What is Auditory Neuropathy?

Have you ever had your car break down in the middle of the road? That really stinks! Your car has to be safely pulled to the side of the road. And then, for some reason, you probably pop your hood and take a look at your engine.

What’s strange is that you do this even if you have no idea how engines work. Perhaps you think there’ll be a convenient handle you can turn or something. Ultimately, a tow truck will have to be called.

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

With hearing loss, this same sort of thing can happen. The symptom itself doesn’t automatically identify what the cause is. Sure, noise-related hearing loss is the usual cause. But in some cases, something else like auditory neuropathy is the culprit.

What is auditory neuropathy?

When most individuals think about hearing loss, they think of noisy concerts and jet engines, excessive noise that damages your ability to hear. 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 kind of long-term, noise induced damage isn’t the cause of hearing loss. A condition known as auditory neuropathy, while less prevalent, can sometimes be the cause. When sound can’t, for whatever reason, be properly carried to your brain even though your ear is receiving that sound perfectly fine.

Symptoms of auditory neuropathy

The symptoms associated with auditory neuropathy are, at first glance, not all that dissimilar from those symptoms associated with traditional hearing loss. You can’t hear very well in loud settings, you keep turning up the volume on your television and other devices, that kind of thing. This can frequently make auditory neuropathy hard to diagnose and manage.

But auditory neuropathy, however, has some specific symptoms that make recognizing it easier. When hearing loss symptoms present in this way, you can be pretty certain that it’s not normal noise related hearing loss. Though, as always, you’ll be better served 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 problem 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.
• Difficulty understanding speech: In some cases, the volume of a word is just fine, but you just can’t understand what’s being said. Words are unclear and muddled sounding.
• Sound fades in and out: The volume of sound seems to rise and fall like someone is playing with the volume knob. This could be an indication that you’re dealing with auditory neuropathy.

What triggers auditory neuropathy?

The underlying causes of this condition can, in part, be defined by its symptoms. It might not be entirely clear why you have developed auditory neuropathy on an individual level. Both adults and children can experience this condition. And there are a couple of well defined possible causes, generally speaking:

• 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 tries to “interpret” will sound confused if there is damage to this nerve. When this happens, you may interpret sounds as garbled, indecipherable, or too quiet to discern.
• Damage to the cilia that send signals to the brain: If these fragile 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 full 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’s no exact science to preventing it. Still, there are close associations which may indicate that you’re at a higher risk of developing this condition.

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

Children’s risk factors

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

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

Adult risk factors

Here are some auditory neuropathy risk factors for adults:

• Various types of immune disorders
• Mumps and other distinct infectious diseases
• Overuse of medications that cause hearing problems
• Family history of hearing disorders, including auditory neuropathy

Limiting the risks as much as possible is always a smart plan. Scheduling regular screenings with us is a good plan, especially if you do have risk factors.

How is auditory neuropathy diagnosed?

A typical hearing exam consists of listening to tones with a pair of headphones and raising a hand depending on which side you hear the tone on. When you have auditory neuropathy, that test will be of very minimal use.

Instead, we will typically suggest one of two tests:

• Otoacoustic emissions (OAE) test: The reaction of your inner ear and cochlea to stimuli will be evaluated with this diagnostic. We will put a small microphone just inside your ear canal. Then, we will play an array of tones and clicks. The diagnostic device will then measure how well your inner ear responds to those tones and clicks. If the inner ear is an issue, this data will expose it.
• 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. Again, don’t worry, there’s nothing painful or unpleasant about this test. These electrodes place particular emphasis on tracking how your brainwaves react to sound stimuli. The quality of your brainwave responses will help us determine whether your hearing problems reside in your outer ear (such as sensorineural hearing loss) or further in (such as auditory neuropathy).

Diagnosing your auditory neuropathy will be much more successful once we do the appropriate tests.

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 this condition can be treated in several possible ways.

• Hearing aids: Even if you have auditory neuropathy, in milder cases, hearing aids can boost sound enough to allow you to hear better. For some individuals, hearing aids will work just fine! Having said that, this is not usually the case, because, again, volume is almost never the problem. Due to this, hearing aids are often coupled with other therapy and treatment solutions.
• Cochlear implant: For some people, hearing aids won’t be able to solve the issues. In these instances, a cochlear implant could be required. Signals from your inner ear are transmitted directly to your brain with this implant. The internet has lots of videos of individuals having success with these amazing devices!
• Frequency modulation: Sometimes, it’s possible to hear better by boosting or reducing certain frequencies. That’s what occurs with a technology known as frequency modulation. This approach often utilizes devices that are, essentially, highly customized hearing aids.
• Communication skills training: In some situations, any and all of these treatments may 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 right away will, as with any hearing condition, produce better outcomes.

So if you think you have auditory neuropathy, or even just ordinary hearing loss, it’s important to get treatment as soon as you can. The sooner you schedule an appointment, the more quickly you’ll be able to hear better, and get back to your everyday life! Children, who experience a lot of cognitive growth and development, especially need to have their hearing treated as soon as possible.