Ear disorders

[Anatomy and Physiology] [Cholesteatoma] [Chronic Ear Infections]

[CSF (Spinal Fluid) Leakage] [Diving and Ear Problems] [Hearing Loss]

[Otosclerosis and stapedectomy] [Facial Nerve Disorders] [Sudden Hearing Loss]

[Superior Canal Dehiscence] [Meniere’s Disease] [Surfer’s Ears] [Tinnitus] [Vertigo]

Anatomy of the Ear

How Sound is Heard:

– Sound, which is transmitted as sound waves (vibration of the air), enters the ear canal and reaches the eardrum.
– The sound waves lead to the vibration of the eardrum, which also vibrates the small bones behind the ear drum.

– The vibration motion of the bones makes the fluid in the inner ear organ of hearing or “cochlea” to vibrate.

– The vibration waves in the inner ear fluid causes the sensory (hair) cells in the inner ear (cochlea) to bend. The hair cells change the movement into electrical signals.

– These electrical signals are transmitted through the hearing (auditory) nerve and up to the brain, where they are interpreted as sound.

Photo of a normal ear drum:

Anatomy of the Ear

External Ear: The ear (external auditory) canal is covered by skin. The skin closer to the outside of the ear is thick, has hair, and produces ear wax (cerumen). This thick skin covers cartilage. The skin covering the ear canal that is further inside is very thin and overlies bone. The ear drum (tympanic membrane) is located at the end of the ear canal. The ear drum seals the outside (external ear canal) from the inside (middle ear). Swimmer’s ear (external ear infection, aka otitis externa) and exostoses are conditions that affect the external ear canal.

Middle Ear: The middle ear is the space behind the ear drum. It is covered by a lining that is similar to the lining of the nose. Normally, there is no skin inside of the middle ear space. The contents of the middle ear include the 3 little bones (ossicles) that take the sound from the ear drum to the inner ear (cochlea). Chronic ear disease generally affects the middle ear. 

Eustachian Tube: The middle ear space is connected to the back of the nose via a tube called the Eustachian tube. The Eustachian tube allows fluid/mucus to drain out of the ear and allows air to enter behind the ear drum. The middle ear space is also connected to the air space within the mastoid bone (the bone behind the ear). Due to this connection, diseases of the middle ear will generally affect the mastoid bone too. 

The nerve that moves the face (the facial nerve) and the nerve that supplies the taste in the front part of the tongue (chorda tympani nerve) travel through the middle ear on their way to the face/mouth.

– Mastoid Bone: The bone behind the ear drum has multiple air pockets within it. These air pockets are connected to the middle ear. The air pockets (called air cells) are lined with the same lining as the middle ear. Diseases of the middle ear will generally affect the mastoid as well.

– Inner Ear: The inner ear includes the hearing (cochlea) and the balance organs (semicircular canals, utricle, and  saccule). Therefore, any disease that affects the inner ear causes hearing and balance problems.

The inner ear is encased in bone and has two areas that are covered by membranes. These two “windows” are areas of potential communication with the middle ear. The two areas are called the round window and the oval window.

The inner ear is filled with two kinds of fluid, the endolymph and the perilymph. The leakage of perilymph as a result of trauma occurs from the oval or round windows and causes hearing and balance dysfunction. An increase in the endolymph pressure leads to the hearing and balance problems in Meniere’s disease. [Back to the Top]

Cholesteatoma

What is cholesteatoma?

Simply put, cholesteatoma is skin in the wrong place. Skin is a normal part of the ear canal and the outside part of the ear drum (tympanic membrane). When this skin grows behind the ear drum or it grows into the bone behind the ear (mastoid bone), it is called a cholesteatoma. This process has the capability to destroy the structures around it. The treatment of a cholesteatoma is its removal by surgery. Close follow-up is necessary to monitor for recurrence.

Presenting Symptoms

Cholesteatomas most commonly present with hearing loss and drainage from the ear. In advanced stages, facial paralysis or dizziness (vertigo) can be a presenting sign.

Causes

There are 3 causes of cholesteatoma

1. A cholesteatoma that develops due to a retraction in the ear drum. When ear disease is  present for a long time, the ear drum can slowly get retracted (suctioned into the middle ear). Over time, the skin from the outside grows into the space behind the ear drum and into the mastoid bone. The cause of this retraction is a dysfunction in the eustachian tube (which allows air to get from the back of the nose to the space behind the ear drum).

2. A cholesteatoma (skin) can grow into the space behind the ear drum through a perforation in the ear drum.

3. A cholesteatoma can be present since birth (congenital). This means that some skin existed behind the ear drum from birth.

How is a Diagnosis Made?

Generally after a complete history and physical exam, some tests need to be performed to diagnose and plan for the treatment of cholesteatomas. A test of the hearing (audiogram and tympanogram) and CT scan (special x-ray) of the temporal bone (ear bone) is necessary for diagnosis and planning of treatment.

Complications

As the cholesteatoma grows, it destroys the bone around it. The structures that can be invaded by the cholesteatoma include the little bones of the ear (ossicles), which causes hearing loss, inner ear (which would cause complete deafness and dizziness), the facial nerve (which would cause facial paralysis), or invade the bone covering the brain and cause meningitis. If left untreated, these complications will occur with time.

