Auditory Neuropathy Causes Hearing Loss

Auditory neuropathy is a rare form of hearing loss. It is created by interfering with nerve signals that flow from the inner ear to the brain. There are no recognized causes and no known remedy.

When both ears are affected, hearing loss ranges from mild to severe. Auditory neuropathy is also referred to as neural or nerve deafness.

In certain cases, afflicted people can hear but struggle to interpret spoken words, especially in crowded places. Hearing aids or cochlear implants can improve your ability to hear and understand what you're saying.

Auditory neuropathy symptoms can vary, and as a result, a person's hearing can improve or worsen. The long-term forecast is also different.

A person's hearing, for example, may improve, degrade, or remain unchanged, and there is no way to predict the outcome.

Auditory Neuropathy Causes Hearing Loss

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Symptoms of Auditory Neuropathy

Auditory neuropathy symptoms vary and fluctuate from person to person. Symptoms may include:

varying degrees of hearing loss from mild to severe

Fade-in and fade-out sounds effects

difficulty comprehending what is being said (speech perception)

Although the hearing is adequate, speech perception is poor.

In loud environments, speech perception suffers.

Many auditory neuropathy symptoms are also caused by other types of hearing loss.

How Do We Perceive Sounds?

Hearing involves the following structures:

The outer ear is the visible part of the ear. Sounds are transmitted to the middle ear via the outer ear.

The membrane that separates the middle and outer ear is known as the acoustic membrane. The eardrum moves in reaction to sound waves. The movement is magnified by tiny bones in the middle ear.

The cochlea, a tiny spiral-shaped organ in the inner ear, detects the increased sound waves.

The vibrations are caught up by minute hair-like nerve endings in the cochlea, which transmit the message to the cochlear nerve via electrical impulses.


The impulse is carried by the cochlear nerve through the eighth cranial nerve to auditory pathways in the brainstem. The brainstem, which is positioned towards the base of the skull, is thought to be the “bridge” between the brain and the spinal cord. Electrical signals are interpreted by the brain, and sound is “heard.”

What Factors Cause Auditory Neuropathy?

Although some ear structures may normally function in auditory neuropathy, information from the ear may be distorted when it reaches the brain. The cause of auditory neuropathy is unknown; however, researchers believe that a number of factors, including damage to:

hair cells in the inner ear

the links that exist between hair cells and the cochlear nerve

the cochlear nerve

No. 8 cranial nerve (the combination of the cochlear and vestibular nerves)

auditory channels in the brain stem

Diagnosis of Auditory Neuropathy

Auditory neuropathy is diagnosed using a range of assays, including:

Auditory Brainstem Response (ABR).

When a person is exposed to a sequence of sounds, brain wave patterns are recorded using electrodes on the ears and head. Auditory neuropathy frequently results in little or no response.

Otoacoustic emissions (OAE) – a tiny microphone is placed in the ear canal to investigate the function of the cochlea's hair cells.

If they are, the microphone picks up on the slight sounds emitted by the hair cells in response to the stimuli. A person suffering from auditory neuropathy typically has some functioning hair cells.

There are other hearing tests available, such as speech recognition.

A person with auditory neuropathy often performs badly when tested with background noise.

Further Examinations

All other possible causes of the same symptoms must be ruled out. as in multiple sclerosis


Auditory neuropathy treatment possibilities

Auditory neuropathy is a fatal disease. Because of the varying nature of this hearing issue, some therapies may be effective in some cases but not in others. Unfortunately, no testing can indicate whether or not a specific treatment will be beneficial; instead, the patient and their hearing healthcare practitioner must explore to find out.

Treatment options for auditory neuropathy include:

Implantation of cochlear implants

A surgically implanted device that stimulates the nerves of the inner ear. Many patients have claimed that their ability to hear speech has improved as a result of these implants.

Systems that use FM (frequency modulation)

A wireless receiver and headphones for amplification without the usage of wires.

Hearing aids are devices that increase the volume of all sounds. Hearing aids are generally ineffective for those with auditory neuropathy.

Communication ability for persons with auditory neuropathy

The medical community is divided about how to teach children with aural neuropathy to communicate. Some advocate teaching the child sign language, while others advocate equipping the youngster with devices (such as hearing aids) and supporting them in learning to hear and speak.

Some medical experts believe that a combination of both procedures is best because it is impossible to forecast whether the child's hearing will improve, remain stable, or worsen.

If the child's hearing improves, he or she must be exposed to spoken language. Adults with past spoken language expertise may benefit from learning to employ complementary signals such as lip-reading.

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