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Why is newborn hearing screening necessary?

Congenital hearing loss is one of the most common birth defects. Its incidence is as high as 1 ‰ ~ 3 ‰. Every year, there are 20000-30000 new born deaf children, and the hearing loss of 0-6-year-old deaf children is mainly severe and extremely severe. It ranks first among all kinds of newborn diseases that can be screened. The hearing loss of newborn and infant can affect the development of speech, cognition and emotion, and the severe can lead to deaf mute, which brings heavy burden to family and society. According to the routine physical examination or parents’ observation, the hearing impairment can only be found after the child is 2 years old, the period of rapid language development of the child is missed, and the best time for rehabilitation is delayed. Newborn hearing screening can find congenital hearing loss in the early stage, through early intervention and early rehabilitation, make it deaf but not dumb, and return to the mainstream society.


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Newborn hearing screening

1. The primary screening of normal newborns is completed 48 hours after birth and before discharge. Those who fail or miss the primary screening will be re screened about 42 days after delivery. Transient evoked otoacoustic emissions (TEOAE) or distortion product otoacoustic emissions (DPOAE) were used as screening methods. If the re screening fails, it is necessary to refer to the children’s hearing Diagnosis Center for hearing diagnosis within 3 months.

2. The initial screening of NICU newborn was carried out before discharge after the condition was stable. The screening methods were automatic auditory brainstem response (AABR), or otoacoustic emission (OAE) and AABR combined screening to avoid missed diagnosis of auditory neuropathy. Those who fail the primary screening will not be re screened, but will be directly referred to the children’s hearing Diagnosis Center for hearing diagnosis within three months. Because most of NICU newborns have high-risk factors of hearing loss, which may lead to delayed hearing loss, even if they pass the hearing screening, they need to check their hearing every six months to one year, at least 3 years old.

What is neonatal deafness gene screening?

Deafness gene screening is to collect cord blood or heel blood at birth or within 3 days after birth to screen the susceptibility and common genes of deafness. At present, nine common mutations of GJB2, SLC26A4, mitochondrial 12SrRNA and gjb3 genes are screened by deafness gene chip. It can find the high-risk children of delayed hearing loss caused by deafness gene mutation early, and drug-induced deafness sensitive children, so as to achieve early diagnosis and early warning, which is an effective supplement for newborn hearing screening.


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There are three common deafness susceptibility genes

1. GJB2 gene mutation can cause severe sensorineural hearing loss or delayed hearing loss.

2. SLC26A4 gene mutation can lead to large vestibular aqueduct syndrome, which is characterized by progressive and fluctuating hearing loss.

3. Mutations in mitochondrial 12SrRNA gene may lead to drug sensitive deafness.

What should we do if newborn hearing screening fails?

(I) screening

Implementation of two-stage screening: under the premise of informed consent and informed choice of newborn guardians, medical and health care institutions conduct primary screening from 2-5 days after birth to before discharge. Those who fail to pass the screening shall be re screened within 42 days after birth. Those who fail to pass the screening shall be transferred to a tertiary hospital with the qualification of newborn disease (hearing) testing. For the key screening objects, even if they pass the screening, they should be followed up once every six months in 3 years, and the problems found in the follow-up should be referred to the doctor in time for diagnosis and treatment.

(II) diagnosis and evaluation:

Instrument evaluation: for infants who fail to pass the reexamination, Otolaryngology examination, acoustic immittance, otoacoustic emission, auditory brainstem evoked potential examination, behavioral audiometry and other related examinations shall be carried out in the tertiary hospital with the qualification of newborn disease (hearing) examination at the birth of 2-3 months, and medical and imaging examinations shall be carried out. Generally, diagnosis and evaluation shall be made at the age of 6 months to make clear the hearing The extent of the loss and the location of the hearing loss and the possible causes.

(III) treatment and intervention

1. Treatment: the third level hospital with the qualification of newborn disease (hearing) test is responsible for the treatment of the causes of hearing disorders.

2. Hearing compensation or reconstruction: for the children diagnosed with sensorineural deafness, the third level hospital with the qualification of newborn disease (hearing) test shall guide the children to carry out the following early intervention:

(1) selection of hearing aids: for children with residual hearing (light to severe hearing impairment), appropriate hearing aids shall be selected according to relevant standards for hearing correction;

(2) cochlear implant: for children with severe and very severe cochlear sensorineural deafness, cochlear implant can be carried out when conditions permit;

(3) hearing and language training: after wearing hearing aids or cochlear implants, arrange the subjects to the relevant rehabilitation institutions for corresponding hearing language rehabilitation training and family rehabilitation guidance, so as to promote the development of hearing and language of deaf children.

Classification and classification of deafness 1. Classification of deafness

According to who’s hearing loss classification standard in 1980, the average speech frequency pure tone hearing threshold was divided into five levels.

Mild deafness: the pure tone and language hearing threshold of audiometer is 26-40 dB.

Moderate deafness: hearing threshold 41-55db

Moderate and severe deafness: hearing threshold 56-70db.

Severe deafness: hearing threshold 71-91db.

Total deafness: pure tone audiometry threshold exceeds 91dB.

2 classification of deafness

According to the location and nature of the lesion, it can be divided into three categories.

Conductive deafness: deafness caused by pathological changes in the transmission mechanism of the outer and middle ear, and disturbance of sound wave entering the inner ear, such as cerumen embolism, otitis media, etc.

Sensorineural deafness: refers to the pathological changes of the spiral organ of the cochlea, which can not change the sound wave into nerve excitation or the nerve and its central pathway are blocked, which can not transmit the nerve excitation; or the central pathological changes of the cerebral cortex can not distinguish the language, collectively referred to as sensorineural deafness.

Mixed deafness: there are pathological changes in both the transmitting and sensing mechanisms

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