Treatment

Initial treatment with antibiotic drops help in settling the infection that causes the drainage. However, cholesteatomas can only definitively be treated by removal using surgery. This generally requires an incision inside the ear canal or in the back of the ear to get access to the area of the cholesteatoma. The name of the surgery is tympanoplasty (removal of disease from middle ear) with mastoidectomy (removal of disease from the mastoid bone). Commonly, a second surgery is required after 6 to 12 months to ensure that the cholesteatoma has not recurred and for reconstruction of hearing. Due to the presence of infection at the first surgery, hearing reconstruction is generally performed at the second surgery.

Minimally Invasive Treatment of Cholesteatomas

The use of Otoendoscopy (small angled cameras) has been shown to significantly reduce the chance of recurrence after surgery for cholesteatomas. The chance of recurrence of cholesteatomas was reduced from nearly 50% to 5%. In a study that has been published by Dr. Oliaei and his colleagues, it was found that using otoendoscopy will reduce the need for more radical surgery. Areas that are around corners that are usually not visible with a microscope can be visualized and cholesteatomas can be removed from those locations. Use of operating microscopes, endoscopes and lasers have made this surgery safer, less invasive and more thorough. [back to the top]

Chronic Ear Infections

 

Chronic ear infections, also called chronic otitis media is an inflammation or infection of the middle ear. Chronic means recurring or persistent.

 

Causes, Incidents and Risk Factors

Inflammation or infection of the middle ear occurs when the eustachian tube to that ear is blocked. The eustachian tube is the passage from the back of the nose to the space behind the ear drum. Chronic otitis media occurs when the eustachian tube becomes blocked repeatedly (or remains blocked for long periods) due to allergies, multiple infections, ear trauma, or swelling of the adenoids. Bacteria from the back of the nose (nasopharynx) will go through the eustachian tube and cause an infection in the space behind the ear drum (middle ear). [see anatomy]

 

When the middle ear is acutely infected with bacteria (or occasionally, viruses) it is called acute otitis media. A chronic ear infection may be the result of an acute ear infection that does not clear completely, or the result of recurrent ear infections. The infection may spread into the mastoid bone behind the ear (mastoiditis), or pressure from fluid build-up may rupture the eardrum or damage the bones of the middle ear.

 

A chronic ear infection may be more destructive than an acute ear infection because its effects are prolonged or repeated, and it may cause permanent damage to the ear. However, a chronic, long-term infection may show less severe symptoms — so the infection may remain unnoticed and untreated for a long time.

 

Ear infections are more common in children because their eustachian tubes are shorter, narrower, and more horizontal than in adults. Chronic ear infections are much less common than acute ear infections.

 

Symptoms

– Ear pain or discomfort, earache

– Usually mild

– May feel like pressure in the ear

– Pus-like drainage from the ear

– Hearing loss

Note: Symptoms may be continuous or intermittent, and may occur in

one or both ears.

Signs and Tests

An examination of the ear may show dullness, redness, air bubbles, or fluid behind the eardrum. The eardrum may show drainage or perforation (a hole in the eardrum). The eardrum may bulge out or retract inward.

Cultures of drainage may show bacteria. These bacteria may be resistant or harder to treat than the bacteria commonly involved in acute ear infection.

A CT scan of the ear bones may show spreading of the infection beyond the middle ear into the mastoid bone (bone behind the ear).

 

Treatment

Treatment is focused on relief of symptoms and cure of the infection.

Oftentimes, children with otitis media are treated with antibiotics. If the child has multiple infections (generally 6 infections per year), then small tubes are placed in the ear drum to reduce the number of subsequent infections. These tubes will stay in for about 6 months to a year and then fall out on their own. Generally, 80% of children will only need to have the tubes placed only once. Occasionally surgical opening may be made in the eardrum (myringotomy) to allow fluid to drain without placing tubes.

 

Ear tube placement is considered when there is:

     – 6 infections in one year
     – Hearing loss from multiple infections that has caused delay in speech    
         development
     – Fluid behind the ear drum that is present for 6 months in one ear or 4
         months in both ears
     – Significant hearing loss in both ears from persistent fluid behind the ear
         drums

 

Most children will have temporary and minor hearing loss during and right after an ear infection, because fluid can linger in the ear. Although this fluid can go unnoticed, it can cause significant hearing problems in children. Any fluid in the ear that persists longer than 12 weeks is cause for concern — in children, hearing problems may cause speech to develop slowly. Permanent hearing loss is rare, but the risk increases the more infections a child has.

 

The tubes fall out on their own in 6-12 months. The tubes have to replaced in 20% of children because of continued infections. Surgical removal of the adenoids may be necessary to take away the source of the bacteria which cause ear infections if the tubes have to be replaced.

 

Occasionally, ear tubes alone do not resolve the infection that has caused significant destruction or involves the bone behind the ear canal (mastoid bone). In these cases, surgery (tympanoplasty +/- mastoidectomy) may be necessary to debride infected tissue and bone, reconstruct the middle ear bones that have been destroyed, and repair holes (if any) in the ear drum.

 

In adolescents and adults, ear tubes can be placed under local anesthesia in the ENT office. Small holes (perforations) in the ear drum (tympanic membrane) can also be repaired in the office using novel techniques. Many practices now have the capability to do this. Office procedures that are performed under general anesthesia are minimally invasive in that they avoid the risks of general anthesia and involve little to no downtime. [back to the top]

CSF leak

What is CSF? How does it leak?

Cerebrospinal fluid (CSF) is a colorless fluid that cushions the brain, protecting it from mechanical trauma and infections. CSF leaks are caused by small tears in the barrier between the brain and roof of the nasal sinuses or the bony roof of the ear.

