The Clinical Study Of The Large Vestibular Aqueduct Syndrome

Objective: To explore the risk factors influenting vestibular aqueduct' s development, and to investigate the clinical features. Methods: To carry out clinical investigation on 44 patients with the large vestibular aqueduct syndrome(LVAS), and to analysis the risk factors, the first occurrence age,the remote cause and the hearing loss conditions, etc. All of them have been proved to be large vestibular aqueduct syndromeby CT images.Results: 1. General information: There were male 30 cases and female 14cases. The male cases were more than the females by a ratio of 2. 1. The average age was 11.4years, range from 1 year to sixty years old.2.Pregnancy and born history: Eight cases would have familial genetic history, and having upper respiratory infection during Pregnancy (n=8), introuterine infection during pregnancy (n=1), premature infant(n=1), serious hypoxia(n=1), Light hypoxia(n=2), introuterine growth retardation(n=0), and uncertain reason(n=30).3. The first occurrence : 10 cases, weak hearing was found by hearing screening. 1 case was born with poor hearing, with no progression. The average ageof the first hearing loss is 8. 6 years old in other 33 cases, range from eleven months to fifty eight years old.4. The remote cause: The hearing loss after upper respiratory infection in 6 cases, originated from head trauma in 7 cases, and after taking medicine in 3 cases.5. Misdiagnosed before final diagnosis:traumic deafness(n=3), sudden deafness(n=10), deafness after taking medicine(n=13), sensorineral deafness(n=10), congenital deafness(n=12).6. 17 cases has weared hearing aids, 5 cases has implated Cochlear implantation , 24 cases have severe-ertreme severe hearing loss. Conclusion: 1.The large vestibular aqueduct syndrome is a congenital disease which can cause sensorineural hearing loss in recent years, being chatacterized by the larged vestibular aqueduct, and it has the incidence of family heredity.2. Taking medicine during pregnancy, intrauterine infection, premature infant and hypoxia are all risk factors that influente vestibular aqueduct's development.3. Some are misdiagnosed. Most of them are misdiagnosed as deafness after taking medicine,traumatic deafness orcongenital deafness.4. The large vestibular aqueduct syndrome is characterized by fluctuate and progressive hearing loss.5. Severe cases can be severe-extrme severe hearing loss. The cases presenting severe hearing loss could wear hearing aids. The cases presenting extreme hearing loss could implant cochlear implatation.