What Causes A Baby To Be Born Deaf & What is its Treatment?

When a baby is born deaf then it is termed as congenital hearing loss, i.e. hearing loss is present from the child’s birth. There are genetic and non-genetic causes for a baby to be born deaf. Congenital deafness may also develop later which occurs as a result of genetic causes or factors which affected the fetus in utero.

Non-genetic factors are responsible for about 25% of the babies being born deaf. Some of the Non-genetic factors which cause a baby to be born deaf are:

• Premature birth.
• Low birth weight.
• Maternal infections or the mother suffering from infection when she was pregnant, such as German measles (rubella), herpes simplex virus or cytomegalovirus.
• Birth injuries or injuries incurred by the baby during its birth.
• Complications associated with the Rh factor in the blood/jaundice.
• Toxins such as alcohol and drugs taken by the mother during her pregnancy.
• Maternal diabetes (gestational diabetes).
• Lack of oxygen (anoxia).
• Toxemia during pregnancy.

Hereditary or Genetic factors are responsible for more than half of the babies that are born deaf. The hearing loss occurring as a result of genetic defects can be present at birth or the deafness can develop later on in the child’s life. Most genetic hearing loss can be described as autosomal dominant or autosomal recessive. Additional rare types of genetic hearing loss include mitochondrial inheritance patterns and X-linked recessive inheritance.

Autosomal Recessive Hearing Loss In The Baby: In case of autosomal recessive hearing loss of the baby, both the parents carry the recessive gene which is then passed on to the baby. It comes as a surprise for the parents for find out that their child has congenital hearing loss as the parents themselves were not aware of carrying a defective gene. This type of inheritance pattern is responsible for about 70% of the babies having genetic congenital hearing loss.

Autosomal Dominant Hearing Loss In The Baby: A baby is said to have autosomal dominant hearing loss when he/she gets an abnormal gene from one of the parent and this defected gene is able to cause hearing loss in the baby even though the other gene from the other parent is normal. The parent who has the dominant gene will also likely have hearing loss along with symptoms which constitute the genetic syndrome. About 15% of the hearing loss babies are born deaf due to this autosomal dominant pattern.

There is a different category of signs and symptoms of this genetic syndrome which indicate a specific disease. Many genetic syndromes consist of hearing loss as one of their symptoms. Some of the examples are: Usher syndrome, Down syndrome, Treacher Collins syndrome, Alport syndrome, Crouzon syndrome and Waardenburg syndrome.

After a child has been diagnosed with congenital hearing loss, the anticipated and immediate reaction of parents and family members is that of denial. It is important that parents and family members are counseled by doctors and audiologists so that they can cope better with the situation and are motivated to seek solutions for overcoming this problem. It is commonly observed that after the family members and parents have been explained about the various options available for their child born with hearing impairment, they are more easily able to accept their situation. With the acceptance of the situation, half of the battle is won and treatment and rehabilitation of the child who is born deaf can be started.

Treatment for babies with congenital hearing loss or who are born deaf should start before they are 6 months of age. According to research, babies who are treated earlier than 6 months of age are able to develop good communication skills using sign language or spoken language which is almost as good as babies born with normal hearing.

There are many treatment options available for babies who are born deaf. It is important to decide which treatment is best for their child. Things which will be considered before starting treatment include the child’s age, personality, developmental level and extent of the hearing loss and the preferences of the parents. The type of treatment depends on many factors, the important one being the degree of deafness in the child. If the child has a fair amount of residual hearing, then with correct intervention a hearing aid can be fitted which is appropriate to the degree of deafness of the child.

There is a team of experts which decide on the treatment of babies who are born deaf and this panel of experts includes the otolaryngologist, the child’s primary care provider, speech-language pathologist, educator and an audiologist. All these experts work closely with parents and the baby to formulate an Individualized Family Service Plan. The treatment is modified as the child gets older.

Hearing aids are devices which amplify sound thus making it possible for many children to hear spoken words and develop language. Babies who are as young as 4 weeks old benefit from the use of hearing aid. However, children having severe to profound hearing loss will not be able to hear or make out enough sound to make speech audible, even with the help of a hearing aid. For young children, a behind-the-ear hearing aid is usually recommended as it is safer and fits and adjusts more easily as the child grows when compared to the hearing aid which fits within the child’s ear.

Children with profound or complete deafness will have limited benefits from the hearing aids. Depending upon the type and level of hearing loss, cochlear implants can be used instead of hearing aids.

Before fitting a hearing aid, the child’s residual hearing needs to be assessed by a competent audiologist. Then the performance of a hearing aid is checked and the child is then fitted with an appropriate hearing device. The child’s ear mould is also important as the hearing device has to be custom made to adapt to the shape of the child’s ear. There are a variety of good quality hearing aids available today such as digital body worn or analog devices.

Parents also have to decide the mode of communication between them and the child. If the method of communication is going to be oral (speech), then the child needs assistance learning lip reading skills and listening skills so that he/she understands what others are saying. Most of the children with congenital hearing loss also need language therapy or speech therapy.

A child also can learn to communicate using a form of sign language. American Sign Language (ASL) is the preferred language used in United States of America by children and individuals with hearing loss. The grammar and rules of ASL are distinct from English. Several variations of sign language are also present which can be used with spoken English.

Cued speech is a visual model of spoken language used by deaf people. It is difficult for children with hearing loss to learn lip reading as there are many sounds which appear same on the lips. This is where cued speech helps young children with hearing loss to learn spoken languages with normal vocabulary and grammar and to clearly see what is being said. Cued speech clarifies lip reading by using 8 hand shapes in 4 positions.

The time taken to learn the entire system is usually less than 20 hours.

If the child has a permanent conductive hearing loss occurring as a result of malformations of the middle or outer middle ear, or from repeated ear infections, then surgery may be needed. However, presence of fluid in the middle ear commonly causes temporary hearing loss, but chronic ear infection can result in a child to lag behind in language skills. Sometimes, a tube can be inserted through the eardrum which allows fluid drainage from the middle ear. This procedure can be done on an outpatient basis. Children with severe to profound sensorineural hearing loss may also need surgery.

Cochlear implant is a device which can be inserted via surgery in the inner ear of the children who are as young as 1 year. This device helps in stimulation of hearing. With the help of additional speech and language therapy, children with cochlear implants can learn to understand speech and speak relatively well; however, the extent of improvement differs from child to child.

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Also Read:

• Deafness by Birth: How can you Identify it & What are its Treatments?
• Can Hearing Loss be Treated Naturally?
• Sudden Hearing Loss: Causes, Diagnosis, Treatment, Prevention