What are the symptoms of CSF leak?

CSF leak can sometimes be very difficult to diganose. It can present with symptoms of ear fullness due to clear liquid collecting behind the ear drum. At other times CSF can drain through the Eustachian tube into the nose, or if the source of the leakage is at the roof of the nose, then patients can have clear drainage from the nose that can be constant or intermittent.

lAlthough leaks can occur due to trauma, many cases are spontaneous and without any apparent cause.

The major risk of leaving a CSF leak unrepaired is a life threatening infection of the lining of the brain called meningitis, which requires hospitalization and intravenous antibiotics to treat. S

Diagnosis

A complete evaluation including a history, medical history, and examination are first performed. Most commonly, you will be asked to collect, if possible, a sample of the fluid in a sterile container to perform a laboratory test that confirms whether the fluid is CSF.  A CT scan and/or MRI of the sinuses are typically recommended to help identify the exact location of the defect.

Treatment

Although, in many cases a specifically trained Ear Nose and Throat surgeon can repair a CSF leak without assistance, often a neurosurgeon is consulted to help with management including placement of a lumbar drain which is a drain placed in the back to help drain the CSF and decrease the pressure of the leakage.

Surgery for CSF leak of the nose is done using minimally invasive, incisionless endoscopic approach through the nostrils. Magnetic or optical image guidance are used to guide the surgery throughout and make it safer than ever before. Once the leak is identified, it is repaired using the patient’s own tissue, which is often obtained from within the nose. Typically, patients require 2-3 day hospital stay.

CSF leaks of the ear can be repaired in the majority of cases via a minimally invasive surgical technique which avoids opening the skull (craniotomy) or pushing on brain tissue. This is done using a microscopic technique with a hidden incision behind the ear. The surgery is done on outpatient basis with under 23 hour observation post-operatively. In rare cases, this technique is not adequate and a neurosurgeon will need to be consulted to help with a craniotomy for better access. [Back to the Top]

Diving and Perilymphatic Fistula

Overview

The inner ear (hearing and balance organs) is encased in bone and communicates with the middle ear (the space behind the ear drum) in two locations. These two areas are covered with a thin membrane and are termed the round and oval windows. If for some reason a communication develops between the inner ear fluid and the middle ear space, it is called a perilymphatic fistula.  See Anatomy of the Ear for reference.

The leakage of fluids from the inner ear can cause hearing loss (either mild or severe) which may be fluctuating, ringing in the ears, and dizziness. These symptoms are worsened with any pressure change.

Once the diagnosis has been established, the treatment is by performing a surgery and repairing the site of the leak.

Presenting Symptoms

Patients with perilymph fistula presents with a sudden or fluctuating hearing loss and/or dizziness. The disturbance in balance may be a sensation of rotation (vertigo), lightheadedness, disequilibrium, motion intolerance, or any combination of the above. The symptoms are especially seen with coughing, straining, loud noise, bending over, or with pressure changes, such as in an elevator.

Causes

Perilymph fistulas are most commonly caused by sudden pressure changes such as in SCUBA  diving, ascent or descent on a plane, weightlifting among others. Patients who have undergone a stapedectomy operation, are prone to developing perilymph fistulas.

How is a Diagnosis Made?

Generally after a complete history and physical exam, some tests need to be performed to diagnose the cause of facial paralysis. A test of the hearing (audiogram and tympanogram) and a balance test (electronystagmography (ENG)), and other specialized hearing tests (electrocochleography (ECoG)) are performed. A CT scan (special x-ray) of the temporal bone is necessary to ascertain the diagnosis.

Treatment

Treatment of perilymph fistula once the diagnosis is made is surgical. While bed rest and non-surgical treatment has been advocated for the treatment of this condition, which risks further deterioration of hearing and balance function. The surgical procedure, which takes about 30 minutes, involves lifting up the ear drum through the ear canal and patching the round and oval windows.

Recovery

The recovery from perilymphatic fistula surgery involves two weeks of no strenuous activity, no lifting over 20 lbs, sleeping with the head of the bed elevated, and no straining. Normal activities may be resumed after that. The chance that the dizziness will improve after surgery is very high, although the likelihood of hearing recovery is low.

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Hearing Loss

Hearing loss has a multitude of causes which can occur anytime between birth and adulthood. The most common types of hearing loss are that associated with the normal aging process or excessive noise exposure. These types of hearing loss are often readily diagnosed and treated with a hearing aid and discussion of preventive measures. A recent study by Dr. Oliaei and colleagues underlined the importance of early measures to prevent hearing loss by demonstrating presence of children’s toys capable of producing loud sounds that may cause permanent hearing changes. However, it is very important to rule out a more serious cause for the loss of hearing.

Progressive hearing loss during adulthood may be part of normal aging or signify a more serious process. Disorders such as infections or tumors can cause hearing loss which makes it important for a patient to see a physician to rule out these causes before a hearing aid is prescribed. Symptoms such as ear pain or drainage, ringing or roaring in the ear (tinnitus), or dizziness and balance problems can signal a more serious cause for the hearing loss and should be investigated by a physician.

Occasionally a surgical procedure can correct or improve the hearing loss without the need for a hearing aid. New advances in hearing aid technology have made some hearing aids impossible to be seen from the outside and allow tailoring of the sound to the patient’s specific hearing loss. Occasionally a more affordable assisted listening device such as an amplified telephone or headphone are all that is needed to improve a person’s quality of life.

