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Causes of hearing loss in children
Hearing loss can occur at any age. About1/800 ~11000 newborns have severe and extremely severe hearing loss at birth, and more than 2~3 times of newborns have slightly mild hearing loss, including mild to moderate, bilateral or unilateral hearing loss. Childhood, another 2/65438+. There is acquired moderate to severe progressive or permanent hearing loss, and many teenagers are at risk of sensorineural hearing loss due to excessive exposure to noise or head injury.
Hearing impairment can lead to lifelong impairment of children's language acceptance and language expression. The severity of the injury is determined by several factors: the age of hearing loss; The nature of hearing loss-its duration, damage frequency, loss degree and each child's susceptibility (including virus damage, mental retardation and basic language defects), can obtain an accurate assessment of hearing-damage frequency, air and bone conduction loss-and is not affected by age and disability (see section 256 on children's hearing test).
For children with sensorineural hearing loss, the extra burden of conductive hearing loss will seriously reduce their ability to distinguish speech. Compared with other healthy children, hearing loss has a more serious impact on other children with sensory, language and cognitive defects.
Etiology and pathogenesis
Conductive hearing loss Most hearing defects are acquired conductive hearing loss, which is related to otitis media and its sequelae. Almost all children have experienced mild to moderate, intermittent or persistent hearing loss caused by otitis media. Recurrent attacks or severe infections can lead to permanent defects. Children and boys who are most vulnerable to otitis media are those with craniofacial abnormalities (such as cleft palate), immunodeficiency (such as transient hypogammaglobulinemia in infants) and exposure to environmental risk factors (such as swallowing and day care places).
Diseases in any part of the auditory system may lead to hearing loss in children. Malformations of external auditory canal and middle ear (such as Treacher Collins syndrome) that occur alone or as part of the syndrome can lead to loss of conduction function. Children with isolated middle ear malformation may suffer from congenital sensorineural hearing loss.
Cholesteatoma is a benign tumor, which often occurs in patients with untreated otitis media or may be congenital. Acquired cholesteatoma usually occurs in the part of the back of the head with middle ear cleft, while congenital cholesteatoma occurs in the head and abdomen. Cholesteatoma can lead to ossicular chain necrosis and conductive hearing loss, middle ear fissure infection and closure, and can also lead to structural destruction of ossicles. The long-term pathological process of incus is the most common influencing factor, which can lead to significant conductive hearing loss.
Sensorineural hearing loss when the loss of sensation (hair cells) and nerve (spinal ganglion cells) occurs before delivery, it is called congenital sensorineural hearing loss; If hearing loss occurs in 1 or the second year after birth, it is called early-onset progressive sensorineural hearing loss in children. Congenital sensorineural hearing loss can be caused by external and internal factors (Table 260-4 and Table 260-5).
Acquired sensorineural deafness can be caused by the following reasons: autoimmune diseases; Otototoxic drugs such as aminoglycosides, cisplatin and aspirin (the overall effect is reversible); Bacterial meningitis; Congenital and acquired viral infections, such as congenital rubella, cytomegalovirus and mumps; Bacterial endotoxin and exotoxin; Hearing impairment due to exposure to loud music, firearms, engine noise or noisy toys; Hearing loss caused by temporary bone oscillation or fracture (fracture contains conductive components, which is used for hearing loss caused by traumatic destruction of middle ear)
Different pathological conditions will affect the inner ear, the most common is the loss of cochlear hair cells, usually including vestibular system. Cochlear nerve cells-spinal ganglion cells-are often protected for a period of time, but will eventually degenerate due to lack of nutritional factors, such as neurotrophic factors produced by the brain from hair cells. In addition, the loss of spinal ganglion cells can occur without or with a small number of hair cells.
Bone labyrinthine malformation can also lead to sensorineural hearing loss, CT diagnosis The degree of hearing loss related to labyrinthine malformation is from scratch, which can be stable, different or progressive. This hearing loss can contain conductive components. A group of X-linked labyrinthine malformations include oval window damage, and children present with congenital conductive hearing loss. Surgical incision of oval window can lead to a large amount of cerebrospinal fluid loss, which is often accompanied by permanent hearing damage in this ear.
