Have you ever had your vehicle break down in the middle of the highway? It’s not a fun situation. Your car has to be safely pulled off the road. And then, for some reason, you probably open your hood and have a look at your engine.
Humorously, you still do this even though you have no knowledge of engines. Maybe you think there’ll be a handy knob you can turn or something. Eventually, you have to call somebody to tow your car to a mechanic.
And a picture of the problem only becomes evident when experts diagnose it. That’s because cars are complex, there are so many moving parts and computerized software that the symptoms (your car that won’t start) are not enough to inform you as to what’s wrong.
With hearing loss, this same type of thing can happen. The cause is not always evident by the symptoms. 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 consider hearing loss, they think of loud concerts and jet engines, excessive noise that harms your ability to hear. This form of hearing loss, called sensorineural hearing loss is somewhat more complicated than that, but you get the point.
But sometimes, this type of long-term, noise induced damage is not the cause of hearing loss. While it’s less prevalent, hearing loss can in some cases be caused by a condition called auditory neuropathy. When sound can’t, for whatever reason, be properly sent to your brain even though your ear is receiving that sound perfectly fine.
Symptoms of auditory neuropathy
The symptoms related to auditory neuropathy are, at first glance, not all that dissimilar from those symptoms linked to conventional hearing loss. Things like cranking the volume up on your devices and not being capable of hearing very well in loud environments. This can frequently make auditory neuropathy difficult to diagnose and manage.
Still, auditory neuropathy does have a few unique properties that make it possible to identify. These presentations are pretty strong indicators that you aren’t confronting sensorineural hearing loss, but auditory neuropathy instead. Though, as always, you’ll be better served by an official diagnosis from us.
Here are some 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 might be a case of auditory neuropathy.
- Sounds sound jumbled or confused: Again, this isn’t a problem with volume. You can hear sounds but you simply can’t make sense of them. This can apply to all kinds of sounds, not just speech.
- An inability to distinguish words: Sometimes, you can’t understand what a person is saying even though the volume is just fine. Words are confused and unclear.
Some triggers of auditory neuropathy
The underlying causes of this disorder can, in part, be explained by the symptoms. On an individual level, the reasons why you may experience auditory neuropathy might not be entirely clear. This condition can develop in both children and adults. And, broadly speaking, there are a couple of well described possible causes:
- The cilia that send signals to the brain can be damaged: Sound can’t be passed to your brain in complete form once these little fragile hairs have been damaged in a specific way.
- Damage to the nerves: There’s a nerve that transmits sound signals from your inner ear to the hearing center of your brain. The sounds that the brain tries to “interpret” will sound confused if there is damage to this nerve. Sounds may seem jumbled or too quiet to hear when this occurs.
Auditory neuropathy risk factors
No one is quite sure why some people will develop auditory neuropathy while others might not. Because of this, there isn’t a definitive way to counter 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 might or may not develop auditory neuropathy. But you’re more statistically likely to develop auditory neuropathy the more risk factors you have.
Children’s risk factors
Factors that can raise the risk of auditory neuropathy for children include the following:
- Other neurological conditions
- 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
- A low birth weight
- Liver conditions that lead to jaundice (a yellow look to the skin)
- Preterm or premature birth
Risk factors for adults
Here are a few auditory neuropathy risk factors for adults:
- Various kinds of immune diseases
- Family history of hearing conditions, including auditory neuropathy
- Some medications (specifically improper use of medications that can cause hearing problems)
- Mumps and other distinct infectious diseases
In general, it’s a smart idea to limit these risks as much as possible. If risk factors are there, it might be a good plan to schedule regular screenings with us.
How is auditory neuropathy diagnosed?
A standard hearing exam involves listening to tones with a set of headphones and raising a hand depending on what side you hear the tone on. When you have auditory neuropathy, that test will be of very minimal use.
Rather, we will generally recommend one of two tests:
- Auditory brainstem response (ABR) test: During the course of this diagnostic test, you’ll have specialized electrodes attached to specific places on your head and scalp. Again, don’t be concerned, there’s nothing painful or unpleasant about this test. These electrodes put specific focus on measuring how your brainwaves react to sound stimuli. Whether you’re dealing with sensorineural hearing loss (outer ear) or auditory neuropathy (inner ear) will be established by the quality of your brainwaves.
- 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 put just inside your ear canal. Then a battery of clicks and tones will be played. The diagnostic device will then measure how well your inner ear responds to those tones and clicks. If the inner ear is a problem, this data will reveal it.
Diagnosing your auditory neuropathy will be much more successful once we run the applicable tests.
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! Generally speaking, there’s no “cure” for auditory neuropathy. But this condition can be treated in several possible ways.
- Hearing aids: Even with auditory neuropathy, in milder cases, hearing aids can boost sound enough to enable you to hear better. For some individuals, hearing aids will work just fine! That said, this isn’t generally the case, because, once again, volume is virtually never the issue. Due to this, hearing aids are usually combined with other therapy and treatment solutions.
- Cochlear implant: Hearing aids won’t be able to solve the issue for most individuals. It might be necessary to go with cochlear implants in these cases. Signals from your inner ear are transmitted directly to your brain with this implant. They’re pretty amazing! (And you can watch many YouTube videos of them working for patients.)
- Frequency modulation: Sometimes, it’s possible to hear better by boosting or reducing certain frequencies. That’s what happens with a technology known as frequency modulation. This approach often uses devices that are, essentially, highly customized hearing aids.
- Communication skills training: Communication skills training can be put together with any combination of these treatments if needed. This will help you communicate with the hearing you have and work around your symptoms instead of treating them.
The sooner you receive treatment, the better
Getting your disorder treated right away will, as with any hearing disorder, produce better outcomes.
So it’s important to get your hearing loss treated right away whether it’s the common form or auditory neuropathy. The sooner you make an appointment, the more quickly you’ll be able to hear better, and get back to your daily life! Children, who experience a great deal of cognitive growth and development, especially need to have their hearing treated as soon as possible.