Chronological Changes of Hearing in Pediatric Patients with Large Vestibular Aqueduct Syndrome

Abstract

LVAS, a congenital disease, is characterized by fluctuating sensorineural HL. Most hearing at PTA remained stable at least in one ear chronologically. The standard deviation of hearing at 500 Hz was the only prognostic factor for the progression of PTA. An enlarged vestibular aqueduct affects fluctuations of hearing, but the pathogenesis of HL still remains unclear and deserves further investigations.

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

“…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.…”

“…Audiological Features of the SNHL SNHL in patients with EVA has been reported to be fluctuating, stable or progressive [4,6] following trigger activities such as minor head trauma, jogging, or common colds [2,7,19,24,27,28]. Because of lack of serial audiometric data in our patients, we could not examine the progression of SNHL.…”

Analysis of the SLC26A4 gene in patients with Pendred syndrome in Taiwan

“…These patients were suspected to have PS on the basis of the classic findings of sensorineural deafness and EVAs. Most of these patients have been previously described as having otologic features of PS [21].…”

“…The petrosal orifice diameter of the vestibular aqueduct was also measured using the same method. Enlarged vestibular aqueduct was diagnosed when the MDVA was more than 1.4 mm [21].…”

Implante coclear e síndrome do aqueduto vestibular alargado em crianças

“…More recent studies have mapped the hearing loss associated with the enlarged vestibular aqueduct in the region of chromosome 7q31, however, its clinical meaning still requires more investigation. 1,12,15 Children with EVAS may have moderate to severe hearing deficiencies during their early stages of childhood, however their residual hearing allows them to develop oral language with conventional hearing aids and may be completely integrated to regular school conditions. Nonetheless, these children have a worsening in their hearing skills with time and cochlear implants are being offered as an option to keep their hearing and oral communication skills in proper levels 4 .…”