Experience of 76 cases of large vestibular aqueduct syndrome, clinical diagnosis and treatment / 临床耳鼻咽喉头颈外科杂志

ABSTRACT

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.