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 off the road. And then, for some reason, you probably pop your hood and take a look at your engine.
Humorously, you still do this even though you have no knowledge of engines. Maybe whatever is wrong will be obvious. Ultimately, you have to call someone to tow your car to a garage.
And a picture of the issue only becomes evident when mechanics get a look at it. Just because the car isn’t moving, doesn’t mean you can tell what’s wrong with it because vehicles are complex and computerized machines.
With hearing loss, this same kind of thing can happen. The cause is not always evident by the symptoms. There’s the common cause (noise-related hearing loss), sure. But in some cases, something else like auditory neuropathy is the cause.
What is auditory neuropathy?
Most people think of extremely loud noise such as a rock concert or a jet engine when they think of hearing loss. This kind of hearing loss, known as sensorineural hearing loss is somewhat more complicated than that, but you get the point.
But sometimes, this sort of long-term, noise induced damage is not the cause of hearing loss. While it’s less prevalent, hearing loss can sometimes be caused by a condition known as auditory neuropathy. This is a hearing disorder in which your ear and inner ear collect sounds perfectly fine, but for some reason, can’t fully transfer those sounds to your brain.
Symptoms of auditory neuropathy
The symptoms of conventional noise related hearing loss can often look a lot like those of auditory neuropathy. You can’t hear well in noisy settings, you keep turning the volume up on your television and other devices, that kind of thing. This can often make auditory neuropathy hard to diagnose and manage.
However, auditory neuropathy does have a few unique features that make it possible to identify. When hearing loss symptoms manifest like this, you can be fairly sure that it’s not normal noise related hearing loss. Of course, nothing can replace getting an accurate diagnosis from us about your hearing loss.
The more distinctive symptoms of auditory neuropathy include:
- An inability to make out words: Sometimes, you can’t make out what somebody 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 somebody is messing with the volume knob. This could be an indication that you’re experiencing auditory neuropathy.
- Sounds seem jumbled or confused: Once again, this isn’t 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 pertain to all kinds of sounds, not just speech.
What triggers auditory neuropathy?
The root causes of this disorder can, in part, be defined by the symptoms. It might not be completely clear why you have developed auditory neuropathy on a personal level. This condition can develop in both adults and children. And there are a couple of well described possible causes, broadly speaking:
- Nerve damage: The hearing center of your brain receives sound from a specific nerve in your ear. If this nerve gets damaged, your brain can’t receive the full signal, and as a result, the sounds it “interprets” will seem wrong. When this takes place, you may interpret sounds as garbled, unclear, or too quiet to differentiate.
- Damage to the cilia that transmit signals to the brain: If these delicate hairs in your inner ear become compromised in a particular way, the sound your ear detects can’t really be sent on to your brain, at least, not in its complete form.
Risk factors of auditory neuropathy
No one is really sure why some people will develop auditory neuropathy while others might not. That’s why there’s no exact science to preventing it. But you may be at a higher risk of experiencing auditory neuropathy if you present specific close connections.
It should be mentioned that these risk factors aren’t guarantees, you could have every single one of these risk factors and not develop auditory neuropathy. But you’re more statistically likely to experience auditory neuropathy the more risk factors you have.
Risk factors for children
Factors that can raise the risk of auditory neuropathy for children include the following:
- A lack of oxygen before labor begins or during birth
- Liver conditions that result in jaundice (a yellow appearance to the skin)
- A low birth weight
- Preterm or premature birth
- An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)
- Other neurological disorders
Adult risk factors
For adults, risk factors that increase your likelihood of developing auditory neuropathy include:
- Family history of hearing disorders, including auditory neuropathy
- Overuse of medications that cause hearing problems
- Mumps and other distinct infectious diseases
- Various kinds of immune diseases
Limiting the risks as much as possible is always a smart plan. If risk factors are there, it may be a good idea to schedule regular screenings with us.
Diagnosing auditory neuropathy
During a standard hearing examination, you’ll likely be given a pair of headphones and be told to raise your hand when you hear a tone. When you’re dealing with auditory neuropathy, that test will be of very limited use.
Rather, we will generally suggest one of two tests:
- Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have special electrodes attached to specific places 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 specific attention to how those brainwaves respond to sound. The quality of your brainwave responses will help us determine whether your hearing issues reside in your outer ear (such as sensorineural hearing loss) or further in (as with auditory neuropathy).
- 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 battery of clicks and tones will be played. The diagnostic device will then evaluate how well your inner ear reacts to those tones and clicks. The data will help determine whether the inner ear is the problem.
Once we do 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 mechanic to get it fixed, you can bring your ears to us for treatment! In general, there’s no “cure” for auditory neuropathy. But this condition can be treated in several possible ways.
- Hearing aids: Even if you have auditory neuropathy, in milder cases, hearing aids can boost sound enough to allow you to hear better. For some individuals, hearing aids will work just fine! But because volume isn’t usually the issue, this isn’t usually the situation. As a result, hearing aids are usually coupled with other therapy and treatment options.
- Cochlear implant: Hearing aids won’t be able to solve the problem for most people. It may be necessary to opt for cochlear implants in these situations. Signals from your inner ear are transmitted 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: In some cases, amplification or diminution of specific frequencies can help you hear better. With a technology called frequency modulation, that’s precisely what happens. Essentially, highly customized hearing aids are utilized in this strategy.
- Communication skills training: Communication skills exercises can be combined with any combination of these treatments if necessary. 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
Getting your disorder treated promptly will, as with any hearing condition, produce 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 daily life! This can be especially crucial for children, who experience a great deal of cognitive development and linguistic growth during their early years.