Labyrinthitis is a disorder associated with inflammation of the inner ear. The labyrinth is a fluid-filled compartment that consists of the cochlea and the vestibular organs.

The cochlea is the hearing organ and the vestibular organs are responsible for balance and spatial orientation.

Labyrinthitis has several potential causes, and patients of any age and gender may be affected. Patients with labyrinthitis can experience hearing loss in the affected ear, imbalance, dizziness, and nausea. When the balance organ’s input to the brain is suddenly changed, patients experience vertigo, feeling like you are spinning or moving when you are still. Severe symptoms may limit activities of daily living, the ability to work, and restrict recreational activities.

Labyrinthitis is a self-limiting illness that usually gets better in several weeks. Symptom can begin suddenly and gradually worsen over the course of hours to days before improvement is noticed. Failure to seek treatment may put patients at higher risk for permanent hearing loss and imbalance. Although uncommon, it is possible to have some permanent hearing loss despite treatment. While most patients with imbalance and mild dizziness with head movement recover, sometimes it may take months to years to fully recover. Patients with substantial balance issues may benefit from physical therapy, specifically vestibular physical therapy.

What are the Symptoms of Labyrinthitis?

The symptoms of labyrinthitis can include:

• Hearing loss, often in high frequency pitch range
• Decreased ability to understand speech
• Tinnitus, or ringing or buzzing sensation in the ear
• Imbalance and unsteadiness, falling or swaying to one side while walking
• Vertigo, or feeling like you are spinning when you are still
• Involuntary twitching or jerking of the eyeball, called nystagmus
• Nausea and vomiting

What Causes Labyrinthitis?

Viral infection—Viral infections of the inner ear or activation of a virus that is normally inactive and stays within nerve endings are thought to be the most common cause of labyrinthitis. The specific virus that causes this is usually unknown in most cases. A unique type of labyrinthitis may be caused by reactivation of the varicella-zoster virus, called Ramsay Hunt syndrome, or herpes zoster oticus. This is like shingles of the ear and can occur with labyrinthitis in addition to ear pain, facial weakness, and blisters around the ear, ear canal, and eardrum.

Bacterial infection—A bacterial infection of the middle ear (the space behind the ear drum) can spread to the inner ear and cause bacterial labyrinthitis. Children with inner ear deformities are at a higher risk for bacterial labyrinthitis either from a middle ear infection or from the spread of bacterial meningitis to the inner ear. Severe bacterial labyrinthitis can occur with ear pain, ear infection, drainage of pus from the ear, fevers, or chills. Patients may require hospitalization. This type of infection has a higher risk for permanent hearing loss and may also lead to labyrinthitis ossificans, where there is bone formation in the inner ear after the infection.

Autoimmune—Autoimmune labyrinthitis is a rare cause of labyrinthitis and may come and go. It is often associated with other autoimmune disorders such as systemic lupus erythematosus, inflammatory bowel disease, rheumatoid arthritis, or other autoimmune disorders.

Trauma and surgery—Inner ear trauma puts patients at risk for developing labyrinthitis. Fractures involving the inner ear, concussion of the head and inner ear, or bleeding in the inner ear can cause labyrinthitis.

A tumor of the nerves supplying the inner ear, such as a schwannoma (acoustic neuroma), may also involve hearing loss, vertigo, or labyrinthitis. Your ENT (ear, nose, and throat) specialist, or otolaryngologist, may order an MRI to rule out this condition.

Other potential risk factors include allergies, stress, alcohol and tobacco consumption, and certain medications.

What Are the Treatment Options?

Treating most cases of labyrinthitis includes observation, bed rest, and hydration. Steroids, such as prednisone, are typically prescribed to minimize inner ear inflammation. In some cases, steroids may be injected through the eardrum into the middle ear space. Antivirals may also be prescribed. Severe nausea and vomiting may be treated with anti-nausea medications. Vertigo may be treated with antihistamines or sedatives, such as benzodiazepines, although long-term use will impair the recovery of balance function.

The treatment of bacterial labyrinthitis is to control the primary infection, which is usually a middle ear infection. This may require antibiotics, placement of an ear tube, or more advanced ear surgery. Treatment for autoimmune labyrinthitis addresses the underlying autoimmune condition with steroids or other immune modulating medications usually directed by the rheumatologist.