External lymphatic fistula can lead to progressive sensorineural hearing loss, often accompanied by labyrinthine deformity. Traumatic lymphatic fistula is found in children with head trauma. Occasionally, newborns or infants with mild head injuries may have bilateral fistulas, leading to rapid progressive sensorineural hearing loss. This condition must be diagnosed immediately, because surgical intervention can prevent further hearing loss and possible meningitis.
Primary auditory nerve disease (ⅷ) is rare in pediatrics. The most common disease is neurilemmoma in patients with multiple neurofibroma type II, and other rare causes are meningioma and metastatic tumor. Occasionally, children with severe nuclear jaundice will experience sensorineural hearing loss and other signs of brain stem injury, such as motor function injury.
Unilateral hearing loss can occur in any of the above situations, and mumps is a common cause. The results of unilateral hearing loss are often underestimated. This kind of child is hard to hear clearly in a moderately noisy environment, so it can develop into a significant language barrier.
diagnose
Diagnosis is often seriously delayed due to ignorance or neglect. Severe hearing loss is usually diagnosed at the age of 2, while mild to moderate unilateral hearing loss is not discovered until school age. For severe bilateral congenital sensorineural hearing loss, parents may notice that newborns do not respond to their own voice or other sounds around 1 week after birth.
All infants and children should be screened for hearing loss (see Section 256 Hearing Test for Children). The diagnosis of hearing loss must be made as soon as possible, so as to input appropriate language and achieve the best language development. The biggest obstacle to early diagnosis is the delay in referral to experts, even if it is realized or suspected that speech and language delay are caused by hearing loss. If the child's speech development is abnormal, we should consider the differential diagnosis of deafness, mental retardation, aphasia and autism.
Many children with sensorineural hearing loss have vestibular dysfunction, which is characterized by delayed or degraded motor development. In addition, children and young children with otitis media may have vestibular injury characterized by dyskinesia. Abnormal motor development is sometimes inconsistent with more comprehensive developmental backwardness, such as mental retardation, which leads to improper nursing.
treat cordially
The aim is to support the best language development. All children with hearing loss should be assessed for their language function and their language barriers should be corrected through appropriate treatment. The 1 year after birth is a critical period for language development, because children must listen to the language until they speak spontaneously. Deaf children can only develop their language through special training, ideally starting with their diagnosis of hearing loss. It is necessary to provide deaf children with a way of language input. For example, visual symbol language can be used as spoken language in the future.
Conductive hearing loss caused by otitis media can be improved by hearing AIDS or surgery (tympanotomy, with or without hyperplasia incision according to children's age), but decongestants and antibiotics can not improve the hearing loss of these children.
Sensorineural hearing loss can be helped by various hearing AIDS, and amplification hearing AIDS should be installed as soon as possible after diagnosis (even if it is only a 6-month-old baby). For bilateral sensorineural hearing loss, binaural amplifier can be placed behind auricle or used with in-ear hearing aid to obtain maximum hearing and develop hearing localization. Children over 2 years old with extreme bilateral hearing loss cannot fully benefit from the placement of this amplification hearing aid. But cochlear implants are suitable for cochlear implants. No matter whether deafness is congenital or acquired, cochlear implant has led to hearing communication in many extremely deaf children, but it seems to be more effective for those children who have developed language. Children who are deaf after meningitis may have ossification of the inner ear, so it is necessary to implant the cochlea as soon as possible to obtain the best effect. Implanting brain stem stimulation electrodes can help children who are deaf because tumors destroy auditory nerves.
Closing peripheral lymphatic fistula, whether congenital or acquired, can preserve part of hearing and prevent further hearing loss. Immunosuppressive drugs such as glucocorticoid are beneficial to children with inner ear diseases caused by autoimmune diseases.
A system should be provided for children with unilateral deafness in schools, including allowing teachers to send signals to hearing AIDS installed on that good ear with a microphone to improve their ability to listen to people in noisy environments.
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