Briefly describe four kinds of hereditary deafness

遗传性耳聋主要有隐性、显性、伴性染色体遗传和线粒体母系遗传四种遗传方式。常染色体隐性遗传是其中最常见的一种遗传方式,约占遗传性耳聋的80%左右。这种遗传方式的特点是父母双方可能都听力正常,但分别携带单个隐性基因突变(携带者)。在他们的后代子女中,如果孩子从父母那里通过遗传获得父母方所携带的两个基因突变,则极有可能因此产生遗传性耳聋。在我国,60%以上的遗传性耳聋患者是由GJB2或SLC26A4基因突变所造成的,而这两个常见耳聋基因的绝大部分突变都是以隐性方式所遗传。一般来说,以隐性方式遗传的耳聋患者往往在出生时即具有听力障碍,且听力损失情况比较严重,多见于重度、极重度耳聋。

Autosomal dominant deafness accounts for about 15% of hereditary deafness.Contrary to recessive hereditary deafness, dominant hereditary deafness is characterized by the fact that as long as the patient carries a single dominant gene mutation, it can cause deafness. One of the patient’s parents and older ancestors often has deaf patients, and the family history is more obvious.Most deafness with dominant inheritance has mild hearing loss. Hearing conditions are usually acceptable at birth, but gradually deteriorate with age in later stages.

The ratio of sex-linked chromosome inheritance and mitochondrial maternal inheritance is relatively low, accounting for only 2-3% of hereditary deafness.However, compared with other countries and races, the mitochondrial gene mutation A1555G is relatively more common in the Chinese population.This mutation can cause patients to be sensitive to aminoglycoside antibiotics, which can easily lead to drug-induced deafness.Therefore, people with mutations in the mitochondrial gene A1555G should try to avoid exposure to aminoglycoside antibiotics such as gentamicin and streptomycin. This is especially true for newborns who are more susceptible to infections and often need to use antibiotics. important.

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