KidsMD Health Topics

Sensorineural Hearing Loss

  • Overview

    Sensorineural hearing loss is an extremely common type of hearing loss. This type of hearing loss affects the inner ear.

    How Children's approaches sensorineural hearing loss:

    Our Audiology program will perform the following tests:

    Additionally, our team will:

    • monitor the hearing loss
    • provide counseling to you and your family regarding the hearing loss
    • recommend hearing aids and ways to maximize the use of residual hearing
    • refer your child for additional language or developmental assessments
    • direct you to community resources for educational or financial assistance
    • refer your child to Habilitative Audiology to learn about the option of a cochlear implant if your child has a severe to profound hearing loss in both ears


    Hearing Loss Experience Journal

    The Hearing Loss Experience Journal is a collection of stories, pictures, and personal experiences contributed by children, teens, and young adults who are deaf or hard of hearing and their families. It represents the “collective wisdom” of these families as well as their health care providers. Read the journal here.

    Children's Hospital Boston
    300 Longwood Avenue
    Boston MA 02115


  • In-Depth

    This type of hearing loss can happen at birth (congenital), or become acquired over time.



    • loud noise exposure
    • trauma
    • infections
    • damage from certain medications that can be harmful to the ears
  • Tests

    Why should my child have a hearing test?

    An infant should have a hearing test if he or she did not pass the newborn hearing screening test in one or both ears. Even if your baby passed the newborn hearing screening test, your baby's hearing should be tested again if there is a reason why your baby might develop a hearing loss.

    Reasons your child might develop a hearing loss:

    • infections at birth such as cytomegalovirus (CMV)
    • being on a ventilator for a long time
    • having congenital diaphragmatic hernia or needing ECMO therapy
    • having repeated courses of IV antibiotics and diuretics
    • having a syndrome in which hearing loss often occurs
    • having a close relative who had hearing loss in childhood

    Your child's hearing also should be checked if:

    • She is not starting to understand words and to talk at the usual age.
    • She has frequent ear infections or persistent middle ear fluid.
    • You think your child is not responding normally to sounds.

    Your doctor can help to determine whether your child's hearing should be tested again. All children should have a hearing screening test in preschool and/or before starting kindergarten.

  • Some hearing problems are medically or surgically correctable if they are caused by middle ear fluid. Other hearing problems are treated with the use of hearing aids and speech and language therapy. If a hearing problem is found, your audiologist and pediatrician will arrange for your child to be seen by an otolaryngologist (ear, nose, and throat specialist) and will guide you to other services to help your child learn to communicate.

  • Research & Innovation

    Most cases of deafness are caused by the dysfunction or death of cells in the cochlea, the snail-shell-shaped structure in the inner ear. The laboratory of Douglas Cotanche, PhD, formerly a researcher in Children's Department of Otolaryngology, was able to grow all the assorted cell types in the cochlea from just one source: neural stem cells.

    Neural stem cells were first isolated from mice in 1998 by Evan Snyder, MD, PhD, formerly of Children's Department of Neurology. Cotanche's team implanted the cells deep inside the sound-damaged cochleas of guinea pigs and mice. Six weeks later, the cells had migrated throughout the cochlea and formed satellite cells, spiral ganglion cells and Schwann cells, which make up the cochlea's nervous tissue, as well as the hair cells and supporting cells of the organ of Corti (the actual hearing organ). 

    The researchers couldn't show complete rebuilding of the cochlea, but they believe that with more time and more stem cells, most of the cochlea could be repopulated. The next step is to implant human neural stem cells in animals and test whether the new cochlear cells connect with the auditory nerve and the brain, and whether they respond to sound stimulation—in other words, whether they restore hearing.

    Gene-therapy trial will attempt to restore hearing in deaf mice

    Ear note

    In the Journal of Clinical Investigation, a team co-led by Jeffrey Holt, PhD, of the Department of Otolaryngology at Children’s Hospital Boston demonstrated that two related proteins, TMC1 and TMC2, are essential for normal hearing – paving the way for a test of gene therapy to reverse a type of genetic deafness. 

    “This is the first time anything like this has been done,” says Holt.

    Learn more.

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