Have you ever been in the middle of the road and your car breaks down? It’s not a fun experience. Your car has to be safely pulled to the side of the road. Then you likely pop your hood and take a look at the engine. Who knows why?
What’s strange is that you do this even if you have no idea how engines work. Perhaps whatever is wrong will be obvious. Inevitably, a tow truck will have to be called.
And a picture of the problem only becomes apparent when mechanics get a look at it. That’s because cars are complicated, there are so many moving parts and computerized software that the symptoms (a car that won’t start) aren’t enough to tell you what’s wrong.
With hearing loss, this same kind of thing can happen. The symptom itself doesn’t automatically identify what the cause is. Sure, noise-related hearing loss is the common culprit. But in some cases, something else like auditory neuropathy is the culprit.
What is auditory neuropathy?
When most people consider hearing loss, they think of noisy concerts and jet engines, excessive noise that harms your hearing. This form of hearing loss, known as sensorineural hearing loss is a bit more complex than that, but you get the point.
But sometimes, long-term hearing loss can be caused by something else besides noise damage. While it’s less common, hearing loss can in some cases be caused by a condition called auditory neuropathy. This is a hearing disorder where your ear and inner ear collect sounds just fine, but for some reason, can’t fully transmit those sounds to your brain.
Symptoms of auditory neuropathy
The symptoms of traditional noise related hearing loss can often look a lot like those of auditory neuropathy. Things like turning the volume up on your devices and not being capable of hearing very well in loud environments. This can frequently make auditory neuropathy difficult to diagnose and manage.
Auditory neuropathy, however, has some unique symptoms that make diagnosing it easier. When hearing loss symptoms manifest like this, you can be fairly certain that it’s not typical noise related hearing loss. Obviously, nothing can replace getting a real-time diagnosis from us about your hearing loss.
The more distinctive symptoms of auditory neuropathy include:
- Difficulty understanding speech: In some cases, the volume of a word is normal, but you just can’t distinguish what’s being said. The words sound garbled or distorted.
- Sounds seem jumbled or confused: This is, once again, 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 apply to all kinds of sounds around you.
- Sound fades in and out: The volume of sound seems to rise and fall like somebody is playing with the volume knob. If you’re encountering these symptoms it might be a case of auditory neuropathy.
What causes auditory neuropathy?
The root causes of this disorder can, in part, be explained by its symptoms. It may not be completely clear why you have developed auditory neuropathy on an individual level. This condition can develop in both adults and children. And there are a couple of well described possible causes, broadly speaking:
- Damage to the cilia that transmit signals to the brain: Sound can’t be passed to your brain in complete form once these little delicate hairs have been damaged in a particular way.
- Damage to the nerves: There’s a nerve that carries sound signals from your inner ear to the hearing portion of your brain. If this nerve gets damaged, your brain doesn’t get the full signal, and as a result, the sounds it “interprets” will sound off. Sounds may seem garbled or too quiet to hear when this happens.
Risk factors of auditory neuropathy
Some individuals will develop auditory neuropathy while other people won’t and no one is really certain why. Because of this, there isn’t a definitive way to prevent auditory neuropathy. But you may be at a higher risk of experiencing auditory neuropathy if you present particular close associations.
Keep in mind that even if you have all of these risk factors you still may or may not experience 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 increase the risk of auditory neuropathy for children include the following:
- A lack of oxygen before labor begins or during birth
- A low birth weight
- An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)
- Preterm or premature birth
- Liver conditions that cause jaundice (a yellow look to the skin)
- Other neurological conditions
Adult risk factors
Here are a few auditory neuropathy risk factors for adults:
- Specific infectious diseases, such as mumps
- Certain medications (specifically improper use of medications that can cause hearing issues)
- Auditory neuropathy and other hearing disorders that are passed on genetically
- Immune disorders of various types
Limiting the risks as much as possible is generally 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 typical hearing test involves listening to tones with a set of headphones and raising a hand depending on what side you hear the tone on. That test won’t help very much with auditory neuropathy.
Instead, we will typically recommend one of two tests:
- Otoacoustic emissions (OAE) test: This diagnostic is designed to measure how well your inner ear and cochlea respond to sound stimuli. We will put a small microphone just inside your ear canal. Then a series of clicks and tones will be played. Then your inner ear will be measured 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 specialized electrodes attached to certain places on your scalp and head. This test isn’t painful or unpleasant in any way so don’t worry. These electrodes place specific focus on measuring how your brainwaves respond to sound stimuli. The quality of your brainwave reactions will help us determine whether your hearing problems reside in your outer ear (such as sensorineural hearing loss) or further in (as with auditory neuropathy).
Diagnosing your auditory neuropathy will be much more effective once we do the applicable tests.
Is there treatment for auditory neuropathy?
So you can bring your ears to us for treatment in the same way that you bring your car to the mechanic to have it fixed. Generally speaking, there’s no “cure” for auditory neuropathy. But there are a few ways to treat this disorder.
- Hearing aids: In some milder 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 individuals, hearing aids will work perfectly fine! That said, this is not typically the case, because, once again, volume is almost never the problem. Due to this, hearing aids are often coupled with other therapy and treatment options.
- Cochlear implant: For some people, hearing aids will not be able to get around the problems. 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. The internet has plenty of videos of individuals having success with these remarkable devices!
- Frequency modulation: In some cases, amplification or reduction of specific frequencies can help you hear better. That’s what happens with a technology called frequency modulation. Basically, highly customized hearing aids are used in this approach.
- Communication skills training: Communication skills training can be put together with any combination of these treatments if needed. This will allow you to 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 condition, lead to better outcomes.
So if you suspect you have auditory neuropathy, or even just ordinary hearing loss, it’s essential to get treatment as soon as possible. The sooner you make an appointment, the more quickly you’ll be able to hear better, and get back to your everyday life! This can be especially crucial for children, who experience a lot of cognitive development and linguistic growth during their early years.