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先天性外淋巴瘘(CPLF)为颞骨先天性薄弱或缺损致外淋巴液(腑脊液)自发性漏入中耳,无外伤和用力病史。作者对匹兹堡儿童医院1983～1986年收治的244例不明原因的感音神经性聋(SNHL)、眩晕或两者皆有的患儿,进行前瞻性研究。其中男126,女118,年龄5月～17.1岁,平均6.05岁。所有患儿均做了脑干电反应或行为观察测听,并于初测后每3月随访复查一次,时限2年。大多数患儿尚做了颞骨CT扫描和常规X线摄影。统计244例患儿中132例(54％)双侧SNHL有CPLF症状,85例(35％)单侧耳聋伴眩晕,单有眩晕者仅6％(15/244)。在244例SNHL患儿中有CPLF症状者共229例为94％,但出现眩晕症状者仅11％(27/244)。
Congenital external lymphatic fistula (CPLF) is a congenital weakness or defect of the temporal bone caused by spontaneous leakage of extra-peritoneal fluid into the middle ear without any history of trauma and force. The authors undertook a prospective study of 244 unexplained sensorineural hearing loss (SNHL), dizziness, or both in the Pittsburgh Children’s Hospital from 1983 to 1986. Male 126, female 118, age from May to 17.1 years, with an average of 6.05 years old. All children were brainstem response or behavioral observation audiometry, and in the initial test every 3 months follow-up review time limit of 2 years. Most children have done a temporal bone CT scan and conventional radiography. There were 132 cases (54%) of bilateral SNHL with symptoms of CPLF in 244 cases, unilateral deafness with vertigo in 85 cases (35%) and only 6% (15/244) in patients with vertigo alone. A total of 229 patients with symptomatic CPLF in 244 children with SNHL were 94%, but only 11% (27/244) had vertigo symptoms.