia accounted for 83%(219/264),the severity of microtia is positive correlated with the pure tone auditory threshold.Atresia of external auditory canals up to 90.6%(492/543),and 7.6%(41/543)for stenosis.100%(88/88)auditory ossicle deformation.The ratio of multiple deformations as follows:HFM 67.2%(252/375 cases),facial paralysis 7.5%(28/375 cases),fingers/toes deformity 1.3%(5/375 cases),deputy ear 14%(68/485 ears),preauricular fistula 10.7%(52/485 ears),tragus deformation 7.4%(36/485 ears).Distant organs deformation for heart 5.0%(2/40 cases),kidney 2.8%(1/36 cases).The incidence of complications is 16.2%(32/198 cases)for cholesteatoma of the external auditory canal,3.0%(6/198 cases)for posterior ear abscess and 2.5%(5/198)for cholesteatoma of the middle ear.There were 4%(15/375 cases)for Treacher-Collins syndrome and 2.4%(9/375 cases)for Goldenhar syndrome.Conclusions:The incidence of CDMUE was more the male than the female,and the right side than the left side.and the third degree of microtia was the most.The more severity of deformation,the worse of pure tone hearing.98.2%of the patients with congenital external auditory deformity were atresia or stenosis,and 100%ossicular deformity.Multiple deformations were HFM,facial paralysis,fingers/toes deformity,deputy ear,preauricular fistula,tragus deformation.preauricular fistula,and deformity of the ear.Common complications include cholesteatoma of external auditory canal and/or middle ear,and posterior ear abscess.Distant organs deformation for heart and kidney.CDMUE patients may have syndrome.Part twoCharacteristic analysis of Pure tone Test for Patients with Congenital Deformity of Middle and outer earsObjective:To analyze the Characteristics of Pure tone Test for Patients with Congenital Deformity of Middle and outer ears(CDMUE).Methods:A retrospective analysis of the characteristics of pure tone test in 213 cases(253 ears)with CDMUE.Results:conductive deafness 198 ears(78.26%),mixed deafness 53 ears(20.95%),and sensorineural deafness only 2 ears(0.78%).206 cases(246 ears)meeting critition are analyzed for the characteristics of pure tone,the average air conduction threshold analysis:moderate-severe deafness(56-70dB HL)51.63%(127/246)and severe deafness 33.74%(83/246).The conductive and mixed deafness patients in moderate-severe and severe accounted for 59.60%(118/198),26.77%(53/198)and 18.75%(9/48),62.50%(30/48)respectively.Among which 56-80 dB HL 82.83%(164/198)for conductive deafness,and 56-80 dB HL 58.33%(28/48)for mixed deafness.Bone conduction hearing loss happened mainly at 2KHz(72.92%),and the average threshold was 30.42±7.84dB HL(10-60 dB HL).Conclusions:patients with CDMUE show mainly conductive deafness,and moderate-severe and severe deafness.While bone conduction hearing loss happened mainly and slightly at 2KHz.Part threeThe analysis of the relationship between pure tone threshold and development of oval window and ossicles in patients with Congenital Deformity of Middle and outer earsObjective:To analyze the relationship between pure tone threshold and development of oval window and ossicles in patients with Congenital Deformity of Middle and outer ears.Methods:53 cases(53 ears)were divided into group A(oval window development)36 cases(36 ears)and group B(oval window occlusion)17 cases(17 ears).And Group A was divided into group A1(stape deformity)23 cases(23 ears)and group A2(stape development,malleus and/or incus deformity)13 cases(13 ears).Statistical analysis was performed on pure tone hearing at each frequency and mean language frequency hearing thresholds between groups.RESULTS:For group A(oval window development),only 250 Hz the auditory threshold was over 70 dB and the other frequency and average threshold in 0.5-4 kHz less than 70 dB HL.In group B(oval window occlusion),only 4kHz and 8kHz the auditory threshold was less than 70 dB HL,the other frequency hearing threshold and average threshold in 0.5-4 kHz all over 70 dB HL.The hearing threshold at any frequency in group B(oval window occlusion)was greater than that in group A(oval window development),and there were statistical differences in hearing thresholds at 0.125kHz,1kHz,2kHz,4kHz and average speech threshold(0.5-4 kHz).There was no significant difference in hearing threshold between the A1 group(stape deformity)and A2 group(stape development,malleus and/or incus deformity)at all frequencies.However,the percentages of patients with hearing threshold<60 dB in the average speech frequency of A1 and A2 groups were 21.74%(5/23)and 61.54%(8/13),respectively,and there was statistical significance.Conclusion:The threshold value is correlated with the degree of deformity,so the results of pure tone threshold can help estimate the oval window and ossicle development of congenital middle and outer ear deformity.