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Hearing loss A Genetic Disorder that affects Speech and Language by Kelly Jean Sullivan

 



Hearing loss A Genetic Disorder that affects Speech and Language


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Genetic factors cause more than 50 percent of all hearing loss in children, whether present at birth or developed later in life.

Definition

Hearing loss exists when there is diminished ability to the sounds usually being heard. The terms hearing loss means not able to hear sound in the speech frequencies. The severity of a hearing loss is categorized according to the increase in the intensity of sound above the normal levels that are needed so that the listener can hear it.

     Deafness is as a loss of hearing in a person who is unable to understand speech even in with amplification. In profound deafness, even the highest intensity sounds produced by an instrument used to measure hearing by producing pure tone sounds cannot be detected.

Total deafness is when there are no sounds at all. Hearing loss, (1998).

     Speech perception = another aspect of hearing that involves the listener's clarity of a word rather than the intensity of the sound made by the word. We can measure this by tests of speech discrimination.  The Tests measure the child's ability to understand speech, not to only to detect sounds. Schaefer (2012)

       Deafness affects 1 per 1000 children and about fifty percent of childhood hearing loss in the USA. Hearing Loss attributed to genetic factors. The Causes of Hearing Loss are genetic and are due to changes in the genes that are involved in the hearing process.

      Hearing loss = this is the most common type of genetic congenital hearing loss - autosomal

Sometimes, hearing loss is due to a combination of genetic and environmental factors. There is, for example, a genetic change that makes some people more likely to develop hearing loss after taking certain antibiotic medications Nightengale (2017)

      Some hearing loss is genetic. There are hereditary causes called gene mutations. Hereditary hearing loss causing hearing impairment include Otosclerosis, Usher's syndrome, and Pendred syndrome. Research shows that eighty percent of prelingual deafness is genetic, the most common being, autosomal recessive and nonsyndromic. One of the major causes of severe to profound autosomal is genetic the recessive disorder =non syndromic hearing loss in most populations is a mutation disorder. Pollack (2018).

     The most common cause of congenital hearing loss=

 The Genetic factors that might cause congenital hearing loss include autosomal recessive genes.

    -Fifty percent of hearing loss in little ones is due to genetic causes. There are also several things in the environment that can cause hearing loss.

-Environmental causes can be mother’s infection during pregnancy and complications after birth. Sometimes both genes and environment work together to cause hearing loss.  Some medicines can cause hearing loss, but only in people who have certain mutations in their genes. Krof (2000)

        Genes are what hold the instructions that tell the cells of how to grow and work. Genes are involved in hearing development. When a gene does not form correctly, this is called a mutation.  Some mutations run in families, and others do not. If more than one person in a family has hearing loss, this is a red flag for the rest of the family. Schaefer (2012)

       Seventy percent of all mutations that cause hearing loss are non-syndromic. This means that the person does not have any other symptoms. The rest thirty percent of the mutations causing hearing loss are syndromic. Syndromic is when a person has other symptoms besides hearing loss.  An example of this would be that a child with hearing loss can also be blind. Nightengale (2017)

       The cochlea is the part of the ear that changes sounds in the air into nerve signals to the brain is a very complex and specialized part of the body that needs many instructions to guide its development and function. The instructions come from genes. Changes in any one of these genes can result in hearing loss. The GJB2 gene is one of the genes that contain the instructions for a protein called connexin 26. It is this protein that will play an important role in the functioning of the cochlea. Pollack (2018)

Causes of childhood hearing loss=

     .Hearing loss in children can also be conductive, sensorineural, or mixed. It's important for the parents and the teachers, know the signs of and addresses hearing loss in children. Early hearing loss - especially that which is undiagnosed - can cause significant development and emotional problems for children that have long-lasting effects. Halliday (2017)

Congenital hearing loss=

     Congenital hearing loss means it was present in an infant at birth. There are various causes of congenital hearing loss, though they are not always easily identified. Genetic factors that might cause congenital hearing loss include:

Autosomal recessive hearing loss - This is the most common type of genetic congenital hearing loss - autosomal recessive accounts for around seventy percent of all genetic hearing loss cases.

