2.2. Causes of deafness
When you begin working with deaf or hard-of-hearing CYP, it is important to check with the family and via medical records if the cause of their deafness is known. In many cases, the aetiology of deafness is not known or the family may have declined aetiological investigations. It may not be possible to discover the cause of deafness for many CYP.
The aetiology of deafness can influence how we work with a child or young person and impact the advice that we may give, eg communication style, placement options, and additional professionals.
There are different possible causes of deafness. Paediatric deafness can be generally described as congenital or acquired.
Congenital deafness is present from birth and can be classified as genetic or non-genetic. Genetic deafness is caused by the baby’s genes. Non-genetic deafness includes deafness caused by infections during pregnancy, infections the newborn baby has, trauma, prematurity, low birth weight, or hyperbilirubinemia.
Acquired deafness occurs after birth. This could be caused by infection, injury, or ototoxic drug treatment.
Deafness can be further described with the following definitions.
Genetic deafness can be passed on or inherited via DNA due to a mutated gene. There may not be any known family history of deafness, depending on whether the gene is dominant or recessive. If it is a dominant gene, this can come from either parent. In cases where it is recessive, both parents must be carriers of the gene in order for the mutation to be passed on to their children. Genetic causes can be subdivided into syndromic (more than one symptom) and non-syndromic (deafness only). Specific causes are discussed in more detail in the A–Z list section (2.3).
Environmental deafness can be caused by external factors such as medications, noise, and chemicals. An example of this could be gentamicin, given to treat a serious infection, or some types of chemotherapy drugs used to treat cancers.
Cisplatin-based chemotherapy agents used in childhood cancer treatment can result in sensori-neural deafness. Children having such treatment should be having their hearing monitored regularly. Any change in hearing thresholds may inform future treatments.
Gentamicin/streptomycin ameno glycoside
This is an antibiotic given to people in an emergency. In most cases, it serves to fight against a serious bacterial illness that could cause death. However, in some babies and older people, use of the drug can result in sensori-neural deafness.
Deafness can be caused by structural abnormalities of the outer, middle, or inner ear. Some examples of this are microtia, otosclerosis, and enlarged vestibular aqueducts (EVA). These are discussed in more detail in the A–Z list section (2.3).
Diseases of the ear
A range of different diseases of the ear have the potential to cause deafness, for example, cholesteatoma, Meniere’s disease, mastoiditis, and persistent otitis media with effusion (glue ear). You can find more detailed information about specific causes in the A–Z list section (2.3).
There are various other causes of deafness, such as prematurity and head trauma, which do not fall under the above categories.
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