What is Auditory Neuropathy?

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

Humorously, you still do this despite the fact that you have no knowledge of engines. Maybe you think there’ll be a handy knob you can turn or something. Ultimately, you have to call someone to tow your car to a mechanic.

And it’s only when the experts get a look at things that you get an understanding of the issue. That’s because cars are intricate, there are so many moving parts and computerized software that the symptoms (a car that won’t move) aren’t enough to tell you what’s wrong.

With hearing loss, this same sort of thing can occur. The cause isn’t always evident by the symptoms. There’s the common cause (noise-associated hearing loss), sure. But sometimes, something else like auditory neuropathy is the culprit.

What is auditory neuropathy?

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

But in some cases, this sort of long-term, noise induced damage is not the cause of hearing loss. While it’s less prevalent, hearing loss can sometimes be caused by a condition called auditory neuropathy. This is a hearing condition where your ear and inner ear receive sounds just fine, but for some reason, can’t fully convey those sounds to your brain.

Auditory neuropathy symptoms

The symptoms associated with auditory neuropathy are, at first look, not all that distinct from those symptoms associated with traditional hearing loss. You can’t hear well in noisy settings, 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.

But auditory neuropathy, however, has some specific symptoms that make discovering it easier. These presentations are pretty solid indicators that you aren’t confronting sensorineural hearing loss, but with auditory neuropathy instead. Obviously, nothing can replace getting an accurate diagnosis from us about your hearing loss.

Here are some of the more unique symptoms of auditory neuropathy:

• The inability to distinguish words: Sometimes, you can’t make out what someone is saying even though the volume is normal. The words sound mumbled or distorted.
• Sounds seem jumbled or confused: Once again, this isn’t an issue with volume. The volume of what you’re hearing is just fine, the problem 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.
• Sound fades in and out: Maybe it feels like someone is messing with the volume knob inside of your head! If you’re encountering these symptoms it might be a case of auditory neuropathy.

Some triggers of auditory neuropathy

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

• The cilia that transmit signals to the brain can be damaged: Sound can’t be sent to your brain in complete form once these little fragile hairs have been compromised in a particular way.
• Damage to the nerves: There’s a nerve that transmits sound signals from your inner ear to the hearing center of your brain. The sounds that the brain tries to “interpret” will sound unclear if there is damage to this nerve. Sounds may seem garbled or too quiet to hear when this occurs.

Risk factors of auditory neuropathy

No one is quite certain why some people will develop auditory neuropathy while others might not. That’s why there isn’t an exact science to combating it. Nevertheless, there are close connections which may show that you’re at a higher risk of experiencing this disorder.

Bear in mind that even if you have all of these risk factors you still may or may not develop auditory neuropathy. But the more risk factors shown, the higher your statistical probability of developing this disorder.

Risk factors for children

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

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

Risk factors for adults

Here are a few auditory neuropathy risk factors for adults:

• Immune disorders of various types
• Mumps and other specific infectious diseases
• Overuse of medications that cause hearing issues
• auditory neuropathy and other hearing disorders that are passed on genetically

In general, it’s a smart idea to minimize these risks as much as you can. Scheduling regular screenings with us is a smart plan, particularly if you do have risk factors.

How is auditory neuropathy diagnosed?

During a typical hearing examination, you’ll most likely be given a set of headphones and be told to raise your hand when you hear a tone. That test won’t help very much with auditory neuropathy.

Instead, we will usually recommend one of two tests:

• Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have special electrodes attached to specific spots on your head and scalp. This test isn’t painful or uncomfortable in any way so don’t be concerned. These electrodes track your brainwaves, with particular attention to how those brainwaves respond to sound. The quality of your brainwave responses will help us determine whether your hearing problems reside in your outer ear (as with sensorineural hearing loss) or further in (as with auditory neuropathy).
• Otoacoustic emissions (OAE) test: The reaction of your inner ear and cochlea to stimuli will be evaluated with this diagnostic. A little microphone is put just inside your ear canal. Then, we will play an array of clicks and tones. 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 effectively diagnose and treat your auditory neuropathy.

Does auditory neuropathy have any treatments?

So, in the same way as you bring your car to the auto technician to get it fixed, you can bring your ears to us for treatment! Generally speaking, there’s no “cure” for auditory neuropathy. But this condition can be treated in several possible ways.

• Hearing aids: In some less severe cases, hearing aids will be able to provide the necessary sound amplification to help you hear better, even if you have auditory neuropathy. For some people, hearing aids will work just fine! Having said that, this isn’t typically the case, because, again, volume is virtually never the problem. As a result, hearing aids are frequently coupled with other therapy and treatment solutions.
• Cochlear implant: Hearing aids won’t be able to solve the problem for most individuals. It might be necessary to opt for cochlear implants in these situations. Signals from your inner ear are transmitted directly to your brain with this implant. They’re rather amazing! (And you can find all kinds of YouTube videos of them working for patients.)
• Frequency modulation: In some cases, amplification or diminution of certain frequencies can help you hear better. With a technology known as frequency modulation, that’s exactly what happens. This strategy often utilizes devices that are, essentially, highly customized hearing aids.
• Communication skills training: In some situations, any and all of these treatments might be combined with communication skills exercises. This will let you work with whatever level of hearing you have to communicate better.

It’s best to get treatment as soon as possible

Getting your disorder treated punctually will, as with any hearing disorder, lead to better outcomes.

So it’s important to get your hearing loss treated as soon as possible whether it’s the common form or auditory neuropathy. You’ll be able to go back to hearing better and enjoying your life once you make an appointment and get treated. This can be especially critical for children, who experience a lot of cognitive development and linguistic expansion during their early years.