Congenital deafness refers to the deafness that exists after birth, which can be divided into hereditary and non-hereditary; mainly autosomal recessive inheritance, accounting for more than 75%, non-hereditary congenital deafness accounting for about 20%, hereditary Deafness is divided into two categories: nonsyndromic and syndromic, accounting for about 80% and 20% respectively.There is also a type of deafness caused by mitochondrial diseases. Non-hereditary deafness can be caused by congenital infections and metabolic factors of pregnant women.
Non-hereditary deafness: The mother suffering from viral infections such as rubella, mumps, influenza, or systemic diseases such as syphilis, diabetes, nephritis, sepsis, cretinism, or a large number of ototoxic drugs can make the fetus deaf. Blood Rh factor is contraindicated, too long labor during delivery, dystocia, birth injury and fetal hypoxia and suffocation can also cause deafness.
Deafness caused by deaf genes located on sex chromosomes is called sex-associated hereditary deafness.Autosomal hereditary deafness can be manifested as a variety of bony labyrinth and membrane labyrinth development abnormalities without related malformations. While eliminating or treating the cause of the disease, drugs that can dilate the blood vessels of the inner ear, reduce blood viscosity and dissolve small blood clots as soon as possible Anti-bacterial, anti-viral and steroid hormone drugs can also be used when necessary.It can be used if the drug treatment is ineffective Hearing aid
Once congenital deafness is discovered, it should be treated actively in the early stage. If it is not treated in time, it will eventually lead to loss of language ability.
1. Congenital middle ear malformations should be corrected by surgery, the best time for surgery is over 15 years old;
2. Congenital sensorineural hearing loss is irreversible, and there are no effective drugs or surgical treatments;
(1) Early detection and early diagnosis of congenital deaf children should be achieved;
(2) Deaf children, regardless of age, once they are found to be diagnosed, those with residual hearing should wear them as soon as possible Hearing aid , Carry out hearing and speech rehabilitation training.
The treatment principles of congenital neurological deafness are:
(1) Recovery or partial recovery of hearing loss;
(2) Try to preserve and use residual hearing.
The specific method is as follows:
1. Drug therapy has many reasons for deafness, and the pathogenesis and pathological changes are complex and different. So far, there is no simple and effective drug or therapy suitable for any situation.At present, while eliminating or treating the cause disease, drugs that can dilate inner ear blood vessels, reduce blood viscosity and dissolve small thrombosis, vitamin B drugs, energy preparations, and antibacterial and antiviral drugs can be used as soon as possible. And steroid hormone drugs.Hearing aids can be used for those who do not respond to medication.
2.助听器 是一种帮助聋人听取声音的扩音装置。它主要由微型传音器、放大器、耳机、耳模和电源等组成。助听器种类很多，就供个体应用者讲，就有气导和骨导、盒式与耳级式(眼镜式、耳背式和耳内式)、单耳与双耳交联等。一般需要经过耳科医生或听力学家详细检查后才能正确选用。语频平均听力损失35-80dB者均可使用;听力损失60dB左右效果最好。单侧耳聋一般不需配用助听器。双侧耳聋者，若两耳损失程度大体相同，可用双耳助听器或将单耳助听器轮换戴在左、右耳;若两耳听力损失程度差别较大，但都未超过50dB者，宜给听力较差耳配用;若有一耳听力损失超过50dB，则应给听力较好耳配戴。此外，还应考虑听力损害的特点;例如助听器应该现用于言语识别率较高，听力曲线较平坦，气骨导间距较大或动态听力范围较宽之耳。 传导性聋者气导、骨导助听器均可用。外耳道狭窄或长期有炎症者宜用骨导助听器。感音性聋伴有重振者需采用具备自动增益控制的助听器。合并屈光不正者可用眼镜式助听器。耳背式或耳内式助听器要根据患者的要求和耳聋的情况选用。初用助听器者要经调试和适应过程，否则难获满意效果。
3. The cochlear implant is also called electronic cochlea or cochlear implant.It is often used for young and middle-aged people with normal mentality and good health who are extremely deaf after language learning.It must be the use of high-power hearing aids to be ineffective, no active lesions in the ears, imaging examinations to prove that the structure of the inner ear is normal, the cochlear electrogram cannot be detected, but the promontory or cochlear window electrical stimulation can induce a brainstem response.Electronic cochlea is based on the fact that most of the cochlear spiral nerve fibers and ganglion cells are still alive in the perceptually deaf. The microelectrodes connected to the acoustic-electric transducer outside the body are inserted into the scala tympani or pasted through the cochlear window. Attached to the outer bone wall of the cochlea, it is used to directly stimulate the nerve endings and transmit the simulated auditory information to the center, so as to make the deaf person re-perceive the sound.With speech training, some speech functions can be restored.
4. The former is to use hearing aids to use the residual hearing of the deaf to gradually cultivate their listening habits through long-term planned sound stimulation, and to improve the ability of auditory perception, auditory attention, auditory positioning and recognition, and memory.Speech training is based on the complementary functions of hearing, vision, and touch, with the help of appropriate instruments (audio indicators, speech instruments, etc.) to train deaf children to speak, read lips, understand and accumulate vocabulary, and master grammatical rules through scientific teaching methods. Express thoughts and feelings flexibly and accurately.Occurrence training includes training on breathing methods, lips and tongue movements, noise use, as well as phonemes, pitch, intonation and other items.Auditory and speech training complement each other and promote each other. They should not be neglected. They should start as early as possible and be implemented interspersedly.If family members and teachers can cooperate closely and persevere, they will be able to achieve the goal of being deaf and not dumb.
