"Enlarged vestibular aqueduct"

Auditory Characteristics in Children With Enlarged Vestibular Aqueduct. Enlarged vestibular aqueduct (EVA) is the most common radiographic finding in children diagnosed with congenital sensorineural hearing loss (SNHL). Many institutions use the Cincinnati criteria for diagnosis: width ≥2.0 mm at the operculum and/or ≥1.0 mm at the midpoint. Our goals are to expand our understanding of EVA

Hearing loss trajectory and prediction model for children with enlarged vestibular aqueduct. To explore how hearing changes over time and the characteristics associated with progressive hearing loss in children with enlarged vestibular aqueduct (EVA), and develop a prediction model for anticipation of hearing progression probability. A retrospective analysis was conducted on 48 children (92 ears

Clinical Phenotypic Characterization of the SLC26A4 Mutation in Pendred Syndrome/Nonsyndromic Enlarged Vestibular Aqueduct. To summarize the Solute Carrier Family 26 Member 4 (SLC26A4) mutations and clinical phenotypic characteristics of patients with Pendred syndrome/nonsyndromic enlarged vestibular aqueduct (PS/NSEVA). A retrospective cohort study for the Chinese population was performed

Newborn Hearing Screening Results in Patients with Enlarged Vestibular Aqueduct. Enlarged vestibular aqueduct (EVA) is the most common anatomic abnormality contributing to permanent hearing loss (HL) in children. Although the association between EVA and HL is well-documented, the pass rate for the newborn hearing screening (NBHS) for patients with EVA-related HL is not. Our objective

Evidence of vestibular dysfunction in children with enlarged vestibular aqueduct. To investigate the occurrence and characteristics of balance and vestibular deficits in pediatric patients with enlarged vestibular aqueduct (EVA). Retrospective review of 53 children with EVA who underwent a comprehensive vestibular evaluation in our pediatric balance and vestibular program. Laboratory testing

Programming Levels and Speech Perception in Pediatric Cochlear Implant Recipients With Enlarged Vestibular Aqueduct or GJB2 Mutation. To determine the relationship between hearing loss etiology, cochlear implant (CI) programming levels, and speech perception performance in a large clinical cohort of pediatric CI recipients. Retrospective chart review. Tertiary care hospitals. A total of 136 pediatric CI recipients (218 ears) were included in this study. All patients had diagnoses of either enlarged vestibular aqueduct (EVA) or GJB2 (Connexin-26) mutation confirmed via radiographic data and/or genetic reports. All patients received audiologic care at either Boston Children's Hospital or Massachusetts Eye and Ear in Boston, MA, between the years 1999 and 2020. Electrode impedances

Assessing the clinical utility of volumetric HRCT in pediatric enlarged vestibular aqueduct related hearing loss. |The aim of this study was to evaluate the use of volumetric HRCT measurements in the diagnosis of enlarged vestibular aqueduct syndrome (EVAS) and describe the association of this novel radiographic approach with clinical hearing outcomes. We hypothesized that volumetric

Clinical and genetic analysis of children with hearing loss and bilateral enlarged vestibular aqueducts. To evaluate the clinical and genetic features of children with hearing loss associated with one of the most common malformations of the inner ear: bilateral enlargement of vestibular aqueducts (EVA). Clinical and genetic features were investigated in 28 children with hearing loss diagnosed

Hearing loss patterns in enlarged vestibular aqueduct syndrome: Do fluctuations have clinical significance? To reveal hearing loss patterns in patients with enlarged vestibular aqueduct (EVA) syndrome according to demographic and clinical characteristics. A retrospective, longitudinal study design was utilized to identify patients with EVA. Ears of patients were categorized into one of four

Predictive Modeling and Risk Stratification of Patients With Enlarged Vestibular Aqueduct. To investigate patient-specific characteristics that independently predict for progressive hearing loss in patients with enlarged vestibular aqueduct (EVA). Utilize multivariable predictive models to identify subgroups of patients with significantly different progression risks. Retrospective analysis