Whatever the cause of hearing loss, and whenever it occurs during life, it is important to seek the diagnostic and treatment skills of ear, nose and throat physician to exclude serious causes for hearing loss such as tumors or infections. Once these serious concerns are set aside, attention can be given to addressing the hearing loss via state of the art hearing aid technology that has helped improve the lives of those suffering from hearing loss.

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Otosclerosis and Stapedectomy

Otosclerosis is a condition of the bone of the inner ear where the bone of the inner ear grows and stiffens the stapes bone (the third bone of hearing). This stiffening causes loss of hearing.

Causes

The cause of otosclerosis is unknown and generally considered to be a hereditary condition.

Anyone can get otosclerosis. 10% of Caucasian males, and 18% of Caucasian females have otosclerosis. Of those, 10% will have symptomatic hearing loss. This means that in the white population, 1% of males and 2% of females will have otosclerosis to the degree that causes significant hearing impairment. The disease progresses at a faster rate during pregnancy. It tends to be less common in other races.

How is a Diagnosis Made?

Generally after a complete history and physical exam, some tests need to be performed to diagnose otosclerosis. A test of the hearing (audiogram and tympanogram) and CT scan (special x-ray) of the temporal bone (ear bone) is necessary for diagnosis and to rule out other causes of the hearing loss.

Treatment

There are 3 options for the treatment of otosclerosis.

1. Do nothing. Otosclerosis is not a fatal disease, if the hearing loss does not bother you, you do not have to do anything about it. The disease is generally progressive though. So, as time goes on, the hearing will get worse.

2. Use a Hearing Aid. Depending on the nature of the hearing loss, the patient’s medical condition, and patient preference, a hearing aid may be recommended for the treatment of otosclerosis.

3. Stapedectomy. Stapedectomy is a surgery for the treatment of otosclerosis. During this surgery, the 3rd hearing bone (stapes) is removed under the microscope with a laser and replaced with a prosthesis which connects the 2nd hearing bone (incus) to the inner ear. The surgery takes less than an hour and is 90-95% successful in restoring the hearing to the normal (inner ear function) level. The main risk of the operation is a 1% chance of permanent hearing loss and persistent dizziness.

 

What option is best for you? 

A comprehensive evaluation of your hearing and a consultation with an ear, nose throat specialist to make the diagnosis and discuss treatment options is necessary to decide the best treatment option.

New Innovations in the Treatment of Otosclerosis

Use of Laser technology to activate the “SMART” prosthesis as well as the use of antiinflammatory agents delivered directly into the inner ear, reduces likelikhood of complications and makes this procedure safer than ever before.

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Facial Nerve Disorders

Facial paralysis, often referred to as Bell’s palsy, is a weakness or paralysis of the nerve that control facial expression on one side of the face. The disorder results from an insult  to the nerve that stimulates the movement of the facial muscles. This in turn results in the weakness or complete paralysis of the face on one side.

Facial paralysis is most often caused by a virus infection of the facial nerve. However, other conditions such as tumors, other infections, trauma, among others. The condition is more frequent in diabetics and pregnant women.

Bell’s palsy caused by a virus infection most commonly resolves on its own in several weeks or months. When patients present within a week after diagnosis, corticosteroid (prednisone) and anti-viral medication for 1-2 weeks, to increase the likelihood of recovery. Therefore early evaluation and management by an Ear Nose and Throat Physician is crucial in achieving the best outcome.

Presenting Symptoms

Facial palsy presents with weakness on one side of the face. About half of patients with Bell’s palsy present with pain behind their ear or on their face, which may precede the paralysis by a few days. Tearing on the side of the paralysis is common and so is an altered taste on the same side of the tongue. Some patients may experience intolerance to loud noises on the same side as the paralysis.

How is a Diagnosis Made?

Generally after a complete history and physical exam, some tests need to be performed to diagnose the cause of facial paralysis. A hearing test (audiogram) and usually a test of the facial nerve (electroneurography, ENoG) is performed. Imaging of the temporal bone, brain, and the face may be warranted to evaluate for presence of tumors.

Other Facial Nerve Disorders

Facial Spasms

Facial spasms, or hemifacial spasm are disorders characterized by intermittent spasms of facial muscles, the whole side of the face, or the eye. This condition can be caused by sensitivity of the nerve or pressure from a blood vessel on the brain. This condition is treated using BOTOX injections or by doing a surgery to take off the blood vessel that is pressuring the nerve. This surgery is generally performed in conjunction with a neurosurgeon.

Ramsay Hunt Syndrome (also called Herpes Zoster Oticus)

This condition occurs when the shingles virus affects the facial nerve. Patients will often have pain in the ear and some blisters around the ear or in the ear canal. This disorder is treated in the same way as Bell’s palsy. The likelihood of recovery to normal function in this disorder is 50-60% compared to Bell’s palsy which generally recovers to normal function in 80-90% of individuals.

Facial Neuroma

Facial neuroma (also called facial schwannoma) is a tumor of the facial nerve. This tumor is slow growing and over time can cause facial paralysis. The treatment of this tumor is by removal of the tumor using surgery. The nerve is often replaced with another nerve from the leg or the neck. The recovery of the function is never complete, but tone can be restored with some movement. Sometimes if the tumor is discovered and it is not causing facial paralysis, the tumor may be observed over time and removed only after it causes facial paralysis.

