Clinical Analysis Of 17 Patients With Large Vestibular Aqueduct Syndrome

[Background]Enlarged vestibular aqueduct syndrome is a kind of inborn inner ear deformity,most patients present with progressive or fluctuating sensorineural hearing loss.Owing to it's pathogenesy is complex,earlier diagnosis and treat are short of effective methods.EVAS is a refractoriness disease in otology.In recent years,along with the development of acoustic tests and imaging diagnostic technology,more and more EVAS have been found.From the study of children's sensorineural hearing loss research,among children the rate attain to 12%.Owing to the effect of medicinal treatment is insignificant.The key of hearing preservation is diagnosis and prevention in early stage.[Objective]To analyze the clinical characteristics of 17 patients with Enlarge Vestibular Aqueduct Syndrome(EVAS) in plateau region and identify the prognostic factors.To explore strategies diagnosis and treatment of disease and provide a basis to take appropriate preventive measures for parents.[Methods]The clinical data of seventeen patients with EVAS including incentives, clinical manifestations,the records of serial hearing test(PTA,ABR and OAE) and high-resolution computer tomography or MRI of temporal bone were collected.Then individual database was established and analyzed retrospectively.Count information expressed in percentage,measurement data with mean±standard deviation ((?)+s).Using SPSS 10.0 software for the Chi-square test or the probability calculation.[Results]In 17 patients,there were male 9 cases and female 8 cases.Male and female proportion approaches 1 to 1.The mean age was 5.27±3.31 yrs.The total number of abnormal person account for 15 cases(88.24%) during pregnancy and delivering.11cases(64.71%) have head trauma;1 case(5.9%) has familial history;4 (23.53%) cases have ototoxic hearing loss of Aminoglycoside antibiotic.And there is a fluctuating hearing loss(64.71%),29.41%cases showed as the profound hearing loss.High resolution CT imaging showed that vestibular aqueducts were enlarged with widths MDVA 2.9±1.03 mm,ODVA 3.08±1.02 mm,while MRI revealed large vestibular aqueducts and endolymphatic sacs to expand in T2W in three cases.The first morbidity time have nothing to do with and deformity degree.26 ears(13 cases) of the 34 ears recorded the ASNR testing,the prevalence was 67.65%in EVAS group.[Conclusion]EVAS,a congenital disease,is characterized by fluctuating sensorineural hearing loss.During pregnancy to avoid infection is very important and it is useful for keeping the good hearing level to avoid head trauma.The occurrence of ASNR may indicate the diagnoses of enlarged vestibular aqueduct.There is maybe a few relationship between fluctuate of hearing and vestibular function,but the consult age and type of monstrosity is irrelative.The diagnosis of EVAS depends on the temporal bone CT scan or MRI.