耳聾的病因與分類

1、耳聾的分類與病因,Hearing loss classification & etiology,耳的癥狀學(xué) Ear semeiology,耳痛otalgia耳漏Otorrhea耳癢ear itch耳鳴tinnitus耳聾hearing loss眩暈vertigo面癱 face paralysis耳后腫脹 tumefaction,耳聾的分類,按耳聾病發(fā)的時(shí)間分類 antenatal pr

2、e- birth hearing loss inheritance1.先天性耳聾：出生以前的耳聾 2.后天性耳聾：出生以后的耳聾 postnatal after birth hearing loss,,,,,遺傳,遺傳,非遺傳,非遺傳,耳聾的分類,按病發(fā)的部位分類Hearing loss class according to inflammation part. 1.蝸前聾：傳導(dǎo)性耳聾，外耳、中耳

3、病變。Pre-cochlea hearing loss: conductive hearing loss, external ear and middle ear have inflammation.2.蝸性聾：感應(yīng)性耳聾，耳蝸本身病變。Cochlea hearing loss: sensorineural hearing loss,the cochlea has the inflammation.3.蝸后聾：神經(jīng)性耳聾，大

4、腦聽中樞病變。Central Hearing loss: sensorineural hearing loss, nerve center is the morbid part.,,感音神經(jīng)性耳聾,,傳導(dǎo)性耳聾,耳聾的分類,Conductive hearing loss傳音性（傳導(dǎo)性、蝸前聾）,Sensorineural Hearing loss感音神經(jīng)性（蝸性、蝸后聾）,Mixed hearing loss混合性,Centr

5、um Hearing loss中樞性（蝸后聾）,Hearing loss types,Conductive hearing lossSensorineural hearing lossMixed hearing loss,正常聽力圖 The normal audiogram,頻率 Frequency on the x-axis聲強(qiáng) Intensity on the y-axisFrequency range:

6、125 - 8000 HzIntensity range:-10 - 120 dB (air conduction),,,耳聾程度,,,,PTA(dBHL),國(guó)際標(biāo)準(zhǔn) 我國(guó)標(biāo)準(zhǔn) degree grade type grade,≤25

7、 A 26-40 mild B 41-55 moderate C

8、 2級(jí)重聽 56-70 moderate severe D 1級(jí)重聽 71-90 severe E 2級(jí)聾 ＞90

9、 profound F 1級(jí)聾,,,重聽,聾,我國(guó)聽力“殘疾標(biāo)準(zhǔn)”（1986）與國(guó)際標(biāo)準(zhǔn)（1980）對(duì)照表,傳導(dǎo)性耳聾 Conductive hearing loss,Example of Conductive hearing loss,傳導(dǎo)性耳聾 Conductive hearing loss,1.骨導(dǎo)正常Bone co

10、ndition is normal.2.氣導(dǎo)下降A(chǔ)ir condition3.氣骨導(dǎo)差 > 10 dBA-B gap is more than 10dB.,傳導(dǎo)性耳聾的聽損程度,傳導(dǎo)性耳聾的聽損程度,1.鼓膜穿孔。2.鼓膜穿孔伴聽骨鏈中斷。3.鼓膜和聽骨完全缺如。4.鼓膜完整伴聽骨鏈中斷。5.鼓膜完整+聽骨鏈中斷+卵圓窗封閉。6.外耳道堵塞。,傳導(dǎo)性耳聾的常見病變,耳部畸形Ear malformations

11、耵聹栓塞Impacted earwax外耳濕疹A(yù)cute otitis media外耳道炎Inflammation of the ear canal外耳道塌陷Dent of the ear canal耳道擴(kuò)大Expand of the ear canal異物eyewinker,鼓膜穿孔Perforated eardrum流膿P(yáng)urulence穿孔后愈合Scar over after Perforated

12、腫瘤或膽脂瘤Exostoses and osteomas鼓膜疤痕Eardrum scar鼓膜硬化Eardrum sclerosis,常見的中耳疾病,化膿性中耳炎Suppurative otitis media 分泌性中耳炎Secretory otitis media 耳硬化癥Otosclerosis 聽骨鏈固定Fixation of the ossicular chain 　聽骨鏈中斷Interruption o