Facial Nerve Tests

Depending on the disorder and the time of onset, several tests of facial nerve function or imaging tests, such as CT or MRI scans may be obtained. The facial nerve tests include the electroneuronography (ENoG test). This test allows the specialist identify the extent of the injury to the nerve or onset of paralysis in the first 21 days after the injury. The facial nerve electromyography (EMG) test is meant to identify the prognosis and progress of recovery after 21 days from the injury or onset of paralysis.

Treatment

Treatment of the facial paralysis depends on the cause of the facial paralysis. In cases of infections, antibiotics are prescribed and surgery may be necessary. In other cases, steroid medication (prednisone) is prescribed in combination with an anti-viral medication. In cases of tumor or paralysis resulting from resection of tumors of head and neck, the treatment usually consists of one or more of various facial reanimation procedures.

The most important element of the treatment of facial paralysis is the care of the eye. Although there is generally tearing on the side of the paralysis, the surface of the eye gets dry from the inability to close the eye entirely. Therefore, initially a combination of artificial tears and a lubricating ointment is used to keep the eye moist and the eye is taped closed when sleeping. For persistent or long-term paralysis,span> canthoplasty and/or gold weight placement to the upper eyelid should be considered. Many of these procedures can be performed in the office in selected cases under local anesthesia.

Botox (Botulinum Toxin A) injection is a new treatment that helps reduce hyperactivity and asymmetry of the face.

Recovery

The recovery from facial paralysis is generally dependent on the cause of the paralysis. While recovery from facial paralysis caused by tumors is unlikely, Bell’s palsy or idiopathic facial paralysis patients have an 85% chance of complete recovery. The chance of recovery is less in patients who have diabetes. Again, rapid referral and consultation with an Ear, Nose and Throat specialist is prudent in these cases.

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Sudden Hearing Loss

Sudden hearing loss is an EAR NOSE AND THROAT EMERGENCY! This condition, which is caused by a virus infection or a small stroke of the inner ear comes on suddenly.

Symptoms

Most commonly, patients state that they wake up and notice that their ear is plugged and that they cannot hear on the phone using that ear.  Oftentimes, patients mistake this for an ear plugging as a result of a cold or allergies. One way to test to see if you have sudden hearing loss is to hum. If you only hear the humming in the opposite ear, then you may have sudden hearing loss and should immediately (within 24-48 hours) seek treatment by an Ear Nose Throat Surgeon with special training in management of sudden hearing loss. If you can hear the humming in the ear that is plugged, then it is the routine ear plugging from a cold or allergies, or perhaps fluid in the ear.

Tests

A hearing test is required to make the diagnosis of sudden hearing loss. Since acoustic neuromas (a type of benign brain tumor) occur in 3-10% of patients with sudden hearing loss, an MRI is obtained to make sure that you don’t have a an acoustic neuroma. Sometimes, testing of the blood may be performed to rule out certain conditions including Lyme disease, some rare infections, or autoimmune conditions.

Treatment

Treatment consists of a course of high dose steroids administered directly into the middle ear as well as a course of oral steroids. Studies have shown that this combination treatment is more effective than either treatment alone. Additionally, most experts including Dr. Oliaei agree that the earlier the treatment starts, the better the chance of recovering hearing. The treatment should start within the first 2 weeks after the onset of the hearing loss, idealy within a few days after onset.

Steroid injection into the middle ear is done using a small needle to place the medication behind the ear drum. The special steroid medication (methylprednisolone) will then enter the inner ear and improve the likelihood of hearing recovery.

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Superior Canal Dehiscence

Superior canal dehiscence is characterized by a combination of a few of the following symptoms:

  – Sensation of hearing their own voice in their ear (autophony)

  – Feeling of a plugged ear which doesn’t resolve with popping the ear

  – Dizziness that occurs with loud noise or with pressure. The dizziness is 
    usually very short lasting (a few seconds) and there is a sensation that   
    the visual field rotates a bit with coughing, sneezing, lifting something
    heavy, or with loud noise.

  – Hearing  loss – most commonly conductive but can be mixed. A 
    characteristic of the hearing test is that the inner ear hearing (bony
    thresholds) are better than normal, but when the hearing is tested
    through the ear canal, there is a hearing loss.

  – A feeling that one can hear internal sounds. For example, patients often
    have a sensation of hearing their heartbeat or sometimes even hearing
    their eyes move.

What Causes It?

The bony roof of one the canals of the inner ear (superior canal – the top-most canal) gets eroded away probably because of pressure and pulsations of the brain. Sometimes this can occur after a trauma that causes a sudden pressure change in the brain which can break the thinned bone. This causes the inner ear fluid compartments to have an outflow tract which leads to the symptoms of dizziness, hearing loss, and especially the sensitivity to sound (which causes vertigo).

How Do We Diagnose It?

The best way to diagnose it after clinical suspicion is to perform a CT scan of the temporal bone and confirm the diagnosis with a VEMP test. This scan has to be obtained in a very special way. It has to be done using slice thickness that is under 1 mm. If the scan is done with 1 mm thickness or larger, then it may show a dehiscence when it may not be present.

The VEMP test (vestibular evoked myogenic potential) is a test of a particular part of the balance organ. This test involves sending sound to the ear while the neck muscle is under tension. A lower threshold and higher amplitude on the VEMP test in the presence of a CT finding is highly suggestive of a superior canal dehiscence.

Treatment Options

There are 2 options for dealing with this problem.

1. do nothing and avoid the problems that cause the symptoms. For example, some of our patients have somewhat sedentary lifestyles and do not engage in activities that cause dizziness and they can put up with the minor symptoms.

