Progressive Hearing Loss in Children

A baby boy wearing a hearing aid

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Progressive hearing loss in children can manifest with vague symptoms, such as behavioral issues or speech problems. There are many causes of childhood hearing loss, including infections, certain medications, and neurological disorders.

If you suspect your child is losing their sense of hearing, discuss your concerns with their pediatrician. Hearing tests can determine whether your child's hearing really is diminishing and if so, other tests can be done to determine why so the appropriate treatment can be started as soon as possible. This may involve strategies aimed at improving hearing (if possible) and speech, as well as management of the underlying cause of the progressive hearing loss.

Symptoms

Progressive hearing loss is hearing loss that worsens over time. Most people are not able to detect subtle changes in how well they can hear, including young children who may also not be able to verbalize what they're experiencing.

As a parent, it's helpful to know the symptoms of progressive hearing loss in children so that you're able to recognize them should they occur. These include:

  • Not responding when spoken to, especially when a child cannot see the person speaking
  • Not reacting to noises
  • Raising the volume on the TV or a handheld device
  • Missing instructions in school
  • Behavioral or relationship difficulties
  • Frustration or agitation
  • A limited vocabulary for their age
  • An unusual speech and/or language pattern
  • Learning challenges
  • Complaining of a hearing deficit or ringing in the ears
  • Dizziness
  • Ear or head pain

There are a number of possible explanations for these symptoms and hearing loss is just one of them. Children who experience and display any of them may have hearing loss along with another problem (such as an infection) or may not have hearing loss at all.

Causes and Risk Factors

There are a variety of causes of progressive childhood hearing loss, including infections, congenital malformations, neurological disorders, tumors, toxins, medications, trauma, and nerve damage. Your child may have a genetic predisposition to hearing loss as well.

Babies who are born prematurely or at a low birth weight are at an increased risk of developing progressive hearing loss. And if a mother has certain infections during pregnancy, that too can result in a child's progressive hearing loss.

Childhood hearing loss can be progressive because any damage to the structures that control hearing can worsen over time. Furthermore, as a child's hearing is developing, an impairment can prevent normal auditory development from occurring as it should.

Genetic and Developmental Conditions

Hereditary factors can lead to progressive hearing loss during childhood. For example, mutations of the connexin 26 gene and the PRPS1 gene have been associated with hearing loss.

Genetic conditions such as Pendred syndrome, Alport syndrome, Turner syndrome, and Usher syndrome are linked with progressive childhood hearing loss. Congenital (at birth) problems such as Mondini syndrome, a malformation of the inner ear, can also result in a progressive hearing loss.

Infections

Babies may be born with infections due to maternal transmission. Several infections, such as toxoplasmosis, syphilis, and Zika virus, can cause hearing loss that begins during the newborn years and can progress throughout childhood.

Some childhood infections, such as cytomegalovirus virus (CMV), rubella, and varicella (the virus that causes chickenpox), can cause hearing loss as well.

Ear and Nerve Damage

Injuries to the ear or the nerves that control hearing can cause progressive hearing loss at any age.

Trauma, brain tumors, and nerve disease can also interfere with the normal process by which the brain detects and recognizes sounds.

Furthermore, certain medications can harm the nerves that control hearing. Known as ototoxic medications, examples include aspirin, chemotherapeutic agents, and a powerful antibiotic called gentamycin.

Diagnosis

The Joint Committee on Infant Hearing recommends that children who have any known risk factors for progressive hearing loss should have their hearing tested before age 3, even if a newborn hearing screening (given to most babies before they leave the hospital) is normal. This is because a baby can have an undetectable mild hearing loss at birth that can deteriorate thereafter.

If you or your child have noticed or suspect a hearing loss, your child's pediatrician will need to take a thorough medical history and do a physical examination, including an ear exam and basic hearing tests, and may refer your child to an audiologist for more specialized tests. If your child has known risk factors for progressive hearing loss, your doctor may recommend screenings every few months.

The type of pediatric hearing tests performed depends on the child’s age, health, and development. The following tests may be performed to evaluate your child's hearing:

  • Behavioral hearing tests: In infants and toddlers, an audiologist observes a child’s response to different sounds and frequencies to see if they respond by moving their eyes or turning their heads. In older children, behavioral hearing tests may include games in which the child moves in response to a sound or raises a hand.
  • Auditory Brainstem Response (ABR) Tests: ABR checks hearing by measuring nerve response to sound using tiny earphones and electrodes. This test can be performed at any age, but the child needs to be still for the test. Young infants may sleep through the test and older cooperative kids sit still for the test while visually occupied. Babies over 6 months and very young children who aren’t developmentally able to sit still will need to be sedated for the test. 
  • Auditory Steady-State Response (ASSR) Test: Sometimes performed along with ABR, this test is used to determine the level of hearing loss. The ASSR test is usually performed under sedation. 
  • Central Auditory Evoked Potential (CAEP) Test: Used to check whether the pathways from the brainstem to the auditory cortex are working properly, this test also uses tiny earphones and small electrodes. 
  • Otoacoustic Emissions (OAE) Test: A tiny probe is used to record pulsing sound echoes in the ear canal to test whether outer hair cells in the inner ear are working properly. This is a very quick test that is often used in hospitals to screen newborns. 
  • Tympanometry: This procedure tests the movement of the eardrum and is helpful in identifying middle ear problems, such as fluid collecting behind the eardrum. This is done along with a visual examination of the eardrum. 
  • Middle Ear Muscle Reflex (MEMR): In this test, a soft rubber tip is placed in the ear canal and a series of loud sounds are sent through to check a reflex that protects the ear against loud sounds. This test is sometimes done while a child is sleeping.

Supplementary Tests

Your child may need additional diagnostic tests to identify the cause of their progressive hearing loss. These will be tailored based on their medical history and physical examination. For example, if your child has a rash, fever, or stiff neck, blood tests or a lumbar puncture may be done to identify an infection.

When there is a family history of progressive childhood hearing loss, genetic tests may be helpful. And if the inner ear looks abnormal, your child may need imaging tests such as computerized tomography (CT) or magnetic resonance imaging (MRI).

Treatment

Children with progressive hearing loss are typically treated by a team of specialists including an audiologist who addresses hearing issues and a speech-language pathologist who works with the child to improve communication skills, speech, and articulation.

Some children with hearing loss can use assisted listening devices, small or handheld personal speakers that increase the volume in small group conversations, or inner-ear hearing aids that improve hearing while they are worn.

Sometimes, a procedure such as a cochlear implant can help improve a child's hearing function and prevent it from worsening. And in some instances, such as when a child has a tumor, removal of the tumor may improve hearing.

Similarly, some anatomical defects can be corrected surgically, and this may prevent the progression of hearing loss or even improve hearing.

If your child has an infection, antimicrobial treatment is typically needed. While it might not improve hearing, eradicating an infection can prevent hearing loss from progressing in some cases.

If a pregnant woman is diagnosed with an infection, the mother and the baby may be treated with antimicrobials to prevent harm to the baby.

A Word From Verywell

If your child is diagnosed with progressive hearing loss, surround them with a strong team of doctors and therapists, and take advantage of any support services or mentoring programs available for your family. While the path ahead may not be what you envisioned for your child, partnering with experienced parents and experts can help guide your way forward.

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