Auditory Neuropathy, What is it?

Have you ever been in the middle of the roadway and your car breaks down? It’s not an enjoyable experience. You have to pull your car safely 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 clue how engines work. Perhaps you think there’ll be a handy knob you can turn or something. Eventually, you have to call somebody to tow your car to a mechanic.

And it’s only when the professionals get a look at things that you get an understanding of the issue. Just because the car is not moving, doesn’t mean you can know what’s wrong with it because cars are complex and computerized machines.

The same thing can occur sometimes with hearing loss. The symptom itself doesn’t necessarily identify what the underlying cause is. There’s the normal culprit (noise-associated hearing loss), sure. But in some cases, it’s something else, something like auditory neuropathy.

What is auditory neuropathy?

When most individuals consider hearing loss, they think of noisy concerts and jet engines, excessive noise that damages your ability to hear. This type of hearing loss is known as sensorineural hearing loss, and it’s a bit more involved than basic noise damage.

But in some cases, this kind of long-term, noise related damage isn’t the cause of hearing loss. A condition known as auditory neuropathy, while less prevalent, can in some cases be the cause. When sound can’t, for whatever reason, be properly transmitted to your brain even though your ear is collecting that sound just fine.

Auditory neuropathy symptoms

The symptoms related to auditory neuropathy are, at first glimpse, not all that distinct from those symptoms linked to traditional hearing loss. Things like turning up the volume on your devices and not being able to hear well in loud environments. This can often make auditory neuropathy difficult to diagnose and manage.

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

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

• Sound fades in and out: Perhaps it feels like someone is messing with the volume knob inside of your head! If you’re dealing with these symptoms it could be a case of auditory neuropathy.
• Sounds seem jumbled or confused: This is, once again, not an issue with volume. You can hear sounds but you simply can’t make sense of them. This can go beyond the speech and apply to all types of sounds around you.
• An inability to distinguish words: Sometimes, the volume of a word is just fine, but you just can’t understand what’s being said. Words are confused and unclear.

What triggers auditory neuropathy?

The root causes of this condition can, in part, be defined by its symptoms. It may not be completely clear why you have developed auditory neuropathy on a personal level. This disorder can develop in both adults and children. And there are a couple of well defined possible causes, broadly speaking:

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

Auditory neuropathy risk factors

Some people will develop auditory neuropathy while others won’t and no one is really sure why. Because of this, there isn’t a definitive way to counter auditory neuropathy. However, there are close connections which may reveal that you’re at a higher risk of experiencing this disorder.

It should be noted that these risk factors are not guarantees, you may have all of these risk factors and not develop auditory neuropathy. But the more risk factors shown, the higher your statistical probability of developing this disorder.

Children’s risk factors

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

• Liver conditions that lead to jaundice (a yellow appearance to the skin)
• Other neurological disorders
• A lack of oxygen before labor begins or during birth
• 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

Adult risk factors

Here are a few auditory neuropathy risk factors for adults:

• Mumps and other specific infectious diseases
• Family history of hearing disorders, including auditory neuropathy
• Some medications (specifically improper use of medications that can cause hearing issues)
• Various types of immune diseases

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

How is auditory neuropathy diagnosed?

During a standard hearing examination, you’ll likely be given a pair of headphones and be told 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: Specialized electrodes will be connected to specific spots on your head and scalp with this test. This test isn’t painful or uncomfortable in any way so don’t worry. These electrodes put particular 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 determined by the quality of your brainwaves.
• Otoacoustic emissions (OAE) test: The reaction of your inner ear and cochlea to stimuli will be checked with this diagnostic. We will put a small microphone just inside your ear canal. Then a battery of clicks and tones will be played. The diagnostic device will then determine how well your inner ear reacts to those tones and clicks. If the inner ear is an issue, this data will reveal it.

Once we do 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 auto technician 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 moderate cases, hearing aids can boost sound enough to enable you to hear better. Hearing aids will be a sufficient option for some individuals. But because volume isn’t usually the problem, this isn’t usually the situation. Due to this, hearing aids are often combined with other therapy and treatment solutions.
• Cochlear implant: Hearing aids won’t be able to solve the problem for most people. In these cases, a cochlear implant may be necessary. This implant, essentially, takes the signals from your inner ear and conveys them directly to your brain. They’re quite amazing! (And you can find many YouTube videos of them working for patients.)
• Frequency modulation: Sometimes, it’s possible to hear better by boosting or lowering certain frequencies. With a technology called frequency modulation, that’s exactly what occurs. This approach often makes use of 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 training. This will help you communicate with the hearing you have and work around your symptoms instead of treating them.

The sooner you receive treatment, the better

Getting your disorder treated right away will, as with any hearing condition, lead to better outcomes.

So if you suspect you have auditory neuropathy, or even just regular old hearing loss, it’s important to get treatment as quickly as you can. You’ll be able to get back to hearing better and enjoying your life once you schedule an appointment and get treated. Children, who experience a lot of cognitive growth and development, particularly need to have their hearing treated as soon as possible.