What is Auditory Neuropathy?

Have you ever been in the middle of the road and your car breaks down? That really stinks! You have to pull your car safely to the side of the road. And then, for some reason, you probably open your hood and have a look at your engine.

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

And a picture of the issue only becomes evident when experts diagnose it. Just because the car isn’t moving, doesn’t mean you can tell 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 obvious by the symptoms. There’s the common cause (noise-associated hearing loss), sure. But sometimes, it’s something else, something such as auditory neuropathy.

What is auditory neuropathy?

Most people think of extremely loud noise like a rock concert or a jet engine when they consider hearing loss. This kind of hearing loss, known as sensorineural hearing loss is a bit more complex than that, but you get the point.

But sometimes, this sort of long-term, noise related damage isn’t the cause of hearing loss. While it’s less common, hearing loss can sometimes be caused by a condition called auditory neuropathy. 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 of traditional noise related hearing loss can often look a lot like those of auditory neuropathy. You can’t hear very well in loud situations, you keep cranking up the volume on your television and other devices, that sort of thing. This can frequently make auditory neuropathy difficult to diagnose and manage.

However, auditory neuropathy does have some unique properties that make it possible to diagnose. These presentations are pretty strong indicators that you aren’t confronting sensorineural hearing loss, but auditory neuropathy instead. Though, naturally, you’ll be better informed by an official diagnosis from us.

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

• Sound fades in and out: Maybe it feels like somebody is messing with the volume knob inside of your head! If you’re experiencing these symptoms it might be a case of auditory neuropathy.
• Sounds seem jumbled or confused: Again, this is not a problem with volume. The volume of what you’re hearing is just fine, the issue is that the sounds seem jumbled and you can’t make sense of them. This can pertain to all sorts of sounds, not just spoken words.
• An inability to make out words: Sometimes, you can’t understand what somebody is saying even though the volume is just fine. Words are unclear and unclear.

Some triggers of auditory neuropathy

The underlying causes of this condition can, in part, be explained by the symptoms. On an individual level, the reasons why you might develop auditory neuropathy might not be totally clear. This condition can develop in both adults and children. And there are a couple of well defined possible causes, generally speaking:

• Damage to the nerves: The hearing portion of your brain gets sound from a particular nerve in your ear. If this nerve becomes damaged, your brain can’t receive the full signal, and consequently, the sounds it “interprets” will sound wrong. When this takes place, you might interpret sounds as jumbled, unclear, or too quiet to discern.
• The cilia that deliver signals to the brain can be compromised: Sound can’t be passed to your brain in complete form once these little fragile hairs have been damaged in a particular way.

Auditory neuropathy risk factors

Some people will experience auditory neuropathy while other people won’t and no one is quite sure why. That’s why there’s no exact science to preventing it. Nevertheless, there are close associations which might reveal that you’re at a higher risk of experiencing this disorder.

It should be noted that these risk factors aren’t guarantees, you could have every single one of these risk factors and not experience auditory neuropathy. But the more risk factors shown, the higher your statistical probability of developing this condition.

Risk factors for children

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

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

Adult risk factors

Here are some auditory neuropathy risk factors for adults:

• auditory neuropathy and other hearing conditions that are passed on genetically
• Some medications (especially improper use of medications that can cause hearing problems)
• Certain infectious diseases, such as mumps
• Various types of immune disorders

Limiting the risks as much as you can is generally a smart plan. If risk factors are there, it may be a good idea to schedule regular screenings with us.

How is auditory neuropathy diagnosed?

A normal hearing exam involves listening to tones with a pair of headphones and raising a hand depending on which side you hear the tone on. That test won’t help much with auditory neuropathy.

Instead, we will usually suggest one of two tests:

• Auditory brainstem response (ABR) test: Specialized electrodes will be connected to certain places on your head and scalp with this test. Again, don’t worry, there’s nothing painful or unpleasant about this test. These electrodes place specific focus on measuring how your brainwaves respond to sound stimuli. Whether you’re experiencing sensorineural hearing loss (outer ear) or auditory neuropathy (inner ear) will be established by the quality of your brainwaves.
• Otoacoustic emissions (OAE) test: The response of your inner ear and cochlea to stimuli will be tested with this diagnostic. A tiny microphone is placed just inside your ear canal. Then, we will play an array of clicks and tones. The diagnostic device will then measure how well your inner ear reacts to those tones and clicks. The data will help identify whether the inner ear is the problem.

Diagnosing your auditory neuropathy will be much more successful once we run the applicable tests.

Does auditory neuropathy have any treatments?

So, just like you bring your car to the mechanic to have it fixed, you can bring your ears to us for treatment! Generally speaking, there’s no “cure” for auditory neuropathy. But this disorder can be managed in several 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. For some people, hearing aids will work perfectly fine! But because volume usually isn’t the issue, this isn’t normally the case. Hearing aids are often used in combination with other treatments because of this.
• Cochlear implant: Hearing aids won’t be capable of solving the problem for most individuals. It might be necessary to opt for cochlear implants in these cases. This implant, essentially, takes the signals from your inner ear and carries them directly to your brain. They’re quite amazing! (And you can watch all kinds of YouTube videos of them working for patients.)
• Frequency modulation: In some cases, amplification or reduction of certain frequencies can help you hear better. With a technology known as frequency modulation, that’s exactly what occurs. This approach often utilizes devices that are, essentially, highly customized hearing aids.
• Communication skills training: In some cases, any and all of these treatments might be combined with communication skills training. 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 punctually will, as with any hearing disorder, lead to better outcomes.

So if you suspect you have auditory neuropathy, or even just ordinary hearing loss, it’s important to get treatment as quickly as you can. The sooner you make an appointment, the more quickly you’ll be able to hear better, and get back to your everyday life! This can be extremely critical for children, who experience a lot of cognitive development and linguistic expansion during their early years.