13、f the ossicular chain 鼓室硬化tympana sclerosis,Congenital malformations,(Engström and Engström, 1979, pp.4),Impacted earwax,Courtois, pp. 10,Perforated eardrums,Perforation of eardrum,Exostosis,Osteoma,Acute ot

14、itis media,Chronic otitis media,Otosclerosis,Treatment of conductive hearing loss,MedicineOperationGuidanceHearing instruments,感因神經(jīng)性耳聾 Sensorineural loss,,響度改變 Altered loudnessPoorer sensitivity to soft and intermedi

15、ate soundsSome times increased sensibility to loud sounds失真 Distortion (missing frequencies)言語(yǔ)分辨率下降 Poor discrimination (especially in noise condition)耳鳴 Tinnitus,伴隨癥狀A(yù)ssociated problems,Relationship of loudness

16、 and intensity level,Stach, 1998 pp. 97,Frequency resolving ability,Stach, 1998 pp. 96,,Sensorineural hearing loss,1.氣骨導(dǎo)都下降。Air conductive decline2.氣導(dǎo)早期以高頻下降為主，晚期低頻也下降。High frequency decline at first time, then both

17、decline.3.氣骨導(dǎo)差 < 10 dBThere is no a distance show between the bone condition and air condition.,感音神經(jīng)性聾病因,老年性耳聾噪聲性耳聾突發(fā)性耳聾迷路炎Labyrinthitis 顳骨骨折 temporal bone fracture非器質(zhì)性聾心因性聾或功能性聾偽聾,Retrocochlear loss,He

18、reditary hearing loss,Noise induced hearing loss,Congenital (german measles),Ménières disease,Age related hearing loss,Hereditary + age,Treatment of sensorineural losses,Hearing instruments,混合性耳聾 Mixed hearing

19、loss,,Example of mixed loss,混合性耳聾 Mixed hearing loss,1.骨導(dǎo)下降Bone condition declines.2.氣導(dǎo)下降A(chǔ)ir condition3.氣骨導(dǎo)差 > 10 dBA-B gap is more than 10dB,耳 聾 相 關(guān) 疾 病,耳聾分類,時(shí)間- congenital & acquired性質(zhì)- organic & funct

20、ional/psychogenic 部位- conductive, sensorineural & mixed cochlear, retrocochlear & central病因,,耳聾病因,中耳疾病及耳硬化癥先天性聾中毒性聾感染性聾老年性聾爆震性聾及噪聲性聲損傷內(nèi)耳免疫及自身免疫性內(nèi)耳病全身及其它器官系統(tǒng)的慢性疾病引起的耳聾特發(fā)性突聾外淋巴瘺蝸后

21、性聾詐聾和精神性聾,中耳疾病,急性/慢性化膿性中耳炎耳硬化癥原因不明 原發(fā)于骨迷路 局灶性病變鐙骨性-環(huán)韌帶 鐙骨活動(dòng)受限/固定 CHL耳蝸性-耳蝸/內(nèi)聽道 SNHL雙側(cè) 20-40y 緩慢進(jìn)行性加重 聽力圖：早期氣導(dǎo)上升型－平坦型Carhart切跡（2kHz 15dB) 聲導(dǎo)抗：AsCT：骨迷路、兩窗區(qū)/內(nèi)耳道骨壁上界限分明的局灶性硬化改變,先天性聾,遺傳性 遺傳方式 病變部位 發(fā)病時(shí)間非遺

22、傳性 ⒈ 產(chǎn)前期母體的感染、中毒 ⒉ 分娩期窒息、外傷,中毒性聾,氨基甙類抗生素 鏈霉素、卡那霉素、慶大霉素、新霉素某些抗腫瘤藥 順鉑、卡鉑、氮芥袢利尿劑 速尿、利尿酸水楊酸制劑 阿司匹林（停藥后一般可迅速消失）奎寧 局部麻醉藥 的卡因、利多卡因（輕度，各個(gè)頻率，可恢復(fù)）重金屬 鉛、鎘、汞、砷（不可逆蝸后性聾、伴前庭損害）吸入性有害氣體 氨基苯、CO、CS2、SO2（內(nèi)

