Have you ever been in the middle of the road and your car breaks down? It’s not a fun experience. Your car has to be safely pulled off the road. Then you probably pop your hood and take a look at the engine. Who knows why?

What’s strange is that you do this even if you have no idea how engines work. Maybe whatever is wrong will be obvious. Eventually, you have to call somebody to tow your car to a mechanic.

And it’s only when the experts check out things that you get a picture of the problem. Just because the car is not moving, doesn’t mean you can know what’s wrong with it because cars are complicated and computerized machines.

With hearing loss, this same kind of thing can occur. The cause isn’t always apparent by the symptoms. There’s the normal cause (noise-associated hearing loss), sure. But in some cases, something else like auditory neuropathy is the cause.

Auditory neuropathy, what is it?

When most individuals think about hearing loss, they think of loud concerts and jet engines, excessive noise that harms your ability to hear. This kind of hearing loss, called sensorineural hearing loss is a bit more complex than that, but you get the point.

But in some cases, long-term hearing loss can be the result of something else besides noise damage. A condition known as auditory neuropathy, while less prevalent, can sometimes be the cause. This is a hearing condition where your ear and inner ear collect sounds just fine, but for some reason, can’t fully transfer those sounds to your brain.

Symptoms of auditory neuropathy

The symptoms associated with auditory neuropathy are, at first glimpse, not all that distinct from those symptoms linked to conventional hearing loss. You can’t hear very well in noisy situations, you keep cranking the volume up on your television and other devices, that sort of thing. That’s why diagnosing auditory neuropathy can be so difficult.

Still, auditory neuropathy does have a few unique features that make it possible to identify. These presentations are pretty strong indicators that you aren’t confronting sensorineural hearing loss, but auditory neuropathy instead. Of course, nothing can replace getting a real-time diagnosis from us about your hearing loss.

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

• Sound fades in and out: The volume of sound seems to rise and fall like someone is playing with the volume knob. If you’re experiencing these symptoms it might be a case of auditory neuropathy.
• Sounds sound jumbled or confused: This is, once again, not a problem with volume. You can hear sounds but you just can’t make sense of them. This can go beyond the speech and apply to all types of sounds around you.
• Trouble understanding speech: Sometimes, the volume of a word is just fine, but you just can’t understand what’s being said. The words sound mumbled or distorted.

Some triggers of auditory neuropathy

The root causes of this condition can, in part, be explained by the symptoms. On a personal level, the reasons why you might experience auditory neuropathy may not be completely clear. Both adults and children can develop this disorder. And, broadly speaking, there are a couple of well defined possible causes:

• Damage to the nerves: There’s a nerve that carries sound signals from your inner ear to the hearing portion of your brain. The sounds that the brain attempts to “interpret” will seem unclear if there is damage to this nerve. Sounds might seem garbled or too quiet to hear when this occurs.
• The cilia that send signals to the brain can be compromised: If these tiny hairs in your inner ear become compromised in a specific way, the sound your ear senses can’t really be passed on to your brain, at least, not in its complete form.

Auditory neuropathy risk factors

Some people will develop auditory neuropathy while other people won’t and no one is quite certain why. That’s why there isn’t an exact science to combating it. Still, there are close associations which might show that you’re at a higher risk of developing this condition.

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

Children’s risk factors

Here are some risk factors that will raise the likelihood of auditory neuropathy in children:

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

Adult risk factors

Here are a few auditory neuropathy risk factors for adults:

• Mumps and other specific infectious diseases
• Family history of hearing conditions, including auditory neuropathy
• Various kinds of immune disorders
• Certain medications (especially improper use of medications that can cause hearing issues)

In general, it’s a good idea to minimize these risks as much as possible. If risk factors are there, it might be a good plan to schedule regular screenings with us.

Diagnosing auditory neuropathy

During a normal hearing examination, you’ll most likely be given a pair of headphones and be asked to raise your hand when you hear a tone. When you have auditory neuropathy, that test will be of very limited use.

Rather, we will usually recommend one of two tests:

• Auditory brainstem response (ABR) test: During this diagnostic test, you’ll have special electrodes attached to specific places on your scalp and head. This test isn’t painful or uncomfortable in any way so don’t worry. These electrodes track 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.
• Otoacoustic emissions (OAE) test: This diagnostic is made to determine how well your inner ear and cochlea react to sound stimuli. A little microphone is put just inside your ear canal. Then, we will play a series of tones and clicks. The diagnostic device will then evaluate how well your inner ear responds to those tones and clicks. If the inner ear is an issue, this data will reveal it.

Once we run the appropriate tests, we will be able to more successfully diagnose and treat your auditory neuropathy.

Does auditory neuropathy have any treatments?

So, just like 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 there are a few ways to manage this condition.

• Hearing aids: Even with auditory neuropathy, in milder cases, hearing aids can amplify sound enough to enable you to hear better. Hearing aids will be an adequate solution for some individuals. But because volume usually isn’t the issue, this isn’t normally the case. Due to this, hearing aids are often combined with other therapy and treatment options.
• Cochlear implant: Hearing aids won’t be able to solve the issue for most individuals. It may be necessary to go with cochlear implants in these cases. This implant, basically, takes the signals from your inner ear and conveys them directly to your brain. They’re rather amazing! (And you can watch many YouTube videos of them working for patients.)
• Frequency modulation: In some cases, amplification or reduction of specific frequencies can help you hear better. That’s what happens with a technology known as frequency modulation. Basically, highly customized hearing aids are used in this strategy.
• Communication skills training: In some situations, any and all of these treatments might be combined with communication skills training. This will let you work with whatever level of hearing you have to communicate better.

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

As with any hearing disorder, timely treatment can produce better results.

So if you think you have auditory neuropathy, or even just ordinary hearing loss, it’s important to get treatment as quickly as possible. You’ll be able to go back to hearing better and enjoying your life once you schedule an appointment and get treated. This can be especially crucial for children, who experience a lot of cognitive development and linguistic growth during their early years.

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