     Researchers have identified more than thirty genes that, when they mutate, can cause no syndromic deafness.

     Many genes related to deafness are involved in the development and function of the inner ear. Mutations in these genes result in hearing loss by interfering with critical steps in processing sound.

 

Why is diagnostic confirmation by an audiologist skilled in evaluating infants and young children important?

Hearing loss can be inherited. Around eighty percent of all these cases are inherited by recessive genes.  Twenty-five percent are inherited by dominant genes.  Korf (2000).

     There are two different kinds to look at, syndromic and no syndromic.

Syndromic deafness =when there are other signs or medical problems aside from deafness in the child.  No syndromic deafness happens when there are no other signs or medical problems associated with the child beside the deafness. Blamey (2001)

 We can use something called gene mapping to identify this. Blamey (2001) .The most common recessive syndromic forms of hearing loss are Pendred syndrome and Usher syndrome.

       The congenital defect microbial, deformed or unformed outer ear, can be associated with partial or complete conductive deafness, depending upon the severity of the deformity and whether the middle ear is also affected. It can also be associated with abnormalities of the inner ear giving rise to an additional sensorineural component to the hearing loss. Korf (2000)

How the disorder affects the development of speech and language.

     Hearing loss can affect a child's development of speech and language skills. When a child has difficulty hearing, the areas of the brain used for communication may not develop appropriately. This makes understanding and talking very difficult. Hearing loss can be a result of numerous factors or events. Delage (2007)

      Children with listening difficulties due to a hearing loss or auditory processing problems continue to be at risk for developmental delays.  A hearing loss affects children in four major ways:  It causes a delay in the development of receptive and expressive communication skills. Hilt (1998)

   This is new to me and seems very important to be aware of for the exceptional Ed teacher.

         The earlier hearing loss occurs in a child's life, the more serious the effects on the child's development. The hearing loss can cause a delay in the development of receptive and expressive communication skills. The language deficit causes learning problems that result in reduced academic achievement. Nightengale (2017)

     Hearing loss can affect personal and work life. Hearing loss can affect a person in three main ways: fewer educational and job opportunities due to impaired communication social withdrawal due to reduced access to services and difficulties communicating with others. Delage (2007)

 

How hearing loss affects speech and language development

Speech perception – Another aspect of hearing involves the perceived clarity of a word rather than the intensity of the sound made by the word.

 In humans, that aspect is usually measured by tests of speech discrimination. These tests measure one's ability to understand speech, not to merely detect sound. There are very rare types of hearing loss, which affect speech discrimination alone. One example is auditory neuropathy, a variety of hearing the loss in which the outer hair cells of the cochlea are intact and functioning, but sound information is not faithfully transmitted to the auditory nerve and brain properly. Pollack (2018)

     Hearing loss can affect a child's development of speech and language skills. When a child has difficulty hearing, the areas of the brain used for communication may not develop appropriately. This makes understanding and talking very difficult.  Most hearing losses are identified through screening at birth.

     Children with listening difficulties due to a hearing loss or auditory processing problems continue to be at risk for developmental delays. The Loss of hearing effects children: / it delays in the development of receptive and expressive communication skills.

All children are different in how they develop. There are developmental stages that most children follow. Hearing loss can affect how communication develops in many ways. Hearing loss, (1998). Some of the important things as a teacher we need to be aware of are-

 

Age at the identification of the hearing loss

Family involvement and support

Type and degree of hearing loss

Age of amplification

Consistency of device use

Type of treatment (early intervention, direct therapy, school-based therapy)

Cognitive and motor skill level

Other medical conditions

Hilt, (1998).

    Hearing loss is linked to genetic disorders and can affect speech and language development. The child's development and learning can be a problem in the classroom for the child. The earlier hearing loss occurs in a child's life, the more serious the effects on the child's development. It causes a delay in the development of receptive and expressive communication skills. This has a great impact on speech and language development.

      The language deficit causes learning problems that result in reduced academic achievement. Teachers need to be aware of this so that they can correctly work with children in speech and language development.


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