Early Elevation and Normalization of Electrode Impedance in Patients With Enlarged Vestibular Aqueduct Undergoing Cochlear Implantation. To characterize early changes in impedance in patients undergoing cochlear implantation with and without enlarged vestibular aqueducts (EVA). Case-control retrospective study of patients undergoing cochlear implantation with and without EVA. Impedance

Enlarged Vestibular Aqueduct: Disease Characterization and Exploration of Potential Prognostic Factors for Cochlear Implantation. There is an unmet need to match the anticipated natural history of hearing loss (HL) in enlarged vestibular aqueduct (EVA) with clinical management strategies. The objectives of this study are therefore to provide a detailed case characterization of an EVA cohort

Enlarged vestibular aqueduct: Intraoperative electrocochleography findings during cochlear implantation. Enlarged vestibular aqueduct (EVA) is the most frequent inner ear abnormality found on computed tomography in children with sensorineural hearing loss. The effects EVA abnormalities have on electrocochleography (ECochG) are unknown. Positive deflections in summation potential evoked by tone

Correlation of air-bone gap and size of Enlarged Vestibular Aqueduct in children. Enlarged vestibular aqueduct (EVA) is an inner ear malformation that represents an important cause of pediatric hearing loss. While certain elements in the history or audiogram may suggest EVA, it is most often diagnosed using computed tomography (CT). The present investigation was conducted to determine

Torticollis in children with enlarged vestibular aqueducts. To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). An online/phone survey was administered to parents of 133 children diagnosed with the following disorders: EVA, GJB2 (Connexin 26) mutations associated congenital hearing loss and epistaxis (control). The survey included questions regarding symptoms

Audiometric findings in children with unilateral enlarged vestibular aqueduct. To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution. A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine

Sensorineural Hearing Loss in the Nonimplanted Ear Following Cochlear Implantation in a Patient With Bilateral Enlarged Vestibular Aqueducts. To document the case of a patient with bilateral enlarged vestibular aqueducts who experienced sensorineural hearing loss in the nonimplanted ear following unilateral cochlear implantation complicated by perilymph gusher requiring lumbar drain insertion and to highlight the need to counsel regarding the risk of potential hearing loss to the contralateral ear when preparing for cochlear implants in the setting of inner ear malformations. One patient with bilateral enlarged vestibular aqueducts in a tertiary referral center. Cochlear implantation complicated by perilymph gusher requiring lumbar drain insertion. Bone conduction hearing thresholds, word recognition

The Natural History of Hearing Loss in Pendred Syndrome and Non-Syndromic Enlarged Vestibular Aqueduct. The aim was to investigate the progress of hearing loss over time in a cohort of pendred syndrome and non-syndromic enlarged vestibular aqueduct (PS/NSEVA) with one or two confirmed pathogenic variations in SLC26A4. Retrospective cohort study. At our tertiary referral center, a retrospective search of all patients with enlarged vestibular aqueduct, hearing loss and SLC26A4 mutations yielded 103 individuals by March 2017, 96 of whom had records of hearing levels; both an early audiometry and the latest between 3 and 668 months follow-up. Pure-tone average (PTA; average of thresholds at 0.5, 1, 2 and 4 kHz) was calculated for both ears at time 1 and time 2. Neonatal screening results were

Sex-specific enlarged vestibular aqueduct morphology and audiometry. Enlargement of the vestibular aqueduct (EVA) is one of the most common congenital malformations in pediatric patients presenting with sensorineural or mixed hearing loss. The relationship between vestibular aqueduct (VA) morphology and hearing loss across sex is not well characterized. This study assesses VA morphology

Vestibular Manifestations in Subjects With Enlarged Vestibular Aqueduct. To describe the results of a thorough evaluation in a large series of patients with an enlarged vestibular aqueduct (EVA), focusing on vestibular manifestations with etiological considerations. Retrospective chart review of patients with EVA. Tertiary referral center. A total of 22 EVA patients with a median age of 8 years that patients may develop vestibular symptoms during their clinical course, and all patients with an enlarged vestibular aqueduct should be cautioned regarding the potential development of vestibular pathology. Moreover, the non-negligible incidence of secondary BPPV mandates positional tests when evaluating EVA patients with vertigo.