Enlarged Vestibular Aqueduct Syndrome is characterized by a widening of the vestibular aqueduct, associated with sensorineural hearing loss, or sometimes with mixed hearing loss, which may be congenital or acquired during childhood.The sensorineural hearing loss may be classified into mild, moderate and severe, associated with sudden periods of improvement or aggravation. Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH.We strongly recommend that you talk with a trusted . We studied 12 children,. Enlarged vestibular aqueduct syndrome is a clinical condition characterized for an autosomal recessive inheritance and a progressive perceptive deafness linked to a broadening of vestibular great . We report the clinical vestibular features and vestibular function testing of two . One significant cause of progressive hearing loss is the congenital enlargement of the cochlear aqueducts. Vestibular symptoms were present in only three (4%) of the patients. Enlarged vestibular aqueduct is the most common form of inner ear abnormality and can be associated with disequilibrium symptoms in a minority of patients (Valvassori, 1983; Jackler and de la Cruz, 1989; Levenson et al., 1989; Arcand et al., 1991; Belenky et al., 1993; Okumura et al., 1995). Associated phenotypes have been discussed and an attempt has been undertaken to correlate them with a corresponding genotype. The objective of this study is to describe the vestibular symptoms in pediatric patients with enlarged vestibular aqueduct (EVA) anomaly. Symptoms range from severe episodic vertigo to unsteadiness (usually in adults) to poor coordination and imbalance in children.The symptoms of vestibular disorders are notoriously difficult for adults to describe; for children, the task is even more challenging. While a strong correlative relationship between EVA and hearing loss is well established, its association with vestibular dysfunction is less well understood. Methods Retrospective chart review of pediatric and adult patients with EVA anomaly who were treated at the University of Utah Hospital or Primary Children's Medical Center, between 1995 and 2005. If a vestibular aqueduct is enlarged, the endolymphatic duct and sac usually grow large too. The prevalence of enlarged vestibular aqueduct syndrome has been estimated to range from 1-12% in population with sensorineural hearing loss. Enlarged vestibular aqueduct (EVA) is one of the most commonly identified malformations of the temporal bone (1). Seventy-seven patients were identified with an enlarged vestibular aqueduct with a male-to-female ratio of 1:1.5. Vestibular aqueduct enlargement, initially described by Valvassori and Clemis (1978), is the most common imaging abnormality in patients with congenital inner-ear defects ( Irving and Jackler, 1997 ). An enlarged vestibular aqueduct is usually accompanied by an enlargement of the endolymphatic duct and sac, which help maintain the volume and ionic composition of endolymph necessary for transmitting hearing and nerve signals to the brain. By collecting individual, detailed patient data and . MeSH terms Adolescent Adult Child Child, Preschool Female Hearing Loss, Sensorineural / etiology While this is uncommon, hearing loss can progress later in life especially after head trauma. Enlarged vestibular aqueduct - a congenital malformation of the inner ear that can be seen on imaging (CT or MRI) which can cause hearing loss. [35] found that 13/15 patients (86%) presented vestibular hypofunction or Enlarged Vestibular Aqueduct Syndrome (EVAS) Also known as Large Vestibular Aqueduct Syndrome, EVAS is a non-congenital, syndromic form of hearing loss caused by an enlarged vestibular aqueduct*, usually with a diameter larger than 1.5mm (Valvassori & Clemis, 1978). Hearing loss can be sensorineural, conductive, or both. University Hospitals Offers Comprehensive Research on Enlarged Vestibular Aqueduct Syndrome (EVA) Specialized physician-scientists at University Hospitals Rainbow Babies & Children's Hospital are changing the current research landscape on enlarged vestibular aqueduct syndrome (EVA) by charting a new pathway for clinical research discovery. 2 The description of LVA syndrome in 1978, which included associated inner ear anomalies, 3 led to an interest in the LVA as an . To evaluate the association between torticollis and enlarged vestibular aqueduct (EVA). The syndrome manifests as sensorineural or mixed hearing loss. The hearing loss is associated with temporal bone abnormalities, most commonly enlargement of the vestibular aqueduct, but it can also include the more severe Mondini dysplasia, a complex malformation in which the . 2 The description of LVA syndrome in 1978, which included associated inner ear anomalies, 3 led to an interest in the LVA as an . Enlarged vestibular aqueduct syndrome is a clinical condition characterized for an autosomal recessive inheritance and a progressive perceptive deafness linked to a broadening of vestibular great . Large vestibular aqueduct syndrome First described by Valvassori and Clemis in 1978 50 cases out of 3,700 tomograms, or 1.4% congenital hearing loss and vestibular symptoms enlarged vestibular aqueducts from 1.5- 8.0 mm 50% of the individuals with LVA were children and teenagers female to male ratio was 3:2 An enlarged vestibular aqueduct (EVA) is one of the most commonly identified inner ear bony malformations in children with sensorineural hearing loss of unknown cause. SeSAME Syndrome, Enlarged Vestibular Aqueduct, Digenic. Inclusion criteria were: (1) Enlarged vestibular aqueduct was diagnosed against the classic diagnostic criteria proposed by Valvassori and Clemis, i.e., the midpoint of the vestibular aqueduct >1.5 mm on axial CT images . Although it has been 32 years since Valvasorri and Clemis recognized the clinical importance of the enlarged vestibular aqueduct, many controversial aspects of the diagnosis remain. Enlargement of the vestibular aqueduct (EVA) is one of the most common inner ear malformations associated with sensorineural hearing loss in children. During normal embryogenesis phase the vestibular aqueduct will evolve from a diverticulum into a long and narrow canal. (2) Enlarged vestibular aqueduct patients manifested with the vestibular symptoms at presentation, which were ascertained . It has not, however, been defined as a distinct clinical entity in children. This condition is referred to as large vestibular aqueduct (LVA) syndrome, also known as enlarged or dilated vestibular aqueduct syndrome.
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