Large vestibular aqueduct syndrome and cochlear implantation

Patient: Hello, Dr. Yin, my son is 3 years old and suddenly lost the hearing in his right ear. He was later diagnosed with bilateral inner ear malformation. I want to ask Professor Yin, can this disease be cured? Yin Shankai, Department of Otolaryngology, Shanghai Sixth People’s Hospital: According to the findings of your child’s imaging report, hearing aids or electronic cochlear implants can be considered. You should pay attention to avoid head banging, strenuous exercise, infection, forceful nose blowing, etc., and prevent excessive emotion. For specific treatment, you can go to a specialized hospital and have a detailed physical examination, specialized examination and auxiliary examination. Patient: Dr. Yin, thank you so much. I don’t want to give my child a hearing aid, but if I can protect my left ear, I don’t need it. Yin Shankai, Department of Otolaryngology, Shanghai Sixth People’s Hospital: How is your child’s treatment going so far? Is there any improvement in the hearing of the right ear? Children with large vestibular aqueduct syndrome usually have recurrent episodes of hearing loss, and it is difficult to restore hearing to the pre-episode level after each episode. Often the child has a hearing aid or cochlear implant before complete hearing loss. Your child’s congenital malformation is bilateral and it is difficult to say what will happen in the future. What you can do now is to avoid head banging, strenuous exercise, infections, forceful nose blowing, etc. You should also prevent excessive emotional stress. People should also try to stay away from firecrackers and other things. As for the final choice of treatment, it is up to the parents to decide. Patient: Wearing hearing aids or implanting artificial electronic ears requires rehabilitation speech training, and since the child is still young, he will not cooperate well with certain things. I don’t know the effect of the treatment because I won’t be able to finish the injection until tomorrow and I haven’t gone for a review yet. Are there no cases of this syndrome that have been cured? Or cases where the attacks are only in one ear and not in the good ear? Yin Shankai, Department of Otolaryngology, Shanghai Sixth People’s Hospital: The main symptom of this disease is sensorineural deafness. The incidence is higher in women. Bilateral involvement is more common, and unilateral onset is about 6%-40%. Deafness can start at any time from birth to adolescence, with sudden or insidious onset and progressive or fluctuating hearing loss. It can be severe to profound deafness at the time of first diagnosis, while mild to moderate hearing loss is less common. Inner ear malformations in children are the result of embryonic underdevelopment and are present at birth, bilaterally. This means that both sides can develop. Early childhood is an important stage in a child’s speech development and parents should pay attention to it. Parents should pay attention to this. Consult your local hospital for specific treatment options.