A multidisciplinary team involving your ENT specialist, audiologist, and vestibular therapist is important to evaluate your hearing, minimize the potential long-term effects of labyrinthitis, and discuss options for possibly restoring your hearing. For severe hearing loss, the patient may be a candidate for a bone-conduction hearing aid or a cochlear implant. When the balance organs are damaged, the brain needs time to adjust to improve balance function again. Vestibular therapy and balance exercises help many patients regain their balance.

Although labyrinthitis is not life-threatening, vertigo may rarely be a sign of a more serious disorder such as a stroke or brain tumor. Patients with sudden, persistent vertigo or additional worsening symptoms such as double vision, slurred speech, facial droop, limb weakness, or numbness should seek immediate medical attention.

What Questions Should I Ask My Doctor?

1. What are my treatment options?
2. When should I get a repeat hearing evaluation?
3. What tests should I have performed?
4. Do I need an MRI?
5. What are the options for hearing restoration?
6. Should I seek vestibular therapy?

Autoimmune inner ear disease (AIED) is an inflammatory condition caused by an uncontrolled immune system response that attacks the inner ear causing progressive sensorineural hearing loss (SNHL) that usually starts in one ear and then affects the other ear.

The body thinks a part of the inner ear should not be there and makes antibodies and immune, also called antigen-antibody, complexes that attack the inner ear. This causes blood vessel inflammation, inner ear tissue damage, and hearing loss. Antigens are the molecules that cause an immune response and antibodies are proteins that protect the body against these antigens.

AIED is rare and diagnosed only when all other causes have been ruled out. The estimated prevalence of AIED is about 15 out of 100,000 people. AIED is felt to be responsible for less than one percent of all SNHL cases, realizing many AIED cases might not be diagnosed due to lack of specific tests.

AIED is considered “primary” when the inner ear is the only organ affected. However, in 15 to 30 percent of cases, AIED is “secondary” when it occurs as part of a larger autoimmune disorder that affects the whole body, such as rheumatoid arthritis, lupus, scleroderma, ulcerative colitis, or Sjogren’s syndrome. AIED is more common among middle-aged women.

Diagnosing AIED is challenging because there is no definite blood or imaging criteria to measure. Timing the progression of hearing loss over weeks to months is an important diagnostic clue for AIED. If AIED is suspected, early corticosteroid and immunosuppressive treatment may prevent irreversible hearing loss. A multidisciplinary team approach between an ENT (ear, nose, and throat) specialist, or otolaryngologist, an audiologist, and a rheumatologist is recommended to manage the condition.

What Are the Symptoms of AIED?

Common symptoms of AIED can include:

• Progressive SNHL in both ears that occurs over weeks to months that is not always the same in both ears
• Fluctuating hearing
• Dizziness or imbalance (approximately 50 percent of AIED cases)
• Ringing in the ears, or tinnitus
• Ear fullness (approximately 25 to 50 percent of AIED cases)
• Conductive hearing loss may be present due to Eustachian tube obstruction from inflamed middle ear lining and/or if AIED is because of systemic autoimmune diseases
• Symptoms of systemic autoimmune diseases, such as fatigue, achy muscles, swelling and redness, low-grade fever, and more

What Causes AIED?

The causes of AIED can include:

• The body’s uncontrolled immune system attacks the inner ear protein, forming immune complexes and antibodies and causing progressive hearing loss in both ears.
• Cochlin is a protein located in the inner ear that is attacked by the immune system.
• Endolymphatic sac, a structure of the inner ear, can become dilated as the immune response of the inner ear.

What Are the Treatment Options?

If you are having hearing loss that is getting worse in both ears over weeks to months, you should see an ENT specialist who can make a diagnosis after reviewing your hearing tests and imaging scans. If your doctor suspects that you may have AIED, you may respond well to medical therapy—steroid and immunosuppressive medication—if started early.

Corticosteroid is the main treatment to suppress the immune response to reduce inner ear swelling and inflammation. Early detection of AIED and prompt steroid treatment may help reverse your SNHL. If you cannot tolerate steroid treatment, there are alternative medications, such as cyclophosphamide, methotrexate, azathiorprine, and rituximab.

Your doctor may also evaluate you for a hearing aid or other assistive listening devices. If hearing loss is substantial, your doctor may recommend a hearing device called a cochlear implant. Speak with your doctor about other specific treatment options.

What Questions Should I Ask My Doctor?

