The Large Vestibular Aqueduct Syndrome in Children: A Review of 12 Cases and the Description of a New Clinical Entity

Enlarged Vestibular Aqueduct in Congenital Non-Syndromic Sensorineural Hearing Loss in Egypt

“…There was no significant difference between VA operculum or midpoint and the PTA configuration in the three groups. Similar findings were reported in the literature [1,8,12,19,22,29].…”

“…The degree of SNHL in patients with EVA has been described by many authors to range from mild to profound and even patients with EVA without any hearing impairment has also been reported [19,20,[28][29][30][31]. In our patients with EVA: 4 ears had moderate SNHL and 2 ears had profound SNHL.…”

“…Several authors [2,3,19,22,29] suggested the potential causes of SNHL in patients with isolated EVA including hydro-electrolyte imbalance of inner ear fluids resulting in accumulation of excess endolymph. The resulting volume or pressure effect participates in an enlarged endolymphatic duct and sac.…”

Comparison of 45° Oblique Reformats with Axial Reformats in CT Evaluation of the Vestibular Aqueduct

“…Subsequently, the clinical entity associated with isolated enlargement of the vestibular aqueduct was described. [13][14][15] Patients demonstrated progressive hearing loss in childhood and were at risk of sudden hearing loss with mild trauma. The identification of a distinct clinical syndrome associated with the anatomic finding of an enlarged vestibular aqueduct elevated the importance of detecting this abnormality on imaging.…”

The Large Vestibular Aqueduct Syndrome as a Cause for Sudden Deafness in Children

“…Disturbances in this stage of embryological development could lead to the persistence of the early form of the vestibular aqueduct. Levenson et al [2] found twice as many cases of isolated large vestibular aqueducts as MondiniAlexander dysplasia, and Mafee et al [3] concluded that the LVAS is the most common deformity of the inner ear.…”