What is Auditory Neuropathy?

Have you ever had your vehicle break down in the middle of the road? It’s not an enjoyable experience. You have to pull your car safely to the side of the road. And then, for whatever reason, you probably pop your hood and have a look at your engine.

What’s funny is that you do this even though you have no idea how engines work. Perhaps you think there’ll be a handy knob you can turn or something. Eventually, you have to call someone to tow your car to a mechanic.

And it’s only when the professionals check out things that you get an understanding of the issue. Just because the car isn’t moving, doesn’t mean you can tell what’s wrong with it because vehicles are complex and computerized machines.

With hearing loss, this same type of thing can occur. The cause is not always obvious by the symptoms. Sure, noise-related hearing loss is the common culprit. But sometimes, it’s something else, something such as auditory neuropathy.

What is auditory neuropathy?

When most people consider hearing loss, they think of noisy concerts and jet engines, excessive noise that damages your hearing. This kind of hearing loss is called sensorineural hearing loss, and it’s somewhat more involved than simple noise damage.

But in some cases, long-term hearing loss can be the result of something else besides noise damage. A condition called auditory neuropathy, while less prevalent, can in some cases be the cause. When sound can’t, for whatever reason, be effectively transmitted to your brain even though your ear is receiving that sound just fine.

Auditory neuropathy symptoms

The symptoms of traditional noise related hearing loss can often look very much like those of auditory neuropathy. You can’t hear well in loud situations, you keep turning the volume up on your television and other devices, that sort of thing. That’s why diagnosing auditory neuropathy can be so difficult.

Auditory neuropathy, however, has some distinctive symptoms that make recognizing it easier. When hearing loss symptoms manifest like this, you can be fairly certain that it’s not standard noise related hearing loss. Though, as always, you’ll be better informed by an official diagnosis from us.

Here are a few of the more unique symptoms of auditory neuropathy:

• Sounds sound jumbled or confused: This is, once again, not an issue with volume. The volume of what you’re hearing is completely normal, the issue is that the sounds seem jumbled and you can’t make sense of them. This can go beyond the speech and pertain to all types of sounds around you.
• An inability to make out words: Sometimes, you can’t make out what someone is saying even though the volume is normal. Words are confused and unclear.
• Sound fades in and out: The volume of sound seems to go up and down like somebody is playing with the volume knob. This could be a sign that you’re experiencing auditory neuropathy.

What causes auditory neuropathy?

These symptoms can be articulated, in part, by the root causes behind this specific disorder. It might not be very clear why you have developed auditory neuropathy on an individual level. Both children and adults can experience this disorder. And there are a couple of well described possible causes, generally speaking:

• Damage to the cilia that send signals to the brain: If these little hairs inside of your inner ear become compromised in a particular way, the sound your ear detects can’t really be passed on to your brain, at least, not in its complete form.
• Nerve damage: There’s a nerve that transmits sound signals from your inner ear to the hearing portion of your brain. If this nerve gets damaged, your brain can’t receive the full signal, and consequently, the sounds it “interprets” will seem off. Sounds may seem garbled or too quiet to hear when this happens.

Risk factors of auditory neuropathy

Some individuals will develop auditory neuropathy while others won’t and no one is really sure why. As a result, there isn’t a definitive way to prevent auditory neuropathy. But you might be at a higher risk of developing auditory neuropathy if you show specific close associations.

It should be mentioned that these risk factors aren’t guarantees, you might have every single one of these risk factors and still not develop auditory neuropathy. But the more risk factors present, the higher your statistical probability of experiencing this disorder.

Risk factors for children

Here are a few risk factors that will increase the likelihood of auditory neuropathy in children:

• Other neurological conditions
• Preterm or premature birth
• A lack of oxygen during birth or before labor begins
• A low birth weight
• Liver disorders that lead to jaundice (a yellow appearance to the skin)
• An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)

Risk factors for adults

Here are some auditory neuropathy risk factors for adults:

• auditory neuropathy and other hearing conditions that run in the family
• Immune diseases of various kinds
• Some medications (specifically incorrect use of medications that can cause hearing issues)
• Mumps and other distinct infectious diseases

In general, it’s a good plan to limit these risks as much as you can. If risk factors are there, it may be a good idea to schedule regular screenings with us.

How is auditory neuropathy diagnosed?

During a normal hearing assessment, you’ll most likely be given a set of headphones and be told to raise your hand when you hear a tone. When you’re dealing with auditory neuropathy, that test will be of extremely limited use.

One of the following two tests will typically be used instead:

• Otoacoustic emissions (OAE) test: The response of your inner ear and cochlea to stimuli will be tested with this diagnostic. A little microphone is placed just inside your ear canal. Then, we will play a series of tones and clicks. Then your inner ear will be assessed to see how it responds. The data will help determine whether the inner ear is the problem.
• Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have specialized electrodes attached to certain places on your head and scalp. This test isn’t painful or uncomfortable in any way so don’t be concerned. These electrodes measure your brainwaves, with particular attention to how those brainwaves respond to sound. Whether you’re experiencing sensorineural hearing loss (outer ear) or auditory neuropathy (inner ear) will be determined by the quality of your brainwaves.

Diagnosing your auditory neuropathy will be much more effective once we run the appropriate tests.

Is there treatment for auditory neuropathy?

So, in the same way as you bring your car to the mechanic to get it fixed, you can bring your ears to us for treatment! Auditory neuropathy generally has no cure. But this condition can be treated in a few possible ways.

• Hearing aids: In some moderate cases, hearing aids will be able to provide the necessary sound amplification to help you hear better, even with auditory neuropathy. Hearing aids will be an adequate option for some people. But because volume isn’t usually the issue, this isn’t usually the situation. As a result, hearing aids are usually coupled with other therapy and treatment options.
• Cochlear implant: Hearing aids won’t be able to solve the problem for most individuals. In these cases, a cochlear implant might be necessary. This implant, basically, takes the signals from your inner ear and conveys them directly to your brain. They’re pretty amazing! (And you can watch many YouTube videos of them working for patients.)
• Frequency modulation: In some cases, it’s possible to hear better by boosting or reducing certain frequencies. That’s what happens with a technology known as frequency modulation. Basically, highly customized hearing aids are utilized in this approach.
• Communication skills training: Communication skills training can be put together with any combination of these treatments if needed. This will allow you to work with whatever level of hearing you have to communicate better.

It’s best to get treatment as soon as you can

Getting your condition treated promptly will, as with any hearing condition, produce better outcomes.

So if you think you have auditory neuropathy, or even just regular old hearing loss, it’s important to get treatment as soon as possible. You’ll be able to go back to hearing better and enjoying your life after you schedule an appointment and get treated. This can be extremely critical for children, who experience a great deal of cognitive development and linguistic expansion during their early years.