Have you ever been in the middle of the roadway and your car breaks down? That really stinks! Your car has to be safely pulled to the side of the road. Then you likely open your hood and have a look at the engine. Who knows why?
What’s funny is that you do this even if you have no idea how engines work. Perhaps whatever is wrong will be obvious. Ultimately, you have to call somebody to tow your car to a garage.
And a picture of the issue only becomes obvious when experts diagnose it. That’s because cars are complicated, there are so many moving pieces 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 sort of thing can happen. The cause isn’t always obvious by the symptoms. Sure, noise-related hearing loss is the typical culprit. But sometimes, it’s something else, something like auditory neuropathy.
Auditory neuropathy, what is it?
Most people think of extremely loud noise like a rock concert or a jet engine when they consider hearing loss. This kind of hearing loss, known as 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 prevalent, hearing loss can in some cases be caused by a condition known as auditory neuropathy. This is a hearing condition where your ear and inner ear receive sounds just fine, but for some reason, can’t fully convey those sounds to your brain.
Auditory neuropathy symptoms
The symptoms associated with auditory neuropathy are, at first look, not all that distinct from those symptoms associated with conventional hearing loss. Things like cranking up the volume on your devices and not being capable of hearing very well in loud environments. That’s why diagnosing auditory neuropathy can be so challenging.
Auditory neuropathy, however, has some distinctive symptoms that make diagnosing it easier. When hearing loss symptoms manifest in this way, you can be pretty sure that it’s not typical noise related hearing loss. Though, as always, you’ll be better informed by an official diagnosis from us.
Here are a few of the more unique symptoms of auditory neuropathy:
- The inability to distinguish words: Sometimes, you can’t understand what someone is saying even though the volume is normal. Words are confused and muddled sounding.
- Sound fades in and out: Maybe it feels like somebody is playing with the volume knob in your head! If you’re encountering these symptoms it may be a case of auditory neuropathy.
- 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 pertain to all kinds of sounds around you.
What triggers auditory neuropathy?
The root causes of this condition can, in part, be explained by its symptoms. It may not be completely clear why you have developed auditory neuropathy on an individual level. Both adults and children can experience this condition. And, broadly speaking, there are a couple of well described possible causes:
- The cilia that transmit signals to the brain can be damaged: Sound can’t be sent to your brain in complete form once these little delicate hairs have been compromised in a specific way.
- Damage to the nerves: The hearing center of your brain receives sound from a specific nerve in your ear. If this nerve gets damaged, your brain doesn’t receive the full signal, and as a result, the sounds it “interprets” will sound wrong. Sounds might seem jumbled or too quiet to hear when this happens.
Auditory neuropathy risk factors
No one is quite sure why some individuals will develop auditory neuropathy while others might not. As a result, there isn’t a definitive way to prevent auditory neuropathy. But you might be at a higher risk of experiencing auditory neuropathy if you present certain close associations.
Keep in mind that even if you have all of these risk factors you still may or may not develop auditory neuropathy. But the more risk factors present, the higher your statistical likelihood of developing this condition.
Risk factors for children
Here are a few risk factors that will increase the likelihood of auditory neuropathy in children:
- Other neurological disorders
- An abundance of bilirubin in the blood (bilirubin is a normal byproduct of red blood cell breakdown)
- A low birth weight
- Liver disorders that cause jaundice (a yellow look to the skin)
- A lack of oxygen before labor begins or during birth
- Preterm or premature birth
Risk factors for adults
For adults, risk factors that increase your likelihood of experiencing auditory neuropathy include:
- Family history of hearing disorders, including auditory neuropathy
- Various kinds of immune disorders
- Some medications (specifically incorrect use of medications that can cause hearing problems)
- Specific infectious diseases, like mumps
In general, it’s a smart idea to minimize these risks as much as you can. If risk factors are present, it might be a good plan to schedule regular screenings with us.
Diagnosing auditory neuropathy
A standard hearing exam involves listening to tones with a pair of headphones and raising a hand depending on which side you hear the tone on. That test won’t help much with auditory neuropathy.
One of the following two tests will normally be done instead:
- 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 little microphone just inside your ear canal. Then, we will play a series of tones and clicks. Then your inner ear will be assessed to see how it responds. If the inner ear is a problem, this data will expose it.
- Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have specialized electrodes attached to specific places on your scalp and head. Again, don’t be concerned, there’s nothing painful or unpleasant about this test. These electrodes track your brainwaves, with specific attention to how those brainwaves respond to sound. The quality of your brainwave reactions will help us identify whether your hearing problems reside in your outer ear (as with sensorineural hearing loss) or further in (as with auditory neuropathy).
Once we run the appropriate tests, we will be able to more successfully diagnose and treat your auditory neuropathy.
Does auditory neuropathy have any treatments?
So you can bring your ears to us for treatment just like you bring your car to the mechanic to have it fixed. auditory neuropathy generally has no cure. But this condition can be treated in several possible ways.
- Hearing aids: Even with auditory neuropathy, in moderate cases, hearing aids can boost sound enough to enable you to hear better. For some people, hearing aids will work just fine! But because volume usually isn’t the problem, this isn’t typically the situation. As a result, hearing aids are frequently coupled with other therapy and treatment solutions.
- Cochlear implant: Hearing aids won’t be capable of solving the problem for most people. In these situations, a cochlear implant might be needed. Signals from your inner ear are sent directly to your brain with this implant. They’re pretty amazing! (And you can find many YouTube videos of them working for patients.)
- Frequency modulation: In some cases, it’s possible to hear better by boosting or reducing certain frequencies. That’s what occurs with a technology called frequency modulation. This approach frequently makes use of devices that are, basically, 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 with the hearing you have and work around your symptoms instead of treating them.
The sooner you get treatment, the better
Getting your disorder treated right away 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 you can. You’ll be able to go back to hearing better and enjoying your life after you schedule an appointment and get treated. This can be extremely crucial for children, who experience a lot of cognitive development and linguistic growth during their early years.