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A Journal on Otorhinolaryngology, Head and Neck Surgery,
Plastic Reconstructive Surgery, Otoneurosurgery

Indexed/Abstracted in: EMBASE, Scopus




Otorinolaringologia 2003 December;53(4):151-4

language: Italian

The large vestibular aqueduct syndrome

Sammartano D., Ferrara S., Saraniti C., Modica A., Cernigliaro G., Ferrara P.

Clinica Otorinolaringoiatrica «R» Università degli Studi di Palermo, Palermo


The ­large ves­tib­ular aque­duct (LVA) syn­drome is char­ac­ter­ized by ­stable and/or fluc­tu­ating sen­sor­i­neural ­hearing ­loss, ­shown by ­tonal audiom­etry, ­that ­begins in the ­first ­years of ­life, is pro­gres­sive and ­worsens fol­lowing ­traumas and is asso­ciated to the ves­tib­ular aque­duct enlarge­ment ver­i­fied by CT and/or MR. The ­hearing ­loss can be the ­only clin­ical ­sign of LVA or it can be asso­ciated to ver­tigo, ­feeling of ­instability, tin­nitus, head­ache. The ­most prob­able ­causes of the ­hearing ­loss are dis­cussed. ­This ­study ­refers to 2 typ­ical clin­ical ­cases for audio­metric exam­ina­tion, ­imaging and clin­ical symp­tom­a­tology. The ­onset and the ­course of the LVA syn­drome are ­slow and pro­gres­sive. The ­authors sug­gest ­that its path­o­gen­esis is cor­re­lated to an evo­lu­tionary ­defect of the ­cochleo-ves­tib­ular func­tion, due to an endo­lym­phatic ­liquid ­reflux, ­from the endo­lym­phatic sac ­toward the ­inner ear, ­allowed by the con­sid­er­able dimen­sions of the ves­tib­ular aque­duct. The ­authors con­sider CT and MR elec­tive diag­nostic inves­ti­ga­tions in all ­those ­patients, par­tic­u­larly chil­dren, ­with not jus­ti­fied ­mono or bilat­eral sen­sor­i­neural ­hearing ­loss, spe­cially ­when it is fluc­tu­ating, ­because it can be asso­ciated to LVA. The treat­ment is symp­to­matic, the ­only pre­ven­tion is to ­avoid the trau­matic ­events ­that can deter­mine a fur­ther ­hearing ­loss.

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