On Thursday’s episode of Grey’s Anatomy (titled “Shiny Happy People”), a teenage patient called Hayley is thought to have schizophrenia after trying to claw her own eyes out. Not unlike a House episode we recently covered, the diagnosis turns out wrong and instead she is found to have a condition called superior canal dehiscence syndrome.

Photo by ABC

Danger level: Medium

What is it?

Superior canal dehiscence syndrome (SCDS) is a rare condition affecting the ear which causes the person having it to hear sounds inside their body, among other things. It was first described in 1998.

Who gets it?

Since the condition was first described only in 1998, not much is known in that area. A study conducted on cadavers (dead bodies) found ears matching the condition in about 0.5% of bodies checked.

It appears to affect women and men alike. The condition usually affects people around the age of 40, although it may start earlier or later.

What causes it?

Our ear, as we described here before, is divided into 3 parts: The outer ear, middle ear, and inner ear. Our inner ear is the innermost part, and it’s responsible for hearing and balance.

The inner ear.

Our inner ear is composed of 3 canals filled with fluid that sense our position in 3D, which helps us keep our balance. Just like a gyroscope that tells our brain the position of our head in real time. The superior canal is one of those canals.

Overlying the superior canal is one of our bones, called the temporal bone. In SCDS that bone is thinner than usual since it hasn’t developed properly.

Through a mechanism too complicated for this article, the symptoms of SCDS develop due to this bone defect.

How does it feel?

There are typical symptoms people with SCDS may experience:

1. Autophony – This means the person can hear their own speech or other noises inside their body (such as the heartbeat, eye movements, chewing, etc.) very loudly.
2. Dizziness/vertigo – Problems with balance, since, as mentioned above, our inner ear is responsible for keeping our balance. (You can learn about head spinning in our article published a while ago).
3. Tullio phenomenon – In this case, sound can cause the patient to lose balance. Sound can also cause rapid movement of the eyes, called a nystagmus.

These are the main symptoms, although there are others as well. This fascinating video shows a true story:

www.youtube.com/watch?v=f6vAkdGw8T4

How is it discovered?

One way is doing a CT scan of the temporal bone, which will show its thinning (or dehiscence).

There are other ways. Among them is the method shown in the Grey’s Anatomy episode, called a videonystagmography (VNG). In this method, hi-tech video goggles with infrared cameras are worn while doing things that can provoke rapid eye movements in the condition.

How is it treated?

The condition can be treated by a surgery, which repairs the gap in the temporal bone. Another surgical method is plugging the superior canal.

What happens after treatment?

The success rate of the operation is quite high. Most patients don’t have their old symptoms after the operation.

The bottom line – How do I avoid it?

There is no known way to prevent the condition.

If you happen to have SCDS, there’s a support group for you.

Related posts:

1. Meniere’s Disease – From Head Spinning To Hearing Loss
2. Familial Mediterranean fever – Fever and Inflammation Inside The Body
3. As Seen on Grey’s Anatomy – Tethered Spinal Cord Syndrome

Summer’s going away, at least around these parts of the woods. Many of you may be going to the pool to enjoy what’s left of it. Read on to find out about otitis externa – an ear infection you can catch while swimming there.

Danger level: Medium

What is it?

Otitis externa is an inflammation of the outer part of the ear causing earache.

Who gets it?

In the US, otitis externa happens to 4 out of every 1000 people each year.

It’s most common in swimmers, divers, and others whose ears are exposed to water for a long period of time.

It can be seen in all ages, but is most common around 7-12 years.

What causes it?

Our ear has an inner and an outer part. The outer part is the one spanning from the ear you can see, inside until the eardrum. The inner part is the part which comes after the eardrum. When your outer ear gets inflamed, the condition is called otitis externa. When the inner part gets inflamed, it is called otitis media, which we will cover in a future article.

Our ear is built so that infections will not occur in it. To prevent infections from happening, it has a few things going for it:

1. Cerumen (earwax) – This is a substance which created an acid environment in our ear. This repels bacteria and prevents infection. It also repels water, and collects debris in the ear, moving it out of the ear.
2. The ear’s shape – Our ear canal has a downward direction. This helps us get rid of water accumulating in the ear.

