What causes hereditary hearing loss?

Genetics is the main causes of hereditary hearing loss. Many parents are unfamiliar with how genetic causes can cause hearing impairment. As you work to identify the cause of hearing loss of your child, it will be a basic understanding of genetics and helpful for major categories of inheritance.

Genetic factor makes the hearing loss more severe due to aging, induced by noise, drugs or infections. Around 50% of childhood hearing loss is genetic. There is about 400 genetic hearing loss identified by the researchers.

genetic hearing loss

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Internal ear sensory hair cells play an important role in our hearing, and mutation in these cells can prevent them from working properly, which results in hearing loss. Finally, gene mutations can be the cause of many non-hearing, hereditary situations which are combined with internal deformity, resulting in deafness in life or later.

The causes of hereditary hearing loss include Otosclerosis, Usher’s syndrome, and Pendred syndrome.

Connexin Gene 26

Now the question arises what is connexin 26? Gene carries the instruction of protein called connexin 26 (cx26).

Connexin 26 (Cx26) is a protein present on the (GJB2) quality. And it is the most well-known reason for innate sensorineural hearing loss. It changes are in charge of in any event 20% of all hereditary hearing misfortune and 10% of all youth hearing misfortune. Kids born with bilateral (both ears) severe to profound sensorineural hearing losses. Generally, refer to the Connexin 26 mutation tests.

Importance of Connexin 26

The GJB2 quality contains the directions for assembling various proteins, including Connexin 26. Connexin proteins all are designated “hole intersection proteins”. They are fundamental for cells to speak with one another. Without adequate dimensions of Connexin 26.  The potassium stream from hair cells in the cochlea disturbs. As a result in very elevated amounts of potassium in the Corti s organ, results in sensorineural hearing loss.

Two categories of genetic hearing disorder

  • Syndromic: Syndromic reflect that a person has other symptoms along with hearing loss such as people with hearing loss also blind.
  • Nonsyndromic: Nonsyndromic means that the person does not have any other symptoms, and it associated with gens

Different types or causes of hereditary hearing loss

Dominant inheritance:

Genes can work in different ways. Sometimes a child only needs to inherit one gene, from either their mother or their father (dominant inheritance), to have that particular characteristic.

Recessive inheritance:

Sometimes the child would need to have the same gene passed on from both parents in order to have a condition (recessive inheritance).

X-linked or Sex-linked:

This hearing occurs when the changed gene depends on the X chromosome. As we know the female has 2 X chromosome and male has 1 X chromosome. The female chromosome rarely shows the effects on a changed gene on the X chromosome while the male X chromosome shows the effect on a changed gene.

Mitochondria Gene Effect

Genes are stored in Mitochondria. Mitochondria are the powerhouse of the cell. The mistake in mitochondrial genes affects the functioning of the body part such as ears, eyes, kidneys, muscle, and heart. These type of changes result in hearing loss.

Genes pass from one generation to another generation. So as mitochondrial genes are passes. Changed mitochondrial genes determine the severity of the loss.


Scientists around the world are working to map out the genetic mutations associated with hearing loss and are often doing new discovery. Genetic mutations associated with hearing loss and are often doing new discovery. Other scientists are experimenting with gene therapy, which can be a significant treatment for future hereditary and genetic hearing loss, perhaps in the combination of stem cell therapy.

If you have family members who have experienced hearing loss later in life, then you may have a genetic disturbance for hearing loss. Some types of hearing losses are not apparent until later in life. Because they come into the picture when a person comes into contact with environmental factors. For example, mitochondrial related hearing loss is inherited genetically. And one person can be triggered after taking aminoglycoside (one type of antibiotic).

It is important to check your hearing to know whether you are also facing hearing loss. Talk to your doctor and audiologist if you are having trouble hearing because they can help to determine what is the cause of the loss, as well as the best course of treatment.

Read also:

Genetic Syndromes Associated with Hearing Loss

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