2. Surgery for repair of a superior canal dehiscence. As a general rule, these surgical techniques are invasive and often require an opening in the skull performed by a neurosurgeon (craniotomy) so they are reserved for severe cases.

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Meniere’s Disease

Meniere’s disease is a disorder caused by increased fluid pressure in the inner ear. The fluid chamber of the inner ear that has increased pressure is called the endolymphatic chamber and therefore the disease is also known as endolymphatic hydrops (increased fluid pressure in the endolymphic chamber). The exact cause of the disease is unknown, though genetic and environmental causes are thought to contribute to its development.

Symptoms

There are 4 main symptoms associated with Meniere’s disease. These are: episodic vertigo (spinning dizziness), tinnitus (roaring or ringing in the ears [usually just one ear]), fluctuating hearing loss, and pressure sensation in the ear. An episode of Meniere’s usually starts with a pressure sensation in the ear with increasing roaring sound in the ear and hearing loss and a sudden attack of vertigo. The vertigo generally lasts at least 30 minutes and may last up to or greater than 24 hours. Once the vertigo stops, the patients generally experience some imbalance which takes days to weeks to resolve. The hearing generally returns, but over time, the hearing and balance function are lost with each attack of the vertigo. Some patients may just have fluctuating hearing loss without vertigo or episodic vertigo without hearing loss. These are termed cochlear hydrops and vestibular hydrops respectively.

Causes of Attacks

There are some known triggers of a Meniere’s attack, which include, high salt foods, too much caffeine, drinking alcohol, and stress. While the triggers differ between patients, the above 4 triggers are found most commonly.

Disease Course

Approximately 60% of patients with Meniere’s disease stop having attacks after a few years (also called the disease burning out). The rest continue to have problems. Of these, a high percentage (60%-80%) are controlled with a very strict control of their diet and lifestyle changes and sometimes medications. The strict diet includes limiting your diet to a daily sodium of 1500 mg, eliminating all caffeine (not even decaf coffee, which has caffeine), and no alcohol. The lifestyle change includes reduction of stress by biofeedback, meditation, yoga, daily exercise, etc.

Some people (20%-30%) may develop the disease in the other ear after a few years. A small percentage of the patients will continue to have episodes which may occur every day to once a few months or years. When despite maximal medical therapy and lifestyle changes the patients continue to have frequent episodes of Meniere’s disease and the disease is affecting their daily life, then surgery is considered for treatment.

Treatment

The treatment of Meniere’s disease follows a stepwise fashion from diet and stress control to medical treatment to surgical treatment. The dietary/lifestyle changes for treating Meniere’s Disease are discussed above. The next step is taking medications which are believed to decrease the inner ear fluid pressure. The medicines that cause reduction of fluid pressure in the inner ear also make you lose extra water from the kidneys. These medications, called diuretics, include Dyazide, methazolamide, furosemide, among others.

For controlling the dizziness or imbalance, medications such as meclizine (Antivert), Robinul, scopolamine patches, among others is used. These medications decrease the abnormal signal that the  diseased inner ear sends the brain.

The newest treatment for Meniere’s disease is the placement of medications behind the ear drum. In a recent study, over 90% of patients with Meniere’s disease were found to have significant control of their symptoms with intratympanic steroid (anti-inflammatory medications placed behind the ear drum). The injections (using dexamethasone or methylprednisolone [Solu Medrol]) are generally given after a local anesthetic in the office and are repeated every 2 to 4 weeks until the attacks stop.

Most patients with Meniere’s undergoing intratympanic steroid treatment have only required 2-3 injections for full control of their symptoms. We recommend it for patients prior to doing any surgery or in those with a history suggestive of autoimmune Meniere’s disease or those who are unable to take steroids by mouth.

These injections are different from gentamicin injections that cause destruction of the inner ear balance cells. We rarely use gentamicin injections in the ear for Meniere’s disease due to the destruction of the inner ear it causes. Gentamicin injection is reserved for patients with end stage Meniere’s disease with very little hearing or balance function.

Since the advent of intratympanic steroid delivery techniques, surgical treatments have become un-necessary in most cases as they are far more invasive and the results are comparable or inferior to in-office steroid injection techniques.

Surgical Treatment

Surgery for treating Meniere’s disease is used when medical treatment has failed and that the vertigo has become incapacitating. There are essentially three different surgical techniques used: 1. endolymphatic shunt surgery, which helps to decompress the excess fluid in the inner ear, 2. labrynthectomy, or removal of the inner ear and 3. vestibular nerve section or cutting of the balance nerve. Again, with the advent of recent techniques such as middle ear injections of steroids, surgery has become less common.

What About Gentamicin or Steroid Treatment Behind the Ear Drum?

Gentamicin (also called intratympanic gentamicin, putting the medicine behind your ear drum) is an antibiotic that causes the destruction of the inner ear balance organ. The idea behind using this medication is that when the inner ear balance organ on the diseased ear is destroyed, then the patient would not develop vertigo anymore. Treatment with gentamicin works well and can be done in the office, but it has several problems with it. 1. It destroys the inner ear balance function on one side, and if you develop Meniere’s disease in the other ear (20-30% chance), you will have great difficulty with your balance. 2. It has a 10-20% chance that it may cause hearing loss in the ear that is being treated. 3.It does not change the hearing loss, pressure, and tinnitus (ringing/roaring sound) symptoms.

