Key Takeaways:
- Cookie-bite hearing loss gets its name from the 'bite' in the middle of the audiogram, where there is the most hearing loss (with better low and high frequencies).
- This type of hearing loss is typically genetic and can run in families.
- Ideally, children with cookie-bite hearing loss are identified early on and provided with amplification to help them learn at the same rate as their peers.
Have you heard about cookie-bite hearing loss or know someone who has it? Are you wondering more about what it is and what causes it?
In this article, we'll go through everything you need to know about cookie-bite hearing loss, including symptoms, tests, treatment options, and how to protect your ears.
Let's get started!
What Is Cookie-bite Hearing Loss and How Common Is It?
Cookie-bite hearing loss is sensorineural hearing loss, most noticeable in the mid-frequencies. As you might have guessed, this hearing loss has nothing to do with cookies. This moniker comes from the "U" shape of the audiogram.
Generally speaking, a person with cookie-bite hearing loss hears best at the low and high frequencies, with most of the hearing loss occurring in the mid-frequencies.
This hearing loss is typically genetic and most commonly affects both ears rather than only one ear.
It's estimated that 5 - 15% of children with hearing loss have a cookie-bite, and this number likely underestimates the prevalence of this type of hearing loss.
For some, cookie-bite hearing loss has a delayed onset. Additionally, genetic testing is still evolving, and it's only in recent decades research has started digging into this unique form of hearing loss.
From what we know, males and females are both equally susceptible to cookie-bite hearing loss.
Symptoms and Causes of Cookie-bite Hearing Loss
Common Symptoms
- Trouble understanding speech
- Asking for repetition
- Struggling in background noise
For those with cookie-bite hearing loss, having trouble hearing conversation or speech is a common symptom. The reason for this?
Many important speech sounds fall between 500 to 2000 Hz, the region with the most hearing loss for those with cookie-bite hearing loss.
Struggling in background noise is another symptom associated with cookie-bite hearing loss.
Background noise can mask speech sounds within that middle-frequency range, making it harder to hear these sounds. It can be very frustrating because your listening effort is devoted to teasing out the words rather than being more involved in the conversation.
If you notice that you're often asking for repetition or having trouble understanding, it's time to schedule a hearing evaluation to find out more about your hearing. You can try an online test here.
If you visit a local clinic, an audiologist will perform a comprehensive hearing evaluation, which provides detailed and frequency-specific information about your hearing acuity. The audiologist can also run a speech-in-noise test to assess your hearing in background noise.
Causes
- Genetic factors
- Autoimmune disorders
- Vestibular schwannoma (a benign auditory nerve growth)
Cookie-bite hearing loss is most commonly associated with genetic factors, meaning it tends to be an inherited trait. Connexin 26 is one known genetic cause of cookie-bite hearing loss. However, there are other genes associated with cookie-bite hearing loss. Some traits are dominant, while others are recessive. Depending on the gene, this hearing configuration may run in families, skip a generation, or affect some family members and not others. It can also range in severity but tends to progress over the lifespan.
Autoimmune disorders, also known as autoimmune inner ear disease (AIED), can also cause a cookie-bite hearing loss configuration. In this case, hearing loss can fluctuate, and the configuration, or shape of the audiogram, may resemble a cookie-bite. Those with AIED should consult with an ENT, especially if their hearing fluctuates.
Another, more rare, cause of a cookie-bite hearing loss can come from a benign growth on the hearing nerve, called a vestibular schwannoma. Again, this condition is very rare, but it should be evaluated and monitored by an audiologist and/or ENT. If you are experiencing hearing loss in one ear in addition to tinnitus and dizziness, it's time to call your doctor for an evaluation to rule out any underlying medical conditions.
Final Thoughts
Cookie-bite hearing loss can make it difficult to hear and understand speech. And while there is no cure for this hearing loss, treatment options like well-fitted hearing aids can help. If you have trouble hearing, it's always a good idea to get in touch with a hearing professional to schedule an appointment and discuss appropriate treatment options.
