Have you ever had your vehicle break down in the middle of the highway? That really stinks! Your car has to be safely pulled to the side of the road. And then, for whatever reason, you probably open your hood and have a look at your engine.
What’s strange is that you do this even though you have no clue how engines work. Perhaps 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 apparent when mechanics diagnose it. Just because the car isn’t moving, doesn’t mean you can know what’s wrong with it because vehicles are complex and computerized machines.
With hearing loss, this same kind of thing can occur. The cause isn’t always apparent by the symptoms. There’s the usual cause (noise-related hearing loss), sure. But sometimes, something else like auditory neuropathy is the cause.
Auditory neuropathy, what is it?
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, called sensorineural hearing loss is somewhat more complicated than that, but you get the point.
But in some cases, this type of long-term, noise related damage isn’t the cause of hearing loss. A condition known as auditory neuropathy, while less prevalent, can in some cases be the cause. When sound can’t, for whatever reason, be effectively carried to your brain even though your ear is collecting that sound just fine.
Auditory neuropathy symptoms
The symptoms of traditional noise related hearing loss can sometimes look a lot like those of auditory neuropathy. You can’t hear well in loud settings, you keep turning the volume up on your television and other devices, that sort of thing. This can often make auditory neuropathy difficult to diagnose and treat.
Still, auditory neuropathy does have some unique properties that make it possible to diagnose. When hearing loss symptoms manifest in this way, you can be fairly certain that it’s not normal noise related hearing loss. Obviously, nothing can replace getting an accurate diagnosis from us about your hearing loss.
The more distinctive symptoms of auditory neuropathy include:
- 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 could be a case of auditory neuropathy.
- Sounds seem jumbled or confused: Once again, this is not a problem with volume. You can hear sounds but you just can’t understand them. This can pertain to all sorts of sounds, not just speech.
- An inability to distinguish words: In some cases, the volume of a word is just fine, but you just can’t distinguish what’s being said. Words are unclear and muddled sounding.
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 may develop auditory neuropathy may not be totally clear. Both adults and children can develop this condition. And, broadly speaking, there are a couple of well described possible causes:
- Damage to the nerves: The hearing portion of your brain gets sound from a specific nerve in your ear. If this nerve becomes damaged, your brain can’t get the complete signal, and consequently, the sounds it “interprets” will seem wrong. When this happens, you may interpret sounds as garbled, unclear, or too quiet to discern.
- The cilia that send signals to the brain can be damaged: If these tiny hairs in your inner ear become compromised in a particular way, the sound your ear senses can’t really be passed on to your brain, at least, not in its complete form.
Risk factors of auditory neuropathy
Some people will develop auditory neuropathy while other people won’t and no one is quite sure why. That’s why there isn’t an exact science to preventing it. However, there are close associations which may reveal that you’re at a higher risk of developing this disorder.
It should be mentioned that these risk factors are not guarantees, you might have all of these risk factors and not develop auditory neuropathy. But you’re more statistically likely to develop auditory neuropathy the more risk factors you have.
Risk factors for children
Here are some risk factors that will raise the likelihood of auditory neuropathy in children:
- Other neurological conditions
- Liver disorders that result in jaundice (a yellow appearance to the skin)
- 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)
- Preterm or premature birth
- A low birth weight
Risk factors for adults
Here are some auditory neuropathy risk factors for adults:
- Family history of hearing conditions, including auditory neuropathy
- Certain infectious diseases, like mumps
- Overuse of medications that cause hearing issues
- Immune diseases of various types
Limiting the risks as much as possible is always a smart plan. Scheduling regular screenings with us is a smart idea, particularly if you do have risk factors.
Diagnosing auditory neuropathy
During a typical hearing examination, you’ll likely be given a set of headphones and be told to raise your hand when you hear a tone. That test won’t help very much with auditory neuropathy.
One of the following two tests will usually be used instead:
- Otoacoustic emissions (OAE) test: The response of your inner ear and cochlea to stimuli will be checked with this diagnostic. A little microphone is put just inside your ear canal. Then a battery of tones and clicks will be played. Then your inner ear will be measured to see how it responds. The data will help determine whether the inner ear is the issue.
- Auditory brainstem response (ABR) test: Specialized electrodes will be attached to specific spots on your head and scalp with this test. Again, don’t be concerned, there’s nothing painful or uncomfortable about this test. These electrodes track your brainwaves, with particular attention to how those brainwaves respond to sound. The quality of your brainwave responses will help us identify whether your hearing problems reside in your outer ear (such as sensorineural hearing loss) or further in (as with auditory neuropathy).
Once we do the appropriate tests, we will be able to more effectively 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 get it fixed, you can bring your ears to us for treatment! Auditory neuropathy generally has no cure. But this condition can be treated in several possible ways.
- Hearing aids: In some less severe cases, hearing aids will be able to provide the necessary sound amplification to help you hear better, even with auditory neuropathy. Hearing aids will be an adequate option for some people. Having said that, this is not typically the case, because, again, volume is virtually never the issue. As a result, hearing aids are often combined with other therapy and treatment solutions.
- Cochlear implant: For some people, hearing aids won’t be able to get around the issues. It might 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 rather amazing! (And you can watch many YouTube videos of them working for patients.)
- Frequency modulation: In some cases, it’s possible to hear better by boosting or lowering certain frequencies. With a technology called frequency modulation, that’s precisely what happens. This strategy often uses devices that are, essentially, highly customized hearing aids.
- Communication skills training: Communication skills training can be combined with any combination of these treatments if necessary. This will help you communicate with the hearing you have and work around your symptoms instead of treating them.
It’s best to get treatment as soon as you can
Getting your condition treated punctually will, as with any hearing disorder, produce better outcomes.
So it’s important to get your hearing loss treated as soon as possible whether it’s the ordinary form or auditory neuropathy. You’ll be able to get 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 expansion during their early years.
