Auditory Neuropathy, What is it?

Have you ever had your vehicle break down in the middle of the road? It’s not a fun situation. Your car has to be safely pulled off the road. And then, for whatever reason, you probably open your hood and have 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. Sooner or later, you have to call someone to tow your car to a garage.

And it’s only when the professionals get a look at things that you get a picture of the problem. Just because the car isn’t moving, doesn’t mean you can know what’s wrong with it because automobiles are complex and computerized machines.

The same thing can occur at times with hearing loss. The symptom itself doesn’t automatically reveal what the cause is. There’s the usual culprit (noise-related 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 form of hearing loss is called sensorineural hearing loss, and it’s somewhat more involved than basic noise damage.

But sometimes, this kind of long-term, noise related 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 disorder where your ear and inner ear receive sounds just fine, but for some reason, can’t fully transmit those sounds to your brain.

Symptoms of auditory neuropathy

The symptoms related to auditory neuropathy are, at first glimpse, not all that distinct from those symptoms associated with conventional hearing loss. You can’t hear well in noisy situations, you keep cranking the volume up on your television and other devices, that sort of thing. This can sometimes make auditory neuropathy difficult to diagnose and treat.

However, auditory neuropathy does have a few unique features that make it possible to identify. When hearing loss symptoms present like this, you can be pretty sure that it’s not normal noise related hearing loss. Obviously, nothing can replace getting a real-time diagnosis from us about your hearing loss.

The more unique symptoms of auditory neuropathy include:

• Sound fades in and out: The volume of sound seems to rise and fall like someone is messing with the volume knob. If you’re dealing with these symptoms it could be a case of auditory neuropathy.
• Difficulty understanding speech: Sometimes, you can’t make out what someone is saying even though the volume is normal. The words sound mumbled or distorted.
• Sounds sound jumbled or confused: Once again, this is not a problem with volume. The volume of what you’re hearing is just fine, the problem is that the sounds seem jumbled and you can’t understand them. This can go beyond the speech and pertain to all kinds of sounds around you.

Some triggers of auditory neuropathy

The underlying causes of this disorder can, in part, be explained by its symptoms. It might not be very clear why you have developed auditory neuropathy on an individual level. This disorder can develop in both children and adults. And, broadly speaking, there are a couple of well defined possible causes:

• The cilia that deliver signals to the brain can be damaged: Sound can’t be passed to your brain in full form once these little fragile hairs have been damaged in a specific way.
• Nerve damage: The hearing portion of your brain gets sound from a specific nerve in your ear. The sounds that the brain tries to “interpret” will seem confused if there is damage to this nerve. When this happens, you might interpret sounds as jumbled, unclear, or too quiet to discern.

Risk factors of auditory neuropathy

No one is quite sure why some people will develop auditory neuropathy while others may not. Because of this, there isn’t a tried and true way to counter auditory neuropathy. But you might be at a higher risk of experiencing auditory neuropathy if you show specific close connections.

Bear in mind that even if you have all of these risk factors you still may or may not develop auditory neuropathy. But you’re more statistically likely to experience auditory neuropathy the more risk factors you have.

Children’s risk factors

Factors that can raise the risk of auditory neuropathy for children include the following:

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

Adult risk factors

For adults, risk factors that increase your likelihood of experiencing auditory neuropathy include:

• Specific infectious diseases, such as mumps
• Some medications (especially incorrect use of medications that can cause hearing issues)
• Various types of immune diseases
• Family history of hearing conditions, including auditory neuropathy

Limiting the risks as much as you can is always a smart plan. If risk factors are present, it might be a good plan to schedule regular screenings with us.

How is auditory neuropathy diagnosed?

A standard hearing exam consists of listening to tones with a set of headphones and raising a hand depending on which side you hear the tone on. When you’re dealing with auditory neuropathy, that test will be of extremely minimal use.

Rather, we will generally recommend one of two tests:

• Otoacoustic emissions (OAE) test: This diagnostic is designed to measure how well your inner ear and cochlea react to sound stimuli. We will put a small microphone just inside your ear canal. Then a battery of tones and clicks will be played. Then your inner ear will be assessed to see how it responds. The data will help identify whether the inner ear is the issue.
• Auditory brainstem response (ABR) test: During this diagnostic test, you’ll have special electrodes attached to certain spots on your scalp and head. Again, don’t worry, there’s nothing painful or uncomfortable about this test. These electrodes track your brainwaves, with specific attention to how those brainwaves react to sound. The quality of your brainwave reactions will help us identify whether your hearing problems reside in your outer ear (such as sensorineural hearing loss) or further in (as with auditory neuropathy).

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

Is there treatment for auditory neuropathy?

So, just like you bring your car to the mechanic to have it fixed, you can bring your ears to us for treatment! Auditory neuropathy generally has no cure. But this disorder can be treated 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. Hearing aids will be an adequate option for some people. But because volume usually isn’t the problem, this isn’t normally the case. As a result, hearing aids are usually combined with other therapy and treatment solutions.
• Cochlear implant: Hearing aids won’t be capable of solving the issue for most individuals. In these cases, a cochlear implant could be necessary. Signals from your inner ear are sent directly to your brain with this implant. They’re rather amazing! (And you can watch all kinds of YouTube videos of them working for patients.)
• Frequency modulation: Sometimes, it’s possible to hear better by boosting or reducing specific frequencies. That’s what happens with a technology called frequency modulation. This approach often utilizes devices that are, basically, 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 help you communicate using the hearing you have and work around your symptoms instead of treating them.

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

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

So if you suspect you have auditory neuropathy, or even just normal hearing loss, it’s important to get treatment as soon as possible. You’ll be able to go back to hearing better and enjoying your life after you make an appointment and get treated. This can be especially critical for children, who experience a great deal of cognitive development and linguistic growth during their early years.

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The site information is for educational and informational purposes only and does not constitute medical advice. To receive personalized advice or treatment, schedule an appointment.