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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
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.