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The patient Zhu, 4 years old, had bright blue irises in both eyes at birth, and was later found to suffer from bilateral sensorineural hearing loss. At present, a small amount of freckles on the face can be observed.He was diagnosed with Waardenburg syndrome and was treated with a cochlear implant in the hospital.At present, the effect of hearing and speech rehabilitation is good, and he goes to normal kindergarten.

The main reasons for children’s deafness are as follows:

XNUMX. Syndromic deafness:Clinically common syndromes of deafness include large vestibular aqueduct syndrome, Usher syndrome, Aerstrom syndrome, and Waardenburg mentioned in the previous medical record.
Syndrome etc.This part of children often manifests as a group of clinical manifestations of different organs of the body including deafness.Taking Waardenburg syndrome as an example, Waardenburg
Syndrome (Waardenburg
Syndrome, ws) is one of the common clinical diseases that cause syndromic deafness. The inheritance method is autosomal dominant. At least 2% of congenital deafness is caused by Waardenburg syndrome.The pathogenesis of the disease is a syndrome caused by the abnormal function of the neural crest cells caused by the lack of melanocytes derived from the neural crest. Waardenburg syndrome mainly manifests as sensorineural hearing loss and pigment abnormalities. The latter includes heterochromia of the iris, white frontal hair, early white hair, hypopigmentation of the skin, or freckles; other manifestations include ectopic canthus, high and wide nasal roots , Hirsutism, straight eyebrows or flushing in the middle of eyebrows, etc.

preventive solution:Most of the diseases are hereditary, and deafness is mostly manifested as bilateral severe sensorineural hearing loss. The treatment can be based on the degree of deafness by choosing hearing aids or implanting cochlear implants and gaining certain results.In a hearing implantation center of Otorhinolaryngology Head and Neck Surgery of a General Hospital of the People’s Liberation Army, cochlear implants were successfully implanted in more than 1300 patients with severe deafness, including a large part of children with syndrome deafness. 16 children with Waardenburg syndrome underwent cochlear implant surgery in our department and achieved good hearing and speech rehabilitation.Prenatal diagnosis can avoid the birth of some children with syndrome deafness.