EAR DISEASES AND DISORDERS

 

While most people know about hearing loss, many other conditions can affect the ears too. Some are just annoying, but others can cause discomfort. What’s more, these diseases can have a knock-on effect on your hearing, exacerbating any existing hearing loss that you may have. 

Knowing about the conditions that could affect your ears is essential. Here’s a rundown of the most common diseases of the ear. 

Otosclerosis: the abnormal growth of the small bones in the middle ear. It is one of the most common causes of gradual hearing loss in adults, but hearing can be recovered with surgery.

 

Tinnitus: a sensation of noise in the head, such as a constant ringing or buzzing. There is currently no scientific treatment or cure for this condition.

 

Ménière’s disease: a chronic condition that affects the balance and hearing parts of the inner ear. Symptoms include vertigo, hearing loss, tinnitus and a feeling of pressure or “fullness” in the ear. Medication can be prescribed to help alleviate the symptoms. Another option is the Meniett device which is placed in the outer ear and generates micropressure impulses to balance the pressure of the fluid in the inner ear.

 

Presbycusis: this is age-related hearing loss, the gradual loss of hearing in adults as they grow older. It is usually bilateral and symmetrical, meaning it occurs at the same rate simultaneously in both ears.

 

Barotrauma: this is the term for physical damage to the ear caused by changes in barometric (air) or water pressure.

 

Acoustic trauma: this is damage caused to the ear by a sudden, loud noise such as explosions, loud machinery or music concerts. The impact and effects of the damage need to be assessed and monitored over the medium and long term.

 

Hearing loss, impairment or anacusis (deafness): the difficulty to hear due to partial, unilateral, bilateral or total hearing loss. It may be hereditary or the consequence of an illness, traumatism, long-term exposure to noise or aggressive medication for the auditory nerve. Hearing aids or cochlear implants can be used to correct hearing impairment.

 

Exostosis: this is an abnormal bone growth that appears within the ear canal. It is also known as “Surfer’s ear” as it can be caused by exposure to cold wind and water. If it grows in size, surgery may be necessary.

 

Usher syndrome or Retinitis pigmentosa: a rare genetic condition that affects both the sight and hearing.

 

Acoustic neuroma: a benign, slow-growing tumour on the vestibular nerve in the inner ear close to the brain. Treatment includes surgical removal or radiation.

 

Vestibular neuritis: this condition stems from the inflammation of the vestibular nerve in the inner ear, causing vertigo and nausea.

 

Perichondritis: an infection of the skin and tissue covering the cartilage of the external part of the ear. It can cause redness, pain and fever.

 

Mastoiditis: Mastoiditis is an infection of the mastoid bone of the skull. The mastoid is located just behind the ear.

 

Otitis Media and Otitis Externa

The term otitis media means that there is inflammation of the middle ear. Otitis media can be associated with an infection or be sterile. In the first case, otitis media is usually caused by bacteria that migrate into the middle ear via the Eustachian tube. Occasionally otitis media may be caused by fungi (Aspergillus or Candida) or other pathogens, such as the herpes virus. In this situation, usually either there is a problem with immune function or (there is a hole (perforation) in the ear drum. Persons with diabetes are particularly susceptible to unusual pathogens such as pseudomonas. In underdeveloped parts of the world, tuberculosis should be considered.

Otitis Externa

The condition of otitis externa, often called "Swimmers ear", differs from otitis media, although both may be present simultaneously. In otitis externa, the external ear canal is inflamed. In otitis externa, there may be severe pain and a modest reduction in hearing, but generally there is no dizziness because otitis externa is separated from the inner ear by the ear drum and air filled middle ear.

In children a common cause of otitis externa is insertion of something into the ear canal (like a navy bean, for example). In adults, the most common cause is also insertion of something into the ear canal perforating the ear drum (usually a "q" tip, but sometimes hairpins and as well as other thin and pointed objects). Adults may also compulsively clean their ears with cotton tipped applicators, and push wax deep into their external ear canals, resulting eventually in an external otitis. Chronic external otitis is diffuse low-grade infection that can persist for months to years. The disease is characterized by itching, a slight discharge, and a progressive narrowing of the external ear canal.

 

Anotia / Microtia

Anotia means absence of the external ear. Microtia is the term for an incompletely formed external ear. Aural atresia is the absence of the ear canal.

Microtia happens when the outer ear is smaller than normal and doesn’t form correctly. Microtia can range in how severe it is, from being a partially formed ear to being a lump of tissue where the ear should be. Sometimes the ear canal is very narrow or missing completely. The ear canal is the pathway that leads from the outