Have you ever had your car break down in the middle of the road? It’s not a fun 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 funny is that you do this even if you have no clue how engines work. Maybe you think there’ll be a handy knob you can turn or something. Eventually, you have to call someone to tow your car to a garage.
And it’s only when the experts get a look at things that you get a picture of the problem. That’s because cars are complicated–there are so many moving pieces and computerized software that the symptom (a car that won’t move) is not enough to tell you what’s wrong.
The same thing can happen sometimes with hearing loss. The symptom itself doesn’t necessarily indicate what the cause is. There’s the usual culprit (noise-related hearing loss), sure. But sometimes it’s something else–something like auditory neuropathy.
What is auditory neuropathy?
When most people think about hearing loss, they think of loud concerts and jet engines–excessive noise that damages your ability to hear. (This type of hearing loss, called sensorineural hearing loss is a bit more complicated than that–but you get the idea.)
But sometimes, this type of long-term, noise related damage is not the cause of hearing loss. While it’s less common, hearing loss can sometimes be caused by a condition called auditory neuropathy. This is a hearing disorder 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 glance, not all that dissimilar from those symptoms associated with traditional hearing loss. You can’t hear well in noisy situations, you keep turning the volume on your television up–that kind of thing. This can sometimes make auditory neuropathy difficult to diagnose and treat.
However, auditory neuropathy does have some unique features that make it possible to identify. These presentations are pretty strong indicators that you aren’t dealing with sensorineural hearing loss–but with auditory neuropathy instead. (Though, as always, you’ll be better served by an official diagnosis from a hearing specialist.)
The more unique symptoms of auditory neuropathy include:
- An inability to make out words: Sometimes the volume of a word is just fine–but you just can’t understand what’s being said. The words sound garbled or distorted.
- Sounds seem jumbled or confused: Again, this is not an issue with volume. The volume of what you’re hearing is just fine–the problem is that the sounds seem jumbled and you can’t make sense of them. This can go beyond the spoken word and apply to all kinds of sounds around you.
- Sound fades in and out: Maybe it feels like someone is messing with the volume knob in your head! This could be a sign that you’re dealing with auditory neuropathy.
What causes auditory neuropathy?
These symptoms can be explained, in part, by the root causes behind this particular disorder. On an individual level, the reasons why you might develop auditory neuropathy might not be completely clear. This condition can develop in both children and adults. And, broadly speaking, there are a couple of well described possible causes:
- Damage to the cilia that send signals to the brain: If these tiny hairs in your inner ear become damaged in a particular way, the sound your ear senses can’t really be passed on to your brain–at least, not in its full form.
- Damage to the nerves: There’s a nerve that carries sound signals from your inner ear to the hearing center of your brain. If this nerve becomes damaged, your brain doesn’t get the full signal–and as a result, the sounds it “interprets” will seem wrong. When this happens, you might interpret sounds as garbled, indecipherable, or too quiet to discern.
Auditory neuropathy risk factors
No one is quite sure why some people will develop auditory neuropathy while others may not. As a result, there isn’t a tried and true way to prevent auditory neuropathy. However, there are close associations which might indicate you’re at a higher risk of developing this condition.
It should be noted that these risk factors are not guarantees–you could have every single one of these risk factors and not develop auditory neuropathy. But the risk factors present, the higher your statistical likelihood of developing this condition.
Risk factors for children
Factors that can increase the risk of auditory neuropathy for children include the following:
- Preterm or premature birth
- A low birth weight
- A lack of oxygen during birth or before labor begins
- An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)
- Liver disorders that lead to jaundice (a yellow appearance to the skin)
- Other neurological disorders
Risk factors for adults
For adults, risk factors that increase your likelihood of developing auditory neuropathy include:
- Family history of hearing disorders, including auditory neuropathy
- Immune disorders or various kinds
- Certain infectious diseases, such as mumps
- Certain medications (especially improper use of medications that can cause hearing problems)
In general, it’s a good idea to limit these risks as much as possible. If risk factors are present, it might be a good idea to schedule regular screenings with your auditory specialist.
How is auditory neuropathy diagnosed?
During a normal hearing test, you’ll likely be given a pair of headphones and be asked to raise your hand when you hear a tone. When you have auditory neuropathy, that test will be of very limited use.
Instead, your hearing specialist 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. A tiny microphone is placed just inside your ear canal. Then, your hearing specialist will play a series of clicks and tones. The diagnostic device will then measure how well your inner ear responds to those tones and clicks. The data will help determine whether the inner ear is the problem.
- Auditory brainstem response (ABR) test: During this diagnostic test, you’ll have special electrodes attached to specific places on your scalp and head. (Again, don’t worry–there’s nothing painful or uncomfortable about this test.) These electrodes measure your brainwaves–with particular attention to how those brainwaves respond to sound. The quality of your brainwave responses will help your hearing specialist determine whether your hearing problems reside in your outer ear (as with sensorineural hearing loss) or further in (as with auditory neuropathy).
Once your hearing specialist runs the appropriate tests, you’ll 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 mechanic to get it fixed, you can bring your ears to the hearing specialist for treatment! In general, there’s no “cure” for auditory neuropathy. But there are several ways to treat this condition.
- Hearing aids: In some milder cases, hearing aids will be able to provide the necessary sound amplification to help you hear better, even with auditory neuropathy. For some people, hearing aids will work just fine! That said, this is not typically the case–because, again, volume is almost never the problem. As a result 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 issues. In these cases, a cochlear implant may be necessary. This implant, basically, takes the signals from your inner ear and transports them directly to your brain. They’re pretty amazing! (And you can find all kinds of YouTube videos of them working for patients.)
- Frequency modulation: Sometimes, amplification or diminution of specific frequencies can help you hear better. That’s what happens with a technology called frequency modulation. This approach often uses devices that are, essentially, highly customized hearing aids.
- Communication skills training: In some cases, any and all of these treatments may 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.
The sooner you receive treatment, the better
As with any hearing disorder, prompt treatment can lead to better outcomes.
So if you think you have auditory neuropathy–or even just regular old hearing loss–it’s important to get treatment as quickly as possible. The sooner you see a hearing specialist, 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 great deal of cognitive development and linguistic expansion during their early years.
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