评论
打印
Otosclerosis
耳硬化症
Otosclerosis also called otospongiosis, is disease of bony labyrinth and ossicles in ear who become knitted together into an immoveable mass.
耳硬化症又称耳海绵病，是内耳骨迷路和骨质重塑异常导致的疾病。
It mostly involves otic ganglion and stapes in which normal bone is replaced by spongy bones.
它主要累及耳神经节和镫骨，其中正常的骨被海绵骨取代。
Causes
原因
It is mostly inherited as "autosomal dominant" pattern so patients have positive family history.
多为常染色体显性遗传，患者有阳性家族史。
It is more common in white races.
该疾病在白种人更为多见。
It affects females twice as males and mostly discovered during or just after pregnancy.
女性发病率约为男性的两倍，大多是在怀孕期间或刚怀孕后发现的。
Mostly starts between 20-30 years of age.
大多开始于20-30岁之间。
Chronic measles infection predisposes patient to otosclerosis.
慢性麻疹感染使患者易患耳硬化症。
Otosclerosis is often associated with ‘osteogenesis imperfecta’ called ‘Van der Howe Syndrome’.
耳硬化症通常与“成骨不全症”即“范德豪综合症”有关。
Syndrome consists of otosclerosis, osteogenesis imperfecta and blue sclera of eyes.
综合症状包括耳硬化症、成骨不全症和蓝巩膜。
Symptoms
症状
Patient presents with bilateral conductive hearing loss or mixed type; both conductive and sensorineural hearing loss, tinnitus, vertigo, monotonous speech and Paracusis Willisii (patient hearing better in noisy environment).
患者症状表现为双侧传导性或混合型耳聋，传导性和感觉神经性听力丧失、耳鸣、眩晕、言语单调、威利斯误听（患者在嘈杂环境中听力较好）。
On Examination
相关检查
Tuning Fork Tests
音叉检查
Negative Rinne and Weber lateralized to diseased ear (conductive types).
单侧病耳林内氏和韦伯检测呈阴性（传导型）。
Eustachian tube function is normal.
咽鼓管功能正常。
On Audiometric testing:
听力测试：
1) Otoscopy: Reddish hue on tympanic membrane called "schwartz sign".
1）耳镜检查：鼓膜呈红色，称为“施瓦茨症”。
2) Pure Tone Audiometry (PTA): Shows dip at 2,000Hz, called "carhart's notch".
2）纯音测听：表现为2000Hz时，听力下降，称为“卡哈切迹”。
3) Tympanometry: Curve of ossicular stiffness present.
3）鼓室导抗测试：听骨硬度曲线。
Treatment
治疗
1) Non Surgical: a) sodium fluoride as dietary supplement b) hearing aids for sound amplification.
1）非手术治疗：a）氟化钠作为膳食补充剂；b）佩戴助听器。
2) Surgical Stapedectomy with prosthesis, in which diseased stapes is replaced.
2）手术治疗：切除患者的镫骨，植入人工镫骨。
Contra-Indication for surgery:
手术禁忌证：
a) Only hearing ear b) Associated Meniere's disease c) Young children d) Athletes e) Frequent air travellers f) Divers.
a）有听觉的耳朵 b）有梅尼埃病的患者 c）幼儿 d）运动员 e）经常乘飞机旅行 f）潜水员。