In swimmer’s ear, bacteria overcome these defenses and cause an infection and inflammation in our ear. How can they do that?

1. Swimming a lot (or getting a lot of water in your ears for other reasons) thins out the earwax, making it less protective.
2. A breakage of the skin can happen – This can happen when using a cotton swab (Q-tip) to clean the ear. It is recommended that you don’t clean your ears using a cotton swab, as this can expose you to an infection. Believe it or not, our body gets rid of the excess earwax by itself, and you really don’t need to mess with it.

How does it feel?

Swimmer’s ear can cause pain. The pain may get worse by touching the earlobe or when chewing.

Other things which can be felt include itching, a feeling of fullness in the ear, a yellow discharge from the ear, redness in the ear canal or skin of the ear.

Otitis externa (swimmer’s ear). Photo by Dale Miller.

How is it discovered?

Usually, a physical exam in which the doctor will look at your ear canal with an otoscope to see that it’s red and swollen.

How is it treated?

Otitis externa is treated using eardrops which contain antibiotics (which fight the germs causing the infection) and sometimes also steroids (which lessen the inflammation in the ear).

What happens after treatment?

Most people improve within 48-72 hours of starting the antibiotics.

If not treated properly, two things can happen:

1. Chronic otitis externa – This is defined as an infection which lasts more than 4 weeks or happens more than 4 times a year.
2. Necrotizing otitis externa – This usually happens to diabetics and older people. In this case, the infection spreads to the bones around the ear, which can also damage nerves in the area and may lead to death.

The bottom line – How do I avoid it?

1. Keep your ears dry – Dry them after swimming or showering. You can do this by shaking your head to remove water from the ear canal, or using the corner of a towel.
2. Don’t use any foreign objects in your ear – And yes, this includes cotton swabs. Also fingernails may damage the ear canal, so don’t poke fingers inside to clean the ear.

This video, featuring Dr. John Hong of NBC, sums it all up:

Related posts:

1. Labyrinthitis – An Ear Infection Causing Loss of Balance
2. 8 Home Remedies for Summer’s Problems
3. Superior Canal Dehiscence Syndrome – Hearing Sounds Inside Your Own Body (As Seen on Grey’s Anatomy)

Danger level: Medium

What is it?

Labyrinthitis is an inflammation inside your ear, which can cause loss of balance and problems hearing.

Who gets it?

Labyrinthitis can be caused by a virus or bacteria. Usually, when it’s caused by a virus, it appears at ages 30-60 and is more rare in children. If bacteria are the cause, it usually appears in children younger than 2 years.

What causes it?

As explained in the article about vertigo, there are 3 systems responsible for giving us balance: The eyes, the joints and muscles, and the ears.

Our ears have 3 parts: The outer ear – which is what you see hanging one the side of people’s heads until our eardrum; the middle ear – is the part behind our eardrum, responsible for transmitting the sound; and the inner ear, which is responsible both for processing sound and for our balance.

The labyrinth is the part in our inner ear responsible for sound processing and balance.

The labyrinth – this structure is in charge of our balance.

In labyrinthitis, the labyrinth gets swollen and inflamed. Usually it’s caused by a viral infection, but bacteria can cause it as well. Often it comes after an upper respiratory infection (such as a cold or flu).

For a more detailed explanation (including a nice animation) you can watch this video:

How does it feel?

There are some things which appear in labyrinthitis:

1. Vertigo – In vertigo, you feel like the world is spinning around you. The vertigo starts suddenly, without a warning. Usually people with vertigo prefer to not move, and keep their eyes closed. Otherwise, they might feel nauseated and want to vomit.
2. Hearing loss – That loss usually goes away after the inflammation has passed.
3. Tinnitus – Tinnitus is the sound of ringing in your ears. It can be annoying, but it, too, passes after the inflammation subsides.

These symptoms tend to go away after a few days or weeks.

How is it discovered?

Usually, labyrinthitis is diagnosed by hearing your story and by a physical exam. The exam can include hearing tests, and an eye test to see if you have an uncontrollable flickering of the eyes, called nystagmus, which is common during labyrinthitis.