Gentamicin treatment for Meniere’s disease is best for people who have had the disease for a few years and have lost hearing and most balance function in that ear. Generally the second ear will start showing signs of disease in the first 3-5 years after the onset of the disease. If it does not develop by then, it is unlikely to develop. For those who have a significant hearing loss, there is less of a problem if there is some additional loss.

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Surfer’s Ears (Exostoses)

Surfer’s ear is a condition where the bone of the ear canal develops multiple bony growths called exostoses. Over time, this can eventually cause a partial or even complete blockage of the ear canal which can lead to infections and hearing loss.

Symptoms

Surfer’s ear usually presents with an infection of the ear canal or blockage of the ear canal and hearing loss.

Causes

Prolonged exposure to cold water and wind are the cause of Surfer’s ear. Cold water surfers are 6 times more likely to get Surfer’s ear than warm water surfers.

Complications 

When the ear canal is narrowed, water and debris can get trapped behind the narrowing causing infection. These infections of the ear canal (external otitis) are also called Swimmer’s ear. The infections are more difficult to treat in people with surfer’s ears than those with open ear canals. The treatment of the infection will require several visits to an ear nose and throat physician for cleaning of the debris and antibiotic treatment. Once over 90% of the ear canal gets blocked, a significant hearing loss will occur, which can only be relieved by removing the exostoses.

Treatment 

The only way to treat Surfer’s ears definitively would be to remove the bony growths (exostoses). The latest technique for removal of the disease involve use of microscopic techniques and microchisels. This allows an incisionless surgery where the entire procedure is performed through the ear canal. Procedure is now performed on outpatient basis. Traditional techniques have employed a incision behind the ear lobe and were more invasive and required longer healing times.

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Tinnitus (Noise in the Ears)

Tinnitus (tin-i-tus) is the perception of a sound in your ear when there is no noise in the environment. It may sound like a ringing, roaring, clicking, or hissing sound. It can be constant or intermittent.

Tinnitus is generally associated with hearing loss. It can also be a symptom of other problems such as problems with blood vessels or rarely tumors. There are as many as 50 million Americans who have tinnitus. Approximately 2 to 4 million have it so severely that it interferes with their daily activities.

People with severe cases of tinnitus may find it difficult to hear, work, or even sleep.

Causes of Tinnitus

Hearing loss. Most commonly, people with tinnitus have some kind of hearing loss. It is thought that the brain replaces the hearing loss with a perception of a noise.

Loud noise. You may have noticed ringing in the ears after a loud concert or a loud noise exposure. Generally, a loud noise can cause a hearing loss, which in turn causes tinnitus.

Medicine. Many different medicines can cause tinnitus. The most common medicines that cause tinnitus include aspirin, ibuprofen, naprosyn, among others.

Other health problems. Tumors, problems in the heart and blood vessels, jaws, and neck can cause tinnitus.

Diagnosis

First step is to see an ENT and have a hearing test done. Depending on the test results, your ENT may need to obtain imaging of the brain (MRI or CT) to rule out more serious conditions. Once this has been ruled out, then focus can be turned to treatment strategies.

Treatments

  • Neuromonics Device. This new device combines counseling with a music device with songs that are tailored to your hearing loss.
  • Hearing aids. Most people with tinnitus have some degree of hearing loss. Wearing a hearing aid makes it easier for some people to hear the sounds they need to hear by making them louder. The better you hear other people talking or the music you like, the less you notice your tinnitus.
  • Tinnitus Maskers. Maskers are small electronic devices that use sound to make tinnitus less noticeable. Maskers do not make tinnitus go away, but they make the ringing or roaring seem softer. For some people, maskers hide their tinnitus so well that they can barely hear it. The idea is to sound a sound that is bothersome with one that is not. Some people sleep better when they use maskers. Listening to static at a low volume on the radio or using bedside maskers can help. These are devices you can put by your bed instead of behind your ear. They can help you ignore your tinnitus and fall asleep. Other available products include speakers inside a pillow that can play the sound of the ocean or the rain.
  • Medicine or drug therapy. Some medicines may ease tinnitus. These medicines are generally given to reduce the anxiety or depression associated with tinnitus.
  • Tinnitus retraining therapy. This treatment uses a combination of counseling and maskers. You may also use maskers to make your tinnitus less noticeable. After a while, some people learn how to avoid thinking about their tinnitus. It takes time for this treatment to work, but it can be very helpful.
  • Counseling. People with tinnitus may become depressed. Talking with a counselor or people in tinnitus support groups may be helpful.
  • Relaxing. Learning how to relax is very helpful if the noise in your ears frustrates you. Stress makes tinnitus seem worse. By relaxing, you have a chance to rest and better deal with the sound.

What can I do to help myself?

The most important thing about dealing with tinnitus is to not think about it. The more you think about it, the more noticeable it will become and the more it will bother you. The vicious cycle will continue and can bring you to a point of disability. Think about things that will help you cope. Many people find listening to music very helpful. Focusing on music might help you forget about your tinnitus for a while. It can also help mask the sound. Other people like to listen to recorded nature sounds, like ocean waves, the wind, or even crickets.

Avoid anything that can make your tinnitus worse. This includes smoking, alcohol, and loud noise. If you are a construction worker, an airport worker, or a hunter, or if you are regularly exposed to loud noise at home or at work, wear ear plugs or special earmuffs to protect your hearing and keep your tinnitus from getting worse.

If it is hard for you to hear over your tinnitus, ask your friends and family to face you when they talk so you can see their faces. Seeing their expressions may help you understand them better. Ask people to speak louder, but not shout. Also, tell them they do not have to talk slowly, just more clearly.

