Hearing loss can be a disorientating and difficult symptom for those coping with a leukodystrophy. It can also be upsetting for family and friends, who find it increasingly difficult to communicate with their loved one. The leukodystrophies affected by hearing loss are primarily childhood-onset conditions, meaning that in many cases the child will either fail to develop hearing or else quickly lose it.
Newborns will generally respond to noise, either turning their head to noises or startling at sudden loud noises. Hearing sounds is crucial in developing language skills: babies aged around 3-4 months begin to vocalise vowels and consonants and to recognise different voices.
Children with progressive conditions such as leukodystrophies may not respond to noise or may lose this response. While this could indicate hearing loss, it may also be due to mobility difficulties or general low responsiveness. If you are concerned, ask your doctor to refer your child for a hearing evaluation. If hearing difficulties are identified, they should be treated as soon as possible.
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Sensorineural hearing loss, also called labyrinthine hearing loss is caused by problems with the hair cells in the ear which sense sound, or by neural dysfunction. The severity of this varies from complete deafness to mild comprehension difficulties, with some also experiencing ringing or buzzing in the ears (tinnitus).
Cortical hearing loss is loss of hearing due to damage to the auditory cortex of the brain. As there is no damage to the ear, hearing aids will not have an effect on this form of hearing loss.
Different kinds of hearing aids and implants are available depending on what part of the ear is affected. These can restore some hearing, depending on the type or severity of hearing loss. Talk to your doctor and hearing impairment service to find out if these are appropriate: please be aware that some of those with hearing loss caused by leukodystrophy will not benefit from the use of hearing aids or implants.
For those with severe hearing loss, communication can be difficult. Sign language and lip reading are common methods used to help with this, talk to your GP and hearing impairment service to find out more about how to learn and use these methods.