Have you ever been in the middle of the road and your car breaks down? It’s not an enjoyable situation. 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 you think there’ll be a convenient knob you can turn or something. Inevitably, a tow truck will have to be called.
And a picture of the issue only becomes obvious when mechanics get a look at it. That’s because cars are intricate, there are so many moving pieces and computerized software that the symptoms (a car that won’t move) aren’t enough to tell you what’s wrong.
The same thing can occur in some cases with hearing loss. The symptom itself doesn’t necessarily indicate what the cause is. Sure, noise-related hearing loss is the typical cause. But sometimes, something else like auditory neuropathy is the culprit.
Auditory neuropathy, what is it?
When most individuals think about hearing loss, they think of loud concerts and jet engines, excessive noise that harms your hearing. This type of hearing loss, called sensorineural hearing loss is somewhat more complicated than that, but you get the idea.
But in some cases, long-term hearing loss can be the result of something other than noise damage. A condition known as auditory neuropathy, while less common, can in some cases be the cause. When sound can’t, for some reason, be properly carried to your brain even though your ear is receiving that sound perfectly fine.
Auditory neuropathy symptoms
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 settings. This can sometimes make auditory neuropathy difficult to diagnose and treat.
However, auditory neuropathy does have some unique features that make it possible to diagnose. These presentations are pretty strong indicators that you aren’t experiencing sensorineural hearing loss, but auditory neuropathy instead. Of course, nothing can replace getting a real-time diagnosis from us about your hearing loss.
Here are a few of the more unique symptoms of auditory neuropathy:
- Sound fades in and out: The volume of sound seems to go up and down like someone is playing with the volume knob. If you’re encountering these symptoms it could be a case of auditory neuropathy.
- Sounds seem jumbled or confused: This is, once again, not an issue with volume. The volume of what you’re hearing is completely normal, the problem is that the sounds seem jumbled and you can’t make sense of them. This can apply to all kinds of sounds, not just speech.
- An inability to distinguish words: Sometimes, the volume of a word is just fine, but you just can’t understand what’s being said. The words sound mumbled or distorted.
What triggers auditory neuropathy?
These symptoms can be articulated, in part, by the root causes behind this particular condition. It may not be completely clear why you have developed auditory neuropathy on a personal level. This disorder can develop in both children and adults. And there are a couple of well described possible causes, broadly speaking:
- Nerve damage: The hearing portion of your brain receives sound from a specific nerve in your ear. If this nerve gets damaged, your brain can’t get the full signal, and as a result, the sounds it “interprets” will seem off. When this takes place, you might interpret sounds as jumbled, indecipherable, or too quiet to differentiate.
- The cilia that deliver signals to the brain can be damaged: Sound can’t be passed to your brain in complete form once these little delicate hairs have been compromised in a particular way.
Auditory neuropathy risk factors
No one is quite certain why some people will develop auditory neuropathy while others might not. That’s why there isn’t an exact science to preventing it. But you might be at a higher risk of experiencing auditory neuropathy if you present certain close connections.
Keep in mind that even if you have all of these risk factors you still might 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
Here are some risk factors that will increase the likelihood of auditory neuropathy in children:
- Liver disorders that cause jaundice (a yellow appearance to the skin)
- A low birth weight
- An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)
- A lack of oxygen before labor begins or during birth
- Preterm or premature birth
- Other neurological disorders
Risk factors for adults
For adults, risk factors that raise your likelihood of developing auditory neuropathy include:
- Overuse of medications that cause hearing issues
- Mumps and other distinct infectious diseases
- Family history of hearing disorders, including auditory neuropathy
- Various kinds of immune disorders
Limiting the risks as much as possible is generally a smart plan. 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 assessment, you’ll likely be given a pair of headphones and be told to raise your hand when you hear a tone. That test won’t help very much with auditory neuropathy.
Rather, we will typically suggest one of two tests:
- Otoacoustic emissions (OAE) test: This diagnostic is designed to determine how well your inner ear and cochlea respond to sound stimuli. We will put a small microphone just inside your ear canal. Then, we will play an array of tones and clicks. Then your inner ear will be assessed to see how it reacts. If the inner ear is an issue, this data will reveal it.
- Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have special electrodes attached to specific spots on your scalp and head. Again, don’t worry, there’s nothing painful or unpleasant about this test. These electrodes put specific focus on measuring how your brainwaves react to sound stimuli. The quality of your brainwave reactions will help us determine whether your hearing issues reside in your outer ear (such as sensorineural hearing loss) or further in (such as auditory neuropathy).
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, in the same way as you bring your car to the mechanic to have it fixed, you can bring your ears to us for treatment! Generally speaking, there’s no “cure” for auditory neuropathy. But this disorder can be managed in a few possible ways.
- Hearing aids: In some milder cases, hearing aids will be able to supply the necessary sound amplification to help you hear better, even with auditory neuropathy. For some individuals, hearing aids will work perfectly fine! That said, this is not usually the case, because, once again, volume is almost never the problem. As a result, hearing aids are often coupled with other therapy and treatment solutions.
- Cochlear implant: Hearing aids won’t be able to solve the issue for most people. In these cases, a cochlear implant could be required. Signals from your inner ear are sent directly to your brain with this implant. They’re quite amazing! (And you can watch 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 occurs with a technology known as frequency modulation. This approach often uses 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 training. This will let you work with whatever level of hearing you have to communicate better.
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 think you have auditory neuropathy, or even just ordinary hearing loss, it’s important to get treatment as quickly as possible. You’ll be able to go back to hearing better and enjoying your life after you make an appointment and get treated. Children, who experience a great deal of cognitive growth and development, particularly need to have their hearing treated as soon as possible.