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Vertigo

Dizziness is a symptom which can describe many different sensations depending on the person experiencing it. Lightheadedness, imbalance, and a spinning sensation are all commonly referred to as dizziness but each can arise from completely different causes. Metabolic, neuromuscular, and cerebrovascular disorders as well as tumors or trauma are common causes for dizziness but each is experienced in a different way and has its own different method of treatment.

A spinning sensation type of dizziness (vertigo) can be divided into two major types: central and peripheral. Central refers to conditions in the central nervous system (brain and spinal cord) which can cause vertigo. These include primarily strokes, tumors or trauma. Peripheral describes causes of vertigo which arise mainly from the ear or more specifically, from the balance nerves and balance organs in the inner ear. Peripheral vertigo is the most common type and can arise from tumors, trauma, infections, migraines, allergies, and inner ear fluid abnormalities. The following includes a description of several more common causes of dizziness that are routinely treated by specially

Benign Positional Vertigo (BPV also known as BPPV)

Characteristics: This is the most common cause of dizziness in ENT clinics. Short lasting spinning dizziness caused by certain head movements.

What Brings on the Symptoms: The spinning dizziness in BPV comes on when patients turn their head one particular way (either left or right). It will most commonly occur when patients are lying in bed and turn.

Duration: The spinning dizziness lasts less than one minute.

Associated Symptoms: No associated hearing loss, tinnitus (ringing in the ears), etc.

What Causes It: The inner ear balance organ is filled with fluid. Certain areas of the inner ear balance organ contains microscopic crystals that for some reason (sometimes from trauma) can become loose and float in the fluids of the inner ear. When the head is turned in a particular direction, the crystals float in the fluid and cause a rippling effect (like a rock thrown in water). This movement of fluids causes stimulation of the inner ear, which causes a sensation of motion and thus dizziness. The spinning stops as the movement of the crystals and the fluid through the inner ear stops.

Treatment: BPV can usually be corrected by performing an office procedure called an “Epley maneuver”.

Migraine Related Dizziness

Characteristics: This is the second most common cause of dizziness seen in ENT clinics. Episodic vertigo or constant sense of dizziness. Associated with light or sound sensitivity. Sometimes, significant motion sensitivity may be present. Patients may have difficulty watching TV when there is too much movement, or have problems with looking at a computer monitor when scrolling up and down. The dizziness may be associated with fluctuating hearing loss, buzzing or ringing sound in the ear, and pressure in the ear. Many patients have a history of car sickness as a child.

What Brings on the Symptoms: Stress, certain foods (e.g., red wine, chocolate, canned/cured/processed meats, MSG, and/or cheeses in the diet. Change in sleep (too much, too little, or change in pattern), or skipping meals can cause it.

Associated Symptoms: Motion sensitivity, and in some, hearing loss, buzzing or ringing sound in the ear, and pressure in the ear.

Duration: The dizziness lasts at least anywhere from a few minutes and may last up to several weeks or months.

What Causes It: Genetic and environmental causes play a key role.

Treatment: Migraines are generally best managed by stress reduction, good sleep regimen, not skipping meals, and diet modifications. Medications can be given to help the symptoms. The class of medication used depends on a variety of factors.

Acoustic Neuroma

Characteristics: This is a benign but destructive tumor of the balance nerve. The dizziness in acoustic neuromas can present like any other disease and has no particular characteristic. Patients with acoustic neuromas most commonly present with one sided hearing loss (total or partial), one sided ringing in the ears, or sudden hearing loss.

Associated Symptoms: hearing loss, buzzing or ringing sound in the ear, and rarely numbness of the face.

Duration: The duration of the dizziness is variable, it can be short like BPV or long like other diseases.

What Causes It: No known cause exists. Genetics are thought to play a role. Recently, loud noise exposure over a long period of time was found to increase the likelihood of getting an acoustic neuroma.

Treatment: The treatment depends on the size of the tumor, the age and medical status of the patient.

Labyrinthitis

Severe spinning dizziness and imbalance that comes on suddenly and lasts 2-3 weeks and is associated with hearing loss.

Associated symptoms: Intense dizziness associated with nausea and vomiting, hearing loss, and ringing in one ear.

Duration: The dizziness lasts about 2-3 weeks. It takes some time after that to get your balance fully back.

What Causes It: It is thought that a virus infection of the inner ear causes labyrinthitis.

Treatment: Treatment consists of medications to reduce the dizziness and nausea. Since it is caused by a virus, no good treatment is known. Cortisone (anti-inflammatory steroid) medications are given to reduce the symptoms. Injections of steroids into the space behind the ear drum (intratympanic steroids) can help reduce the long-term hearing loss of labyrinthitis. The injections must be done in the first 14 days after the onset of symptoms.

Vestibular Neuronitis

 Severe spinning dizziness and imbalance that comes on suddenly and lasts 2-3 weeks in the absence of hearing loss.

Associated symptoms: Intense dizziness associated with nausea and vomiting.

Duration: The dizziness lasts about 2-3 weeks. It takes some time after that to get your balance fully back.

What Causes It: It is thought that a virus infection of the balance nerve causes vestibular neuronitis.

Treatment: Since it is caused by a virus (much like labyrinthitis), no good treatment is known. Cortisone (anti-inflammatory steroid) medications are given to reduce the symptoms.

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May 9th, 2017 by Sepehr Oliaei, MD