There is a type of childhood deafness that occurs slowly in life. The child may have normal hearing at birth, and later, as he or she grows older and under the influence of various external triggers, he or she may exhibit fluctuating or intermittent hearing, and a few patients may have symptoms such as dizziness and unsteady walking. These symptoms are often not taken seriously by parents, who assume that the cold is affecting their child’s ear function. Many patients only visit the hospital and undergo the appropriate tests to finally obtain a diagnosis after a sudden onset of severe hearing loss. This condition is the large vestibular aqueduct syndrome and is one of the common causes of deafness in children. The large vestibular aqueduct syndrome is a congenital malformation of the inner ear, the cause of which is unknown and may be related to genetic factors, intrauterine infections, etc. Although it is a congenital disorder, most sudden hearing impairments that occur early in children can be treated; the key is early detection. Symptoms: Hearing comes and goes. You should be alert for large vestibular aqueduct syndrome if: Most patients are born with normal hearing. Deafness occurs mostly in infancy and early childhood and manifests as progressive and fluctuating hearing loss, or progressive sensorineural deafness does not appear until the teenage years. As the lesion progresses, many patients develop sudden onset deafness, while others present with slow fluctuating declines of sensorineural deafness. Deafness is mostly bilateral and the hearing loss can range widely, from mild to very severe, and in severe cases, speech impairment. Older children or adults will complain of tinnitus. About 1/3 of patients have vestibular symptoms that manifest as episodes of vertigo and unsteadiness. Younger children present with a lack of good motor coordination. Parents should be reminded here that if a very small head trauma, cold, etc. causes significant hearing loss in children, it is important to seek prompt medical attention and have a doctor check for the presence of an enlarged vestibular aqueduct. Treatment: Early diagnosis, first medication Medication : Medication can be used in case of rapid hearing loss to restore hearing as much as possible and to strive for a better stage of hearing maintenance for the child for a longer period of time, which is very beneficial to the child’s language development. The majority of patients will recover their hearing with a comprehensive treatment plan, but some will have difficulty reaching their pre-onset hearing level. Hearing aid selection For patients who do not show signs of hearing improvement after 3 months of standardized medication, hearing aids can be selected as appropriate. If hearing aids still do not help to improve hearing, cochlear implants should be considered.