1. Do I have a systemic autoimmune disorder?
2. What tests should I have performed to evaluate my hearing loss?
3. Do I need to get an MRI to rule out other inner ear disorders?
4. What are my treatment options for AIED?
5. What medications are available to me?
6. What other healthcare professionals should I see for AIED?

Turbinate hypertrophy refers to an excessive growth or enlargement of the turbinates, which are bony structures located inside the nose. They are covered with a special skin called mucosa, and they help filter, warm, and humidify the air as you breathe. The mucosa naturally swells during the normal nasal cycle and when you lie down. They also swell in response to allergens and other stimuli.

The nasal cavity usually contains three sets of turbinates on each side of the nose: the superior turbinates, the middle turbinates, and the inferior turbinates. Most of the airflow in the nose passes between the middle and inferior turbinates. If these turbinates become enlarged, breathing can become more difficult.

What Are the Symptoms of Turbinate Hypertrophy?

The most common symptoms of turbinate hypertrophy include:

• Difficulty breathing
• Congestion in alternating sides of the nose
• Nasal stuffiness
• Nasal blockage
• Nasal congestion while lying down
• Noisy breathing or breathing through the mouth during sleep
• Increased nasal drainage

What Causes Turbinate Hypertrophy?

Turbinate hypertrophy is typically caused when the lining of the skin covering the turbinate bone becomes enlarged and swollen. This can be an acute (one time) or chronic (ongoing) problem, and can be caused by many conditions including:

• Upper respiratory infection, or the common cold
• Acute sinus infection
• Allergic rhinitis
• Non-allergic rhinitis
• Chronic rhinosinusitis
• Medications
• Hormonal changes

Other related factors or conditions that can cause nasal blockage include a deviated septum, nasal valve collapse, and external nasal deformity, or other kind of sinus disease.

What Are the Treatment Options?

Treating turbinate hypertrophy depends on the specific cause. First, discuss your symptoms with your primary care physician or an ENT (ear, nose, and throat) specialist, or otolaryngologist. They will examine your nose and may recommend additional tests, such as allergy testing or X-rays, depending on your specific diagnosis.

Medical therapy—Medications are often suggested to help reduce symptoms. Depending on the specific cause of the turbinate hypertrophy, possible medications include:

• Nasal saline sprays or rinses
• Nasal antihistamine sprays
• Nasal steroid sprays
• Allergy immunotherapy

For an acute case of turbinate hypertrophy, nasal decongestant sprays (such as Oxymetazoline) should only be used for a very brief period. This is not an appropriate treatment for chronic turbinate hypertrophy. Using nasal decongestant sprays for longer than three days can result in additional swelling in the nasal cavity, worsening symptoms, and addiction with prolonged use.

Surgery—If medications do not help improve your symptoms, your ENT specialist may offer a surgical procedure to decrease the size of the turbinates. This procedure is usually performed through the nostrils and does not result in bruising or an outward sign of surgery.

The goal of surgery is to decrease the size of the turbinate to improve airflow through the nose while preserving the function of the turbinate. There are many different types of surgical procedures to reduce the size of the turbinates, and will depend on your specific diagnosis, the severity of the turbinate hypertrophy, and your ENT specialist’s recommendation. Many of these procedures can be done in the office using specialized devices that can reduce the size of the turbinates while leaving the special skin (mucosa) intact.

Surgery can usually be performed under local or general anesthesia as an outpatient procedure, and may be combined with other procedures to reduce nasal obstruction. The most common associated procedure is called a septoplasty to address a deviated septum.

Complications from turbinate surgery are rare but can include:

• Anesthesia complications
• Pain
• Swelling
• Bleeding
• Infection
• Dryness and crusting inside the nasal cavity
• Regrowth of the turbinate swelling and recurrent nasal congestion

Empty nose syndrome (ENS) is an unusual condition that can occur after turbinate surgery, and is usually associated with significant resection of the turbinates. In this condition, the nose continues to feel congested and obstructed despite the nasal passage being opened after surgery. This is an extremely rare condition and not completely well understood; if you have concerns about having or developing ENS, discuss this with your ENT specialist.

What Questions Should I Ask My Doctor?

1. What is the most likely cause of my turbinate hypertrophy?
2. Which medications are best suited to treat my turbinate hypertrophy?
3. Are there any other conditions that could be contributing to my nasal congestion?
4. Should I have any other evaluations? Allergy testing? Consultations with other specialists?