Autosomal Hearing Loss

by Andrew Mills

The condition in which there is hearing loss or inability to hear right from birth is referred to as congenital hearing loss. Genetic factors play an important role in such cases. Also the factors present prenatally (in utero) could be responsible for hearing loss from birth.

Autosomal hearing loss is essentially related to chromosomes (any except the sex chromosome) and the same can be best explained as below:

  1. Autosomal dominant hearing loss: In this case, one parent carries the dominant gene for hearing loss and typically has a hearing loss passes it on to the child. In such a case, there is at least a 50% chance that the child will also have a hearing loss. The chances are higher if both the parents have the dominant gene (where both parents have a hearing loss also). This can even happen if both grandparents on one side of the family have hearing loss due to genetic causes. Autosomal dominant hearing loss is expected if the child is detected with Treacher Collins syndrome as it is a known effect of this syndrome.
  2. Autosomal recessive hearing loss: In this scenario, usually both the parents have normal hearing but one or both may be carrying a recessive gene. In such a situation, the child usually has a 25% chance of hearing loss as everybody else in the family has normal hearing. Autosomal recessive hearing loss is expected if the child is detected with Usher syndrome as it is a known effect of this particular syndrome.

Autosomal hearing loss once diagnosed and evaluated, can be managed by using an appropriate habilitation option. Use of hearing aids, vibrotactile devices and cochlear implantation are some of the possible treatment options. An experienced team consisting of an otolaryngologist, an audiologist, a clinical geneticist and a pediatrician should be able to help with treatment for autosomal hearing loss.

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