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《Chinese Journal of Otorhinolaryngology》 2003-03
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Clinical analysis of 95 patients with large vestibular aqueduct syndrome

YANG Wei yan, ZHANG Su zhen, ZHAO Cheng jun, FENG Bo Department of Otorhinolaryngology Head & Neck Surgery, Chinese PLA General Hospital, Beijing 100853, China Corresponding author: YANG Wei yan (Email: ywy301@263 net)  
Objective To investigate the occurrence, genetic inheritance, and hearing loss conditions of large vestibular aqueduct syndrome (LVAS), and to measure the width of external aperture of vestibular aqueduct (VA) of LVAS, and to analyze the relationship between the hearing loss and the width of external aperture Methods A retrospective review was conducted by the history, auditory, and vestibular function examinations in 95 patients (190 ears) of LVAS The width of VA was measured by CT scaning criterion of each LVAS patients in the past 10 years (from 1992 to 2002) Results In 95 patients, there were male 65 cases and female 30 cases The male cases was more than the females by a ratio of 2 2∶1 Twelve cases (12 6%) would have familial genetic history The average of the consult age was 7 6 years (range from 1 2 to 39 years) The most patients were misdiagnosed, the hearing deterioration originated from mild head trauma in 21 cases (22 1%), the hearing loss after common cold in 13 cases (13 6%) Nine cases (9 5%) misdiagnosed as sudden hearing loss, 5 cases (5 3%) as ototoxic hearing loss of Aminoglycoside antibolic The profound hearing loss was found in 172 ears (92 4%) The average width of external aperture of LVAS was 7 5 mm ±1 2 mm ( ±s , 77 cases, 154 ears) The level of hearing loss did not proportional to the width of VA The hearing impairment could be derived from protein rich endolymph refluxed into the cochlear and vestibule Conclusion The LVAS is distinct clinical entity characterized by fluctuate and progressive SNHL The diagnosis depends upon the CT scan and MR image If the hearing fluctuated and subsequently improved, the children were kept under conservation treatment If the significantly decreased of hearing effected school performance, the cochlear implant might be considered
【CateGory Index】: R764
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