Large Vestibular Aquduct Syndrome (LVAS), which is characterized by fluctuating sensorineural deafness and vertigo in young children, was officially named a congenital genetic disorder in 1978 and is associated with autosomal recessive inheritance and does not combine any other inner ear abnormalities except for enlargement of the vestibular aqueduct. Hearing loss occurs after a fall or a cold, and then slowly improves, but after a few drops, total hearing loss occurs, which means severe deafness. Zhang Daoxing, Department of Otolaryngology and Head and Neck Surgery, Xuanwu Hospital, Capital Medical University
Large vestibular aqueduct syndrome, early diagnosis and active prevention and treatment are important to prevent further hearing loss. If the hearing loss is fluctuating, CT of temporal bone can confirm the diagnosis, the main manifestations are (1) pure tone hearing loss within 70 dB, hearing brainstem evoked potential threshold within 9O dB; (2) further hearing loss triggered by cold, exertion, head trauma, mental stimulation, vertigo, tinnitus and other symptoms, but in a short period of time (3-5 d) can be recovered or reduced; (3) no clear (3) no clear family history, no mental retardation or other diseases involving the hearing syndrome; (4) the overall condition is slowly changing and the deafness is worsening.
For the treatment of large vestibular aqueduct syndrome: when hearing impairment is aggravated by obvious causes, active treatment with vasodilators, neurotrophic agents, dehydration and adrenal glucocorticoids can have some effect, and hearing can be restored to its original level. At the same time, hyperbaric oxygen therapy can increase the oxygen saturation and improve the microcirculation in the inner ear. For the surgical treatment of large vestibular aqueduct syndrome, some scholars have performed endolymphatic bursa decompression, shunt surgery or muscle flap filling, but the results are not satisfactory and there is a risk of total deafness. Hearing aids and cochlear implants have proven to be the only means of improving hearing in children with this condition. For children with severe hearing loss that cannot be effectively compensated by hearing aids, or those with progressive hearing loss, early cochlear implantation is the only effective option.