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Large vestibular aqueduct syndrome is one of the common non-syndromic hearing impairment. It is one of the most common inner ear abnormalities that cause hearing loss in children.The main performance is gradual or fluctuant hearing loss, from basic normal to extremely severe. Frequently seen in high frequencies hearing loss. The air-bone conduction gaps present in pure tone audiometry test with low frequencies. There were some inducements of intracranial pressure increases before premorbid. Some patients could be accompanied by vertigo or instability. So far, there was still no effective way to terminate the patient deafness progress.If there was no effective intervention,the speech developmental delay of children were an inevitable trend,greatly affect their normal social communication learning ability. So, early diagnosis was critical. Imaging examination was the golden criterion for the diagnosis of LAVS.Characteristic audiological performance and gene diagnosis can be the basis of the further diagnosed. Because the structure and anatomical location of vestibular aqueduct is small and deep, normal imaging examination is difficult to display its morphology and structure,so,for a long time, it did not work very well. Until the advent of High-resolution computed tomography and magnetic resonance imaging, there was a breakthrough and a deeper understanding of the fine structure with inner ear. We reviewed the latest progress of large vestibular aqueduct syndrome imaging studies., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Objective: To investigate the clinical and diagnostic characteristics of audiometric findings and vestibular-evoked myogenic potentials in patients with large vestibular aqueduct syndrome (LVAS)., Method: Thirty LVAS subjects (60 ears) recruited received pure tone audiometry, acoustic immittance, auditory brain stem responses (ABRs), distortion-product otoacoustic emission (DPOAE), Vestibular evoked myogenic potentials (VEMP) and caloric test, and the diagnostic significance of the results was analyzed., Result: All 30 cases (60 ears) showed progressive and fluctuating hearing loss, while 16 cases experienced dizziness when hearing fluctuated. Most of our cases showed sensorineural hearing loss, and 47 ears (94.0%) showed air-bone gap in the low frequencies, with mean gaps of (43 +/- 17) dB HL at 250 Hz, (33 +/- 18) dB HL at 500 Hz, in which the middle ear function showed normal. The acoustically evoked short latency negative response (ASNR) with medium latency (3.06 +/- 0.52) ms was elicited from 18 ears (64.3%). The mean amplitude of vestibular evoked myogenic potentials (VEMP) of 42 ears was (147.10 +/- 107.55) microv, and the threshold of VEMP of 19 ears was 75 dB nHL, of 7 ears was 65 dB nHL., Conclusion: Characteristics of hearing performance, such as progressive and fluctuating hearing loss, air-bone gap at the low frequencies with normal middle ears, the ASNR, and increased amplitude and decreased threshold of the VEMPs, will help clinicians make initial diagnosis of LVAS, and provide a reference for further imaging examination.

Objective: To explore the disease incidence, clinical symptoms, prevention and treatment measures of the large vestibular aqueduct syndrome (LVAS)., Method: Retrospective analyse the medical history, hearing, vestibular function examination and treatment of 76 LVAS patients who were diagnosed in our department of Otolaryngology from 2002 to 2008., Result: Most patients (93.4%) showed sensorineural hearing loss. Part of patients (61.8%) showed air-bone conduction gap in low frequency. The hearing loss of 43 ears is > 40-60 dB HL, > 60 -80 dB HL 47 ears, > 80 dB HL 62 ears. Decline curve is the characteristic of the Audiogram. The decline in high-frequency 112 ears, flat curve in 29 ears, island hearing in 11 ears. Forty-six patients were conducted the vestibular function examination, which showed low vestibular function. Tympanogram showed that 141 ears are type A, 11 ears are type C. High-resolution CT scan revealed that vestibular aqueduct minimum diameter is 2.2 mm and the largest is 6.2 mm, with a wide opening and deep narrower, and showed the "triangle" or "flared". Forty-two cases of this group were simple dilatation of the vestibular aqueduct, and no large vestibular semicircular canal malformation or cochlear malformation. There was no intellectual and other development disorders. In accordance with the degree of hearing loss, 20 cases of patients restored hearing after drug treatment. Eleven were cases fit a suitable hearing aid and carried out the language rehabilitation training. Forty-five very severe patients were implanted the cochlear and mapping one month later., Conclusion: Fluctuative and progressive hearing loss is the main clinical symptoms of large vestibular aqueduct syndrome. The patients should be examined by high resolution CT scan of the temporal bone. There is no precise and effective treatment for the disease. It is very important for the deaf children who have residual hearing to fit hearing aids and carry out the language rehabilitation training as soon as possible. As for the patients who suffer from hearing loss severely and the hearing aid cannot achieve effective compensation, the cochlear implant should be considered.