How to Manage Auditory Neuropathy in Children - Attune
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How to Manage Auditory Neuropathy in Children

Auditory Neuropathy in Children

Has your child complained about their ears recently? Are they finding it hard to hear or can’t understand speech in everyday environments? 

They might have ANSD. 

Auditory Neuropathy Spectrum Disorder (ANSD), also known as Auditory Neuropathy, is hearing loss caused by abnormal auditory nerve function. 

Individuals who suffer from ANSD have a normal cochlea or inner ear function but experience hearing loss from the abnormal transmission of sound along the auditory pathway. 

To put it simply, Auditory Neuropathy means there’s a problem with sending sound from the ear to the brain. When this happens, it can lead to hearing loss and distortion of sound. 

Ten per cent of children with permanent hearing deficits show evidence of ANSD. 

In today’s article, we will discuss the symptoms of ANSD, what causes it and how to manage it. 

How do we Hear? 

Usually, sound enters the ear via the ear canal, which vibrates the eardrum and moves the tiny bones in the middle ear cavity. The vibrations are then sent to the cochlea, stimulating the hair cells. 

The signals from the hair cells are then transmitted along the auditory nerve, where they are sent to the brain to make sense of the sound.

However, if an individual has ANSD, the sounds reach the cochlea normally, but the transmission to the brain along the hearing nerve is damaged. 

Symptoms of auditory neuropathy 

ANSD can affect one or both ears, and hearing loss can vary from mild to profound. 

Although symptoms vary from person to person, the most common can be found below: 

  • Mild to profound hearing loss
  • Sounds that fade in and out
  • Difficulty understanding words
  • Poor speech perception in regular and noisy environments

A diagnostic hearing test can reveal if your child has ANSD or not. 

Auditory Neuropathy

What Causes Auditory Neuropathy?

In babies, Auditory Neuropathy may occur in association with conditions such as extreme prematurity, hyperbilirubinemia and hydrocephalus, or it may be present in infants without obvious underlying symptoms

ANSD in adults may develop alongside age-related hearing loss. Some ANSD is related to neurological disorders that also cause problems outside of the hearing system. 

Possible causes of Auditory Neuropathy include

  • Reduced oxygen supply in utero
  • Premature birth
  • Damage to the connections between the hair cells and cochlea nerve 
  • Damage to the eighth cranial nerve 
  • Damage to the auditory pathways in the brain stem 
  • Neurological disorders such as Charcot-Marie-Tooth disorder and Friederich’s Ataxia 

How is ANSD Diagnosed? 

Electrophysiological hearing tests 

Diagnosing ANSD is complex and requires expertise in analysing electrophysiological hearing tests. 

An auditory brainstem response (ASR) test assesses the auditory nerve function and can accurately diagnose a hearing loss in babies as young as a day old. 

Otoacoustic emissions test 

Another test used to determine whether hearing loss is sensorineural or due to ANSD is the otoacoustic emissions (OAE) test. 

An OAE is a low-level sound emitted by the cochlea related to the function of the outer hair cells. 

If an individual has profound sensorineural hearing loss, OAEs are absent due to the poor function or absence of the hair cells. In some cases of ANSD, OAEs will be present but have been found to be absent in 30 per cent of individuals with ANSD

Auditory Neuropathy Diagnosed

When is Auditory Neuropathy Diagnosed? 

In Australia, all babies have a hearing screening assessment at birth. 

Babies with ANSD are not expected to pass this assessment as it’s an automated ABR screening test. The baby may be referred for further diagnostic hearing assessments with a paediatric audiologist following the test. 

The audiologist will then perform a range of tests to discover the type and severity of hearing loss. They will often repeat the assessment a month later to confirm the diagnosis. 

Later onset ANSD can also be diagnosed in older children or adults when hearing difficulties are reported with or without hearing loss. Hearing aids are not often helpful, and sounds are reported as distorted or unclear. A definitive diagnosis is made with ABR testing. 

How to Manage Auditory Neuropathy 

Once an individual has been diagnosed with ANSD, further investigation is recommended. This will often be undertaken by an Ear, Nose and Throat Specialist, a paediatrician and sometimes a geneticist. 

The child will also be referred for a hearing aid or assistive listening device fitting. This will be trial and error as some children with ANSD don’t benefit from hearing aids. If hearing aids aren’t beneficial, children may be referred for a cochlear implant.

Often the guidance of a speech pathologist combined with behavioural hearing assessments can determine the level of amplification required. And so, regular hearing assessments will be necessary along the way to monitor and evaluate their process. 

If you have concerns about your child’s hearing, book them in for a hearing test. Untreated hearing problems in children can slow down their development, so don’t waste time; book an appointment today on our website or call us on 1300 736 702. 

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