If the doctor suspects another cause for your vertigo, they may perform an MRI or CT scan to rule them out.

How is it treated?

In most cases, no treatment is needed, as labyrinthitis goes away on its own. If bacteria caused it, you may need to take antibiotics, but if the cause is viral, antibiotics will not help (since they don’t treat viruses, only bacteria).

While recovering, your doctor may give you drugs to help with the nausea and vomiting.

What happens after treatment?

In a few days to a few weeks, the symptoms are usually completely gone. Hearing loss may take more time to heal, though. If the cause is a virus, the hearing loss tends to go away, but with bacteria, some level of hearing loss may stay for good.

If you have an infection your airways (such as a cold) or an ear infection, treating them on time might prevent labyrinthitis from developing.

Related posts:

1. Meniere’s Disease – From Head Spinning To Hearing Loss
2. Head Spinning (Vertigo) – What Causes It?
3. Otitis Externa (Swimmer’s Ear) – Why You Should Never Use Q-Tips

Danger Level: Medium

What is it?

Meniere’s Disease is an ear problem which causes hearing loss, ear ringing and dizziness.

Who gets it?

Usually people between the ages of 40-60, but it can come at any age. There are some factors which increase the risk of having the disease:

1. A family member with the disease
2. Some allergies
3. Injury to the head or to the ear
4. Syphilis (rarely)

This disease happens to 50-200 out of 100,000 people, and most of the time involves only one ear.

What causes it?

Our ear is divided into 3 parts: The outer ear, middle ear, and inner ear. Meniere’s disease is a disease of the inner ear.

Our inner ear is responsible for two things: Hearing (naturally), and also balance. There is a fluid inside the inner ear, called endolymph, which helps it work properly. In Meniere’s disease, there is too much endolymph, which causes problems with hearing and balance.

The inner ear is what lies behind your eardrum.

How does it feel?

The disease comes in attacks, which last for up to 24 hours. The main feature of these attacks is a dizziness called vertigo. The main difference between the dizziness we all know (feeling faintish and queasy) and vertigo is that in vertigo it feels as if the world is turning. This has to do with the problems the ear is having keeping our balance. With that comes nausea and vomiting, and problems keeping straight.

The second feature is something called tinnitus, or ringing in your ears.

The third and last feature is hearing loss. First it comes in attacks, but with time it worsens.

How is it discovered?

Meniere’s disease is what’s called in medicine a “diagnosis of exclusion”. This means that once other diseases that are easier to diagnose have been ruled out, we can come to the conclusion that a patient has meniere’s. In order to rule out these diseases an MRI picture can be taken, and also a hearing check called an audiogram will be performed.

How is it treated?

The treatment for meniere’s can be divided in two:

1. Symptomatic relief: These are medications that help ease the attacks. They include drugs against nausea, diuretics (these are drugs that cause the body to urinate more fluid. As mentioned above, there is excess fluid in the inner ear). For this reason, sometimes a low-salt diet is tried, which should cause less fluid to accumulate in the body, including the ear.
2. Direct measures: If all of this doesn’t work, a drug named gentamicin is injected to the ear, which destroys the organ inside the ear which is responsible for balance. Another option is a surgery, that does the same thing mechanically. Both are permanent and irreversible, and therefore are a last resort.

What happens after treatment?

The disease varies between people: some will have severe attacks, while others may have a milder disease. In some people the disease may disappear altogether, while in others it can cause a permanent hearing loss.

Here’s a woman that was actually treated successfully:

In most cases, the reason for the disease is unknown. But, as written above, sometimes the disease is caused by injuries to the head. Therefore, it’s a good idea to wear a helmet when bicycling, riding a motorcycle, playing baseball or other activities which can end in an injury to the head.

Also, since sometimes allergies can be related to Meniere’s, treating your allergies might help.

For a personal perspective on Meniere’s disease, visit the My life and Meniere’s disease blog.

Related posts:

1. Head Spinning (Vertigo) – What Causes It?
2. Labyrinthitis – An Ear Infection Causing Loss of Balance
3. Superior Canal Dehiscence Syndrome – Hearing Sounds Inside Your Own Body (As Seen on Grey’s Anatomy)