What is Great Vestibular Aqueduct? What are the symptoms?

Great vestibular aqueduct syndrome, also known as congenital vestibular aqueduct enlargement, is an autosomal recessive disorder that occurs in a single individual. In addition to the enlargement of the vestibular aqueduct, there are no other inner ear malformations.

Patients generally develop at the age of 2 or so, colds and trauma are often the triggers of their onset, mainly manifested as a decline in hearing fluctuations, that is, hearing loss will slowly recover after hearing loss, and then it will fall again, after several declines, the hearing will completely disappear, forming extremely severe deafness. A small number of patients present with sudden deafness, while others may have episodic vertigo with fluctuating hearing loss.

There is no effective method for this disease, hearing aids and cochlear implants are the only means to improve hearing in children, and those with residual hearing can wear hearing aids, and the hearing loss is extremely severe, and the hearing aids cannot be effectively compensated, or progressive hearing loss should be considered for cochlear implants.

Great vestibular aqueduct is a malformation of the inner ear and is a congenital disease that belongs to genetic defects. Now research has determined that it is a genetic disease. The main manifestations are fluctuating ** tone neural deafness and vertigo in young children.

A CT scan of the temporal bone can confirm the diagnosis, confirm the condition early, and take preventive measures.

There is currently no effective ** method. In the early years, some scholars advocated endolymphatic sac decompression, which has been proven to be ineffective for this disease. Hearing aids can be worn for patients with residual hearing, and cochlear implants may be used for those who are very deaf.

Great vestibular aqueduct syndrome, a congenital malformation of inner ear development, is related to autosomal recessive inheritance, and there is currently no effective method.

Great vestibular aqueduct syndrome mainly begins at the age of 2 years, and the main causes of the onset are generally colds and trauma, mainly manifested by gradual hearing loss, some patients will show sudden deafness, and some patients will have episodic vertigo with hearing loss. Once the patient has hearing loss, he should seek medical attention in time and wear hearing aids to reduce early hearing loss as much as possible; If the results are still not good after wearing hearing aids, or if the patient has developed total deafness, surgery for cochlear implantation should be done as soon as possible.

After surgical cochlear implantation, patients should pay attention to protecting their heads and avoiding impacts; Strengthen ear care, keep the area clean and dry, and avoid infection; Try to avoid inspections and ** involving electromagnetic aspects. At the same time, after the operation, it is necessary to carry out auditory and speech training in time.

Large vestibular aquduct syndrome (LVAS) is a deafness disease discovered with the advent of CT in the late 70s, which is a congenital inner ear malformation hearing impairment with progressive and fluctuating hearing loss. The main manifestations are fluctuating ** tone neural deafness and vertigo in young children. In 1978, it was officially named a congenital genetic disorder, which is related to autosomal recessive inheritance, except for vestibular aqueduct enlargement, and does not have other inner ear malformations.

The onset of illness is usually around 2 years of age, and colds and trauma are often triggers, and even minor trauma can cause severe sensorineural hearing loss and vertigo.

There is no definite and effective method for large vestibular aqueduct syndrome, but early diagnosis and active prevention and treatment have certain significance to prevent further hearing loss. For large vestibular aqueduct syndrome, when the hearing impairment is aggravated by obvious triggers, the use of relevant drugs can have a certain effect, and the hearing can be restored to the original level. Generally, people with residual hearing can wear hearing aids, but cochlear implants can be considered for those with severe hearing loss, hearing aids that cannot be effectively compensated, or progressive hearing loss to severe deafness.

A congenital malformation of the inner ear, which is a hereditary neurological hearing loss, often with fluctuating loss. Usually be sure to pay attention to:

1. Check your hearing regularly, develop good listening follow-up habits, and seek medical attention in time if you have hearing problems;

2. Avoid head trauma, even if it is a minor collision;

3. It is not suitable to participate in strenuous sports, such as roller coasters, bungee jumping, etc.;

4. Do not use ototoxic drugs;

5. Avoid exposure to strong noise;

6. Prevent colds, pay attention to children's emotional control, and reduce stressful actions such as nose blowing.

1.What is Great Vestibular Aqueduct Syndrome (LVAS)?

It is a congenital malformation of the inner ear, mainly due to the enlargement of the vestibular aqueduct, so it is called the large vestibular aqueduct syndrome, and the cochlear hair cells will be destroyed during the onset, so it is often accompanied by symptoms such as sensorineural hearing loss.

2.Is Great Vestibular Aqueduct Syndrome a congenital condition? When does it occur?

LVAS is a congenital disease, and deafness can occur suddenly at any time from birth to adolescence, and most often occurs in childhood.

3.What are the clinical manifestations of large vestibular aqueduct syndrome?

The clinical manifestations of large vestibular aqueduct syndrome are bilateral asymmetric hearing loss, which is characterized by progressive and fluctuating hearing loss.

4.What causes great vestibular aqueduct syndrome?

