Tips for Dealing with Hearing Loss in Adrenoleukodystrophy

Adrenoleukodystrophy (ALD) is a severe heritable disorder that primarily affects males. Symptoms can appear early in life. The tissues most affected are nerves and adrenal glands. One symptom that some ALD patients experience is hearing loss.

ALD is caused by mutations in a gene called ABCD1 that provides instructions for making an enzyme involved in processing very-long-chain fatty acids (VLCFAs). Without the ABCD1 enzyme, VLCFAs build up in cells and tissues. It’s not clear how exactly VLCFAs are toxic to cells and tissues.

ALD and hearing loss

Because the disease is progressive, symptoms such as hearing loss can appear at any time and may worsen over time.

The reason for hearing loss in ALD patients is not clearly understood. It could be due to malformations of the machinery involved in hearing (the bones and structure of the ear). However, ALD also may affect the nerves that transmit sound signals from the ear to the brain due to the buildup of VLCFAs.

How to recognize hearing loss?

In children, hearing loss often is mistaken for lack of attention. You may find that your child’s grades are slipping. Often, children don’t realize they aren’t hearing portions of a conversation they should be able to hear. They may become frustrated when there is background noise. Hearing loss in children can profoundly affect language development, so it’s important to keep an eye out for indications that your child may not be able to hear as well as he or she should.

How is it diagnosed?

If you suspect that your child has hearing loss, it’s important to undergo a hearing test as soon as possible. The doctor will examine your child and may use instruments such as tuning forks to determine if there are problems.

Another important test for children with ALD is an audiometry exam, performed by an audiologist. The audiologist will have your child wear a headset and will test the range of hearing (the specific frequencies of sounds that he or she is able to hear in each ear). The doctor also may perform a speech audiometry exam, which tests your child’s ability to recognize spoken words in each ear. Children with ALD may have difficulty recognizing spoken words in addition to hearing loss. Although the exact cause is not known, research has suggested that this is a feature of hearing loss in ALD.

How is it treated?

Hearing loss often can be treated with hearing aids. These are devices that amplify sounds, making them easier for the ear to transmit to the brain. There are many different types and styles of hearing aids. Your doctor will help you figure out which type is best for your child’s needs.

Part of the way we learn spoken language is by hearing, so for children who have difficulty hearing, speech also can be affected. Speech therapy may be helpful for children with hearing loss.

Last updated: April 15, 2020

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Adrenoleukodystrophy News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.