23、耳、中樞）其它 萬(wàn)古霉素、多粘菌素B中成藥 牛黃清心丸（雄黃為砷劑）,感染性聾,腮腺炎- 兒童 單側(cè) SNHL 高頻 不可逆麻疹- 雙耳 不對(duì)稱性 SNHL 高頻 不可逆帶狀皰疹- 單側(cè) 前庭 SNHL 可逆細(xì)菌性腦膜炎- 10-20% 早期 雙耳 重度 波動(dòng)梅毒- 3-38% 早期/晚期 雙側(cè) 突發(fā) 前庭 重度,老年性聾,原因：聽覺系統(tǒng)衰老癥狀：不明原因 雙側(cè) 緩慢進(jìn)行性加重SNHL-高頻為主 言語(yǔ)識(shí)別能力明顯下降

24、,聲創(chuàng)傷,爆震性/急性- 中耳 內(nèi)耳噪聲性聲創(chuàng)傷- 世界性七大公害之首強(qiáng)度 時(shí)間 頻譜（高頻>低頻，窄帶>寬帶）影響聽閾頻率 損傷部位（外毛-內(nèi)毛細(xì)胞，蝸底-蝸?lái)敚?000Hz切跡,自身免疫性內(nèi)耳?。ˋIED）,自身免疫性損害累及耳蝸&/前庭臨床特點(diǎn) 中年女性 雙側(cè)不對(duì)稱或單側(cè)快速進(jìn)行性/波動(dòng)性 蝸性或蝸后性HL 伴其它自身免疫性疾病 病程較長(zhǎng)（>數(shù)周）診斷 缺乏敏感可靠

25、的試驗(yàn)診斷方法治療 高劑量類固醇藥物和免疫抑制劑 少數(shù)經(jīng)恰當(dāng)治療可獲較好療效的內(nèi)耳病之一,外淋巴瘺,原因：　外淋巴和中耳腔之間的骨質(zhì)破損　膜性組織/韌帶破裂　　　　　　致使外淋巴和中耳腔交通診斷：　　有使中耳或顱內(nèi)壓突然升高的病史　　單耳 突發(fā)性SNHL 伴前庭癥狀　　治療后眩暈不減輕 & SNHL逐漸加重,,特發(fā)性突聾,突然發(fā)生 原因不明 數(shù)分鐘、數(shù)小時(shí) 單耳 高頻SNHL 50％伴

26、眩暈7-10d內(nèi)治療效果較好1/3 1/3 1/3,全身性疾病,甲狀腺功能減退癥腎臟疾病糖尿病高血壓和高血脂癥血液性疾病,助聽器選配對(duì)象/適應(yīng)癥,有殘余聽力藥物或手術(shù)治療無(wú)效，病情穩(wěn)定 傳導(dǎo)性聾　　　耳硬化癥　　　特發(fā)性突聾　　　前庭導(dǎo)水管擴(kuò)大綜合癥,骨導(dǎo)助聽器選配適應(yīng)癥,外耳道閉鎖、狹窄、長(zhǎng)期流膿等不適宜用耳塞者1k和2kHz ABG HL > 40dB,驗(yàn)配助聽器的步驟,詢問病史

27、To ask experience in the past: 聽力不好有多長(zhǎng)時(shí)間了？做過哪些檢查和治療？是否戴過助聽器,效果怎樣? how long you have hearing loss? Did you see a doctor?did you wear a hearing aids?how about your feeling?檢查耳部To look over ear: 耳廓外型是否

28、正常？外耳道是否通暢？ 是否有分泌物？如果有是干性還是濕性,鼓膜是否完整？有無(wú)鼓膜內(nèi)陷、中耳積液、穿孔、炎性息肉等 。 auricle shape?external ear canal?ear drum?middle ear ？Cerumen?,驗(yàn)配助聽器的步驟,3.檢查聽力并對(duì)照檢查結(jié)果作出分析。To analysis hearing test’s result. 4.選擇功率和聽力曲線匹配的助聽器進(jìn)行調(diào)試。To

29、 choose the suitable hearing aids.5.使用電腦軟件、聲場(chǎng)或Aurical的長(zhǎng)期會(huì)話圖譜測(cè)試效果。To evaluate result by using the computer software ,sound field, Aurical. 6. 詢問需要的檔次,說(shuō)服并達(dá)成協(xié)議。To ask some detail and persuade him to purchase hearing aid