Have you ever had your vehicle break down in the middle of the road? It’s not a fun experience. You have to pull your car off the road. And then, for some reason, you probably pop your hood and take a look at your engine.
What’s funny is that you do this even though you have no idea how engines work. Perhaps you think there’ll be a convenient knob you can turn or something. Inevitably, 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. That’s because cars are complex, there are so many moving pieces and computerized software that the symptoms (a car that won’t move) are not enough to inform you as to what’s wrong.
The same thing can occur in some cases with hearing loss. The cause is not always evident by the symptoms. Sure, noise-related hearing loss is the usual cause. But in some cases, it’s something else, something such as auditory neuropathy.
What is auditory neuropathy?
Most people think of really loud noise like a rock concert or a jet engine when they consider hearing loss. This kind of hearing loss is known as sensorineural hearing loss, and it’s somewhat more involved than basic noise damage.
But in some cases, this type of long-term, noise related damage is not the cause of hearing loss. While it’s less common, hearing loss can in some cases be caused by a condition known as auditory neuropathy. This is a hearing condition in which your ear and inner ear receive sounds just fine, but for some reason, can’t fully transmit those sounds to your brain.
Auditory neuropathy symptoms
The symptoms associated with auditory neuropathy are, at first glimpse, not all that distinct from those symptoms linked to conventional hearing loss. Things like cranking the volume up on your devices and not being able to hear well in loud environments. This can often make auditory neuropathy difficult to diagnose and treat.
Auditory neuropathy, however, has some distinctive symptoms that make spotting it easier. These presentations are pretty solid indicators that you aren’t experiencing sensorineural hearing loss, but 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:
- Sounds sound jumbled or confused: Again, this is not a problem with volume. You can hear sounds but you just can’t understand them. This can apply to all sorts of sounds, not just speech.
- Difficulty understanding speech: Sometimes, you can’t understand what someone is saying even though the volume is normal. The words sound mumbled or distorted.
- 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 a sign that you’re dealing with auditory neuropathy.
What triggers auditory neuropathy?
These symptoms can be explained, in part, by the underlying causes behind this specific condition. On a personal level, the reasons why you may develop auditory neuropathy might not be totally clear. Both children and adults can experience this condition. And, broadly speaking, there are a couple of well described possible causes:
- Damage to the cilia that send signals to the brain: If these little hairs in your inner ear become damaged in a specific way, the sound your ear detects can’t really be sent on to your brain, at least, not in its complete form.
- Nerve damage: There’s a nerve that transmits sound signals from your inner ear to the hearing portion of your brain. The sounds that the brain attempts to “interpret” will seem confused if there is damage to this nerve. Sounds might seem jumbled or too quiet to hear when this happens.
Auditory neuropathy risk factors
No one is really sure why some individuals will experience auditory neuropathy while others might not. As a result, there isn’t a definitive way to counter auditory neuropathy. But you may be at a higher risk of developing auditory neuropathy if you present particular close associations.
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 you’re more statistically likely to develop 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:
- Liver disorders that lead to jaundice (a yellow appearance to the skin)
- 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 disorders
- A lack of oxygen during birth or before labor begins
Adult risk factors
Here are a few auditory neuropathy risk factors for adults:
- Immune diseases of various kinds
- Family history of hearing disorders, including auditory neuropathy
- Specific infectious diseases, such as mumps
- Some medications (specifically incorrect use of medications that can cause hearing issues)
Minimizing the risks as much as you can is always a good idea. Scheduling regular screenings with us is a smart idea, especially if you do have risk factors.
Diagnosing auditory neuropathy
A standard hearing test involves listening to tones with a pair of headphones and raising a hand depending on what side you hear the tone on. That test won’t help much with auditory neuropathy.
Instead, we will generally recommend one of two tests:
- Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have specialized electrodes attached to certain spots on your scalp and head. This test isn’t painful or unpleasant in any way so don’t worry. These electrodes track your brainwaves, with particular attention to how those brainwaves react to sound. 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: This diagnostic is made 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 measured to see how it responds. If the inner ear is an issue, this data will expose it.
Once we run the appropriate tests, we will be able to more successfully diagnose and treat your auditory neuropathy.
Is there treatment for auditory neuropathy?
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 disorder can be treated in a few possible ways.
- Hearing aids: In some moderate cases, hearing aids will be able to supply the necessary sound amplification to help you hear better, even with auditory neuropathy. Hearing aids will be an adequate solution for some people. Having said that, this is not typically the case, because, once again, volume is almost never the issue. Due to this, hearing aids are usually coupled with other therapy and treatment solutions.
- Cochlear implant: Hearing aids won’t be capable of solving the issue 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 conveys them directly to your brain. They’re quite amazing! (And you can find all kinds of YouTube videos of them working for patients.)
- Frequency modulation: In some cases, it’s possible to hear better by increasing or reducing certain frequencies. That’s what happens with a technology called frequency modulation. Basically, highly customized hearing aids are utilized in this strategy.
- Communication skills training: In some cases, any and all of these treatments might be combined with communication skills training. 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 possible
As with any hearing condition, timely treatment can lead to better outcomes.
So it’s essential to get your hearing loss treated as soon as possible whether it’s the ordinary form or auditory neuropathy. The sooner you schedule an appointment, the more quickly you’ll be able to hear better, and get back to your daily life! This can be especially critical for children, who experience a lot of cognitive development and linguistic growth during their early years.