A large vestibular aqueduct, an isolated anomaly of temporal bone, is relatively rare and its association with sensorinerual hearing loss in childhood is known as the enlarged vestibular aqueduct syndrome. Using MRI, we found enlarged endolymphatic ducts and sacs in four ears of our two patients. Signal intensity of enlarged endolymphatic sac that is detected higher than that of CSF in Fluid Attenuated Inversion Recovery (FLAIR) image may indicate poor prognisis with respect to bone conduction threshold in pure tone audiogram.
Bone Conduction ; Ear ; Endolymphatic Duct ; Endolymphatic Sac ; Hearing Loss ; Humans ; Magnetic Resonance Imaging ; Temporal Bone ; Vestibular Aqueduct*

The enlarged vestibular aqueduct syndrome (EVAS) is a clinical disease causing anatomical abnormality of bony canal in the temporal bone containing endolymphatic duct and sac. It is also associated with progressive sensorineural hearing loss with an isolated enlarged vestibular aqueduct. Familial inheritance of enlarged vestibular aqueduct syndrome (FEVAS) is rare and the correct mode of inheritance is not yet discovered. We studied familial inheritance in EVAS by performing clinical, audiological, radiographic and chromosomal analyses, and found strong indications that FEVAS may be an autosomal recessive trait. Further study would be focused on genetic evaluation of FEVAS.
Endolymphatic Duct ; Extravehicular Activity ; Hearing Loss, Sensorineural ; Temporal Bone ; Vestibular Aqueduct* ; Wills

Unilateral enlargement of the vestibular aqueduct (EVA)is a relatively rare disease. Bilateral EVA was found to be more common than unilateral EVA. There are significant differences in clinical features and molecular etiology between unilateral EVA and bilateral one. This article reviewed related researches of the unilateral EVA in clinical characteristics, molecular etiology and pathogenic mechanism.
Vestibular Aqueduct ; pathology

Humans ; Syndrome ; Vestibular Aqueduct ; Vestibular Diseases

BACKGROUND AND OBJECTIVES: AThe anatomical aspects of the multiple etiopathogenic factors in Meniere's disease such as narrowed vestibular aqueduct, hypoplasia of the perilabyrinthine portion, hypoplastic vestibular aqueduct, anteromedially displaced sigmoid sinus have been studied by other authors. The purpose of this study is to investigate and measure the temporal bone including the retrolabyrinthine portion, and evaluate hypoplasia of the retrolabyrinthine region, anteromedial displacement of sigmoid sinus in the affected ear of unilateral Meniere's disease. MATERIALS AND METHODS: The four parameters in both sides of temporal bone were measure in 30 patients with unilateral Meniere's disease and in the controls using the axial 3DFT CISS image of temporal MRI. RESULTS: The retrolabyrinthine bone development, which contains the vestibular aqueduct and endolymphatic sac, was significantly poorer in the affected ear with unilateral Meniere's disease. However, the anteromedial displacement of sigmoid sinus were not found in the affected ear with Meniere's disease. CONCLUSION: These results suggest that hypoplasia of the retrolabyrinthine region may have a role in the pathogenesis of Meniere's disease.
Bone Development ; Colon, Sigmoid ; Ear ; Endolymphatic Sac ; Humans ; Magnetic Resonance Imaging ; Meniere Disease* ; Temporal Bone* ; Vestibular Aqueduct

Child, Preschool ; Humans ; Vestibular Aqueduct ; physiopathology ; Vestibular Diseases ; diagnosis

Many congenital dysplasias of the osseous labyrinth have been identified, and the differential diagnosis of these dysplasias is essential for delivering proper patient management. We retrospectively reviewed the computed tomography (CT) and magnetic resonance (MR) imaging findings of 20 children who had congenital sensorineural hearing loss. The children included cases of enlarged vestibular aqueduct and endolymphatic sac (n=8), aplasia of the semicircular canal (n=4), lateral semicircular canal-vestibule dysplasia (n=3), common cavity malformations with a large vestibule (n=1), cochlear hypoplasia (n=1), Mondini's dysplasia with large vestibular aqueduct (n=1), Mondini's dysplasia with a large vestibule (n=1), and small internal auditory canal (n=1). Six cases were unilateral. Nine cases had combined deformities, and nine cases had cochlear implants. CT was performed with a 1.0-mm thickness in the direct coronal and axial sections with using bone algorithms. MR was performed with a temporal 3D T2 FSE 10-mm scan and with routine brain images. We describe here the imaging features for the anomalies of the inner ear in patients suffering from congenital sensorineural hearing loss.
Brain ; Child ; Cochlear Implants ; Congenital Abnormalities ; Diagnosis, Differential ; Ear, Inner* ; Endolymphatic Sac ; Hearing Loss, Sensorineural* ; Humans ; Magnetic Resonance Imaging* ; Retrospective Studies ; Semicircular Canals ; Vestibular Aqueduct

Large vestibular aqueduct syndrome (LVAS) is one of common non-syndromic hearing disorders. With the rapid development of medical imaging, audiology, molecular biology, genetics, cochlear implant surgery, we have made remarkable achievements in the diagnosis and treatment of large vestibular aqueduct syndrome. This article reviewed related researches of the large vestibular aqueduct syndrome.
Cochlear Implants ; Hearing Disorders ; genetics ; Humans ; Vestibular Aqueduct ; abnormalities ; Vestibular Diseases ; genetics

Hearing Loss, Sensorineural/diagnosis* ; Humans ; Syndrome ; Vestibular Aqueduct ; Vestibular Diseases/diagnosis*

OBJECTIVE: The purposes of this study was to analyse the significant of low frequency air-bone gap in enlarged vestibular aqueduct (EVA) patients according to the pure tone audiogram and the benefit for clinic diagnosis of the EVA. METHOD: The audiology testing include (1) play audiometry or pure tone audiometry, tympanometry, middle ear muscle reflex thresholds and stapedius muscle reflex; (2) High-resolution computed tomography (CT) scan of the temporal bone and magnetic resonance imaging was used for diagnoses the EVA. RESULT: All the 78 patients (154 ears) were diagnosed as the typical EVA by CT or MRI. Inner ear malformations were found in 3 ears otherwise the structures of middle ear in all the patients were absolutely normal. The audiology analysis showed 154 ears were type A tympanogram including 126 ears with typical A, 25 ears with As, 3 ears with Ad type. In the 250 Hz pure-tone test:A-B gap were observed in 126 ears(126/154,81. 8%) with the different hearing loss degree: 1 ear mild, 11 ears moderate, 19 ears moderate severe, 40 ears severe and 53 ears profound. In the 500 Hz pure-tone test: A-B gap were found in 102 ears(102/154, 66. 2%) with the different hearing loss degree: 9. ears moderate, 17 ears moderate severe, 35 ears severe and 41 ears profound. CONCLUSION Our study suggested a 66. 2% -81. 8% possibilities to find the EVA through the pure-tone audiometry firstly in the basis of the normal tympanograms with the significant A-B gap.
Acoustic Impedance Tests ; Adolescent ; Audiometry, Pure-Tone ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Vestibular Aqueduct ; Vestibular Diseases ; physiopathology