Introduction to non-syndromic hearing loss

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Brief information

Incidence rate

Genetic chromosomal dominant, or autosomal recessive, or x-linked recessive

Infant age infants

ICD-10 H90.5

Disease description

Hereditary herniaNonsyndromic hereditary herniaTwo major categories. The former refers to lesions in the eyes, bones, kidneys, skin, etc., in addition to deafness. These deafness account for 30% of hereditary paralysis; the latter only has symptoms of deafness, accounting for 70% of hereditary paralysis.

Cause

Nearly 30,000 newborns suffer from congenital hearing impairment, 60% of which are caused by genetic variation. It is estimated that every 22 East Asians carry the GJB2 gene with hearing loss.

symptom

The severity of hearing impairment varies from mild (difficult to understand soft whispers) to severe (even loud noises can't be heard) and can change over time. Moreover, it can occur at any age, may be stable, or it may increase with age.

Mutations in the GJB2 gene cause bilateral symmetrical hearing impairment, and the range of loss varies widely, from mild to very severe, but most are severe or very severe hearing loss. The GJB2 gene mutation is the main cause of congenital hearing impairment in Chinese people, accounting for more than half of those with recessive non-syndromic hearing loss.

Prevention

Hearing impairment caused by mutations in the GJB2 gene must be aided by hearing aids and transplanted cochlea in early hearing recovery therapy. Electrophysiological testing Conventional hospitals or biochemical testing sites cannot determine the type of hereditary hearing loss. Hereditary deafness gene detection, by analyzing the DNA of the subject, identifies genes associated with hearing impairment. Therefore, the clinical diagnosis of hearing impairment provides a basis for this disease and is the most effective method for current cause analysis.

The subjects of hearing genetic counseling are mainly the following:

First, a deaf-mute couple who gave birth to a normal baby wants to know the risk of recurrence of the baby.

Second, a normal couple with a family history of hearing impairment, worried that the disease will be passed on to the next generation.

3. Unmarried or married men and women, one of whom is a nephew, who needs a marriage counselor to give birth guidance before or after marriage.

Fourth, the GJB2 gene carriers, but family members with normal hearing and no hearing loss, they are also within the scope of genetic counseling.

Early diagnosis of congenital hearing impairment helps to take early interventions to help patients develop their language and improve their quality of life.

Categories: Nonsyndromic hearing loss
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Nonsyndromic hearing loss

Introduction to non-syndromic hearing loss

Brief information alias incidence rate genetic pattern chromosomal dominant, or autosomal recessive, or x-linked recessive onset age infancy newborn ICD-10 H90.5 disease description hereditary sputum is divided into hereditary hereditary sputum and non-synthesis There are two major categories of hereditary hernia. The former refers to lesions in the eyes, bones, kidneys, skin, etc., in addition to deafness. These deafness account for 30% of hereditary paralysis; the latter only has symptoms of deafness, accounting for 70% of hereditary paralysis. The cause is nearly 30,000 newborns with congenital hearing impairment, 60% of which are caused by genetic variation. It is estimated that every 22 East Asians carry the GJB2 gene with hearing loss. The severity of symptoms of hearing impairment varies from mild (difficult to understand soft whispers) to severe (even loud noises can't be heard) and can change over time. Moreover, it can occur at any age, may be stable, or it may increase with age. Mutations in the GJB2 gene cause bilateral symmetrical hearing impairment, and the range of loss varies widely, from mild to very severe, but most are severe or very severe hearing loss. The GJB2 gene mutation is the main cause of congenital hearing impairment in Chinese people, accounting for more than half of those with recessive non-syndromic hearing loss. To prevent hearing damage caused by mutations in the GJB2 gene, hearing aids and cochlear implants must be worn in early hearing recovery treatment. Electrophysiological testing Conventional hospitals or biochemical testing sites cannot determine the type of hereditary hearing loss. Hereditary deafness gene detection, by analyzing the DNA of the subject, identifies genes associated with hearing impairment. Therefore, the clinical diagnosis of hearing impairment provides a basis for this disease and is the most effective method for current cause analysis. The subjects of hearing genetic counseling are mainly the following: 1. A deaf-mute couple who has given birth to a normal baby wants to know the risk of recurrence of the baby. Second, a normal couple with a family history of hearing impairment, worried that the disease will be passed on to the next generation. 3. Unmarried or married men and women, one of whom is a nephew, who needs a marriage counselor to give birth guidance before or after marriage. Fourth, the GJB2 gene carriers, but family members with normal hearing and no hearing loss, they are also within the scope of genetic counseling. Early diagnosis of congenital hearing impairment helps to take early interventions to help patients develop their language and improve their quality of life.

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