Progressive Hearing Loss in Children

Progressive hearing loss in children needs to be monitored often in order to maintain best access to speech and language. Choja/Getty Images

When a parent hears the words "progressive hearing loss", there is a lot of uncertainty and anxiety. What is a progressive hearing loss? What does that mean for my child? In the most basic terms, a progressive hearing loss is when a hearing loss (mild or otherwise) has been diagnosed and it becomes worse. In young children who may not be able to verbalize a change in their hearing, parents need to be informed about the type of symptoms to watch for that may indicate a change in hearings, know risk factors and potential causes, and build a relationship with a pediatric audiologist to carefully monitor their child's hearing.


Known Risk Factors for Progressive Hearing Loss

Many known risk factors for progressive hearing loss in children exist. Just a few include having a family with a history of hearing loss, being born prematurely, or having physical malformations of the head or ears. If the mother gets certain infections while she is pregnant or while she is giving birth, that too can result in a progressive hearing loss.

Even if a newborn's hearing tests "normal," the Joint Committee on Infant Hearing recommends that any newborn who has any known risk factors for progressive hearing loss should have its hearing tested before the baby is three years old. This is because the baby might have had an undetectable mild hearing loss that deteriorated after birth.

Unconfirmed Risk Factors for Progressive Hearing Loss

A study was done with children born with cytomegalovirus (CMV) infections, a known cause of progressive hearing loss.

The researchers in this study wanted to see if they could identify any unique factors that could serve as a "warning" that the baby was at risk for developing progressive hearing loss.

First, the researchers looked at data for 21 babies born between 1994 and 1998 to see how much CMV was present in their urine during their first month after birth.

Almost two years later, the same babies were followed up to see if any had developed progressive hearing loss. Nine of those babies did develop hearing loss. Those nine babies had had more CMV in their urine when they were infants. The study's authors felt the results demonstrated a "significant association" between the amount of CMV in the urine and the development of hearing loss.

Symptoms of Progressive Hearing Loss

Families with children who have progressive hearing loss have noted certain symptoms: Their child's audiogram changes for the worse, or their child doesn't seem to hear what the child used to be able to hear, or the child's speech changes.

Causes of Progressive Hearing Loss

Babies may be born with the cytomegalovirus virus, which is known to cause progressive hearing loss. In children, it may also be caused by hereditary factors, such as Connexin 26; a few studies have found cases of progressive hearing loss when Connexin 26 is present. Researchers have also identified a PRPS1 gene in males, a gene that is associated with DFN2, a known rare cause of progressive hearing loss.

More causes include certain syndromes, such as Pendred syndrome, Alport syndrome, and Usher syndrome. Congenital (at birth) problems, such as malformations of the inner ear, can also result in progressive hearing loss. Mondini syndrome is one such malformation.

Stopping Progressive Hearing Loss in Children

Are there any ways to stop hearing loss from progressing in babies? Perhaps. Two small studies of newborns with CMV infections demonstrate a possibility that the drug ganciclovir could prevent or reduce progressive hearing loss. The first study involved nine babies, five of which already had a hearing loss. These babies were given a combination of intravenous plus oral ganciclovir. The result was that two years later, none of the babies had experienced progressive hearing loss, and two babies actually regained some hearing.

In the second study, 25 newborns were given intravenous ganciclovir for six weeks. When the babies were checked at six months old, they did not have further hearing loss. However, when the babies were at least a year old, five of them had developed progressive hearing loss. The study's researchers wondered if a longer treatment period than six weeks would have prevented those five babies from having more hearing loss.

Treatment of Progressive Hearing Loss

Children with progressive hearing loss may be treated with hearing aids or cochlear implants.

How effective are cochlear implants as a treatment? A British study was done of seven children who had progressive hearing loss and used hearing aids. Three of the children received cochlear implants as young children, and the other four got them as teenagers. Some of the children had had hearing loss for years before experiencing sudden further hearing loss, while others had been born with hearing loss that progressed to profound deafness, resulting in needing implants. After being implanted, all seven youngsters had good results. This study illustrated the positive potential for children who had been hearing aid users with well-developed auditory skills, to do very well with cochlear implants.



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Updated by Melissa Karp, Au.D.

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