CHARGE syndrome: CHARGE ear, hearing loss and treatments

This page covers hearing loss resulting from CHARGE ear and relevant treatments.

It includes a brief overview of the parts of the ear affected, the features of CHARGE ear, tests you can expect your child to have, and hearing aids and cochlear implants.

You’ll also find links to more detailed information on some of the tests and symptoms included here.

On this page:

CHARGE ear

Parts of the ear

CHARGE syndrome can affect various parts of the ear, including:

  • Pinna – the outer ear.
  • Earlobe or lobe – the soft, fleshy part of the outer ear.
  • Helix – the outer rim of the ear.
  • Eardrum – a thin piece of skin-like tissue that separates the outer ear from the middle ear.
  • The middle ear – the part connected to the back of the nose and throat.
  • Ossicles – three small bones in the middle ear that send sound from the eardrum to the inner ear.
  • Inner ear – the part mainly responsible for detecting sound and maintaining balance.
  • Eustachian tube – a small passageway connecting the middle ear to the throat.
  • Cochlea – hollow, spiral-shaped bone in the inner ear.
  • Cochlear nerve, also known as the auditory nerve – responsible for sending sound to the brain.
  • Semicircular canals – three tiny, fluid-filled tubes in your inner ear that help you keep your balance.

Features of CHARGE ear

Features of CHARGE ear include:

  • The pinna may be an unusual shape, with small lobes and a misshapen helix.
  • Middle ear problems. The ossicles may be incomplete or stuck together.
  • Regular ear infections or “glue ear” due to poor ventilation of the middle ear because the eustachian tube doesn’t work properly.
  • The semicircular canals may be missing completely, affecting balance. This is a key feature for a diagnosis of CHARGE syndrome.
  • The cochlea may be incomplete (cochlear dysplasia).
  • The cochlea may have a single cavity (space), instead of the usual spiral shape. (Mondini deformity).
  • The cochlear nerve may be thin (hypoplastic) or missing completely (cochlear nerve aplasia).

What tests should I expect my child to have?

Scans

If your child has been diagnosed with CHARGE syndrome, or it’s thought they have hearing loss, they should have:

An MRI scan is particularly important if it’s thought your child has profound hearing loss (total lack of hearing) or that their auditory nerve is thin or missing. 

Audiometric testing 

If your child has a misshapen middle ear, they may have moderate hearing loss, which means they can’t hear sounds below 50 decibels (dB).

If they also have a misshapen cochlea, they may have inner-ear hear hearing loss (sensorineural hearing loss). 

As a result of this damage to the middle ear and the inner ear, they may have mixed hearing loss – ranging from moderate to profound hearing loss.

They should then have an audiometric test.

Audiometric testing is a type of hearing test that measures how someone hears different sounds, pitches or frequencies.

Audiometric testing should take into account:

  • Your child’s age.
  • Their developmental age – how well are they developed physically and mentally for their age.
  • Any other sensory difficulties they may have.
  • Any motor difficulties – anything that affects their ability to move.

A number of audiometric tests may be needed over a period of time, as well as comments from parents and/or carers about what they’ve noticed in the child’s behaviour.

To get the most accurate result, your child may have an auditory brainstem response (ABR) test under general anaesthetic.

Where possible, the results of an ABR test will be confirmed by behavioural tests – listening games that test how your fully awake child responds to sounds. 

Hearing aids

The results of an ABR test can help to decide whether your child should have hearing aids and which type.

If your child’s hearing loss is due to a misshapen middle ear, or they have moderate to severe mixed hearing loss, the type of hearing aid will depend on which features of CHARGE ear they have.

Bone-anchored hearing aid

If the pinna is severely misshapen, or there are chronic middle ear problems, it may be difficult to fit standard air-conduction hearing aids. 

So, your child may be fitted with bone conduction hearing aids, which send sound directly to the inner ear through the bones of their skull. 

Increasingly, a bone-anchored hearing aid (BAHA) is offered. Usually, a soft-band version is tried first followed later by a device implanted surgically, if appropriate.

Read more about the different types of hearing aids.

Cochlear implants

Cochlear implants use electrodes to stimulate nerve endings in the cochlea to send sound to the brain along the cochlear nerve.

Cochlear implants are only recommended for children with severe hearing loss.

Children with CHARGE syndrome have to be assessed very carefully to see if cochlear implants are right for them.

You should be aware, from the start of an assessment, that cochlear implants are not right for your child.

If it is thought that your child’s auditory nerve is missing, electrical auditory brainstem testing (eABR) may be used to decide whether cochlear implants are right for them.

Read more about cochlear implants.

Risks and follow-up

With cochlear implants, there is a risk that the facial nerve could be damaged during surgery, possibly permanently.

Your child’s surgeon should discuss this with you before surgery.

For children with CHARGE syndrome, follow-up after surgery may be more intensive and time consuming than it would be for other children.

Outcomes

If your child has a misshapen cochlea or a thin cochlear nerve, cochlear implants may not be as effective as you might expect.

This is because the smaller number of nerve impulses reduces the amount of sound that can be sent to the brain.

With cochlear implants, your child may develop awareness of sound, including speech, but to the same level as children who do not have CHARGE ear.

Also, if your child has other sensory difficulties, this will also have an impact on the effectiveness of cochlear implants.

That said, many parents have found that cochlear implants can help their child to feel more connected to the world, to have greater awareness of life around them, and be more willing to communicate.

Get support from Sense

We’re here for people with CHARGE syndrome and their families.

This content was last reviewed in April 2022. We’ll review it again next year.