Hearing loss may be induced by minor head trauma or sudden changes in ambient pressure, such as riding in an aircraft, diving, playing musical instruments vigorously, holding air (e.g., lifting weights, defecating), straining nose, etc. Sometimes colds are also related.

5.How is great vestibular aqueduct syndrome diagnosed?

In addition to history, clinical symptom identification, and audiological examination, the diagnosis of large vestibular aqueduct syndrome is mainly based on high-resolution CT and MRI scans of the temporal bone, and the diagnosis is confirmed by the presence of an enlarged vestibular aqueduct. Because high-resolution CT of the temporal bone is generally not recommended for infants younger than 6 months of age, the diagnosis is usually made after 6 months of age.

6.How can I prevent hearing loss after a diagnosis of large vestibular aqueduct syndrome?

Once the diagnosis is confirmed, patients and their parents should be aware of the need to do everything possible to prevent sudden hearing loss in the affected ear, avoid head trauma, and avoid head collisions; It is not advisable to participate in competitive sports, or to play musical instruments, lift weights, dive, blow your nose vigorously, etc., and should prevent excessive emotional excitement.

7.What happens after the onset of Great Vestibular Aqueduct Syndrome?

The acute onset period can be **, as long as it is detected in time, **, usually hearing can be partially restored, or even restored to the pre-onset level.

8.How do I monitor my child's hearing?

Every day, the child is given a command (e.g., to find a toy) through the voice, and the child is trained to respond to the command when he hears the command. In this way, once a day in the morning and once in the evening, if there is an abnormality, you can go to the hospital in time to recheck your hearing.

9.When to choose to use hearing aids? When should I consider a cochlear implant?

It is necessary to prescribe hearing aids as soon as possible, let the child obtain speech as soon as possible, evaluate the effect of hearing aid use, and consider cochlear implantation once the hearing aid is ineffective or ineffective.

10.Can I consider cochlear implantation without hearing aids?

Children generally have good residual hearing in the early years, so hearing aids should be prescribed as early as possible and auditory training should be carried out to monitor the child's hearing condition as soon as possible. When there is a fluctuating change in hearing, it is necessary to be active first**, during the period, it is recommended to use hearing aids, but the gain is still the gain value before the onset of the disease. When the condition is stable, the hearing will need to be rechecked to obtain a definite hearing result, and the hearing aid will be re-adjusted according to the new hearing result.

And adjust the output power of the hearing aid in time.

There is a tendency to familial inheritance, most of which are autosomal recessive inheritance, which may be caused by the inhibition and aberration of the development of vestibular labyrinth in the embryonic period (mainly in the first 3 months), and the specific onset is more complex, and it can be caused by factors such as genetics, infection, environment, and psychological disorders of pregnant women, and it is also believed that postpartum and early childhood developmental disorders occur.

There is a tendency to be inherited in families, and most of the BAI numbers are autosomal DU recessive inheritance, which may be caused by the development of vestibular labyrinth in the embryonic stage (mainly the first 3 months) and aberrations.

The specific onset of the disease is more complex, and can be caused by factors such as genetics, infection, environment, and psychological disorders in pregnant women, and it is also thought to be caused by postpartum and early childhood developmental disorders.

Hello, Great vestibular aqueduct syndrome is a hearing disorder in which vestibular enlargement is accompanied by a congenital inner ear malformation with sensorineural hearing loss, which usually occurs in children. Clinical manifestations: hearing loss, sudden deafness, episodic vertigo and instability in a few patients.

Great vestibular aqueduct syndrome is a congenital genetic disorder that is generally thought to be autosomal recessive. Except for vestibular aqueduct enlargement, there are no other inner ear malformations. When hearing loss is aggravated by obvious triggers, the use of vasodilators, neurotrophic agents, adrenal glucocorticoids and other drugs can have a certain effect.

If hearing is not restored, it is recommended that cochlear implants be considered as soon as possible for children with severe hearing loss.

Hello, the large vestibular aqueduct is a disease due to congenital dysplasia, resulting in vestibular aqueduct enlargement, simple vestibular aqueduct enlargement deformity is more common, but it can also be combined with cochlear or vestibular malformation, endolymph through the enlarged vestibular aqueduct, from the endolymphatic sac to the cochlea or vestibular damage hair cells to cause symptoms of hearing loss and vertigo, it is one of the important causes of hearing loss in children. Although he is a congenital developmental disorder, but the hearing damage it causes mostly occurs after birth, due to slight head trauma physical changes and infectious factors, can cause obvious hearing damage in children, therefore, preventive measures are very important, tell parents in advance, children's hearing may change suddenly because of certain factors, parents can take preventive measures in advance, while paying attention to monitoring children's hearing, and cooperate with doctors to avoid further hearing damage. If hearing loss occurs, hearing aids or cochlear implants may eventually be recommended to enhance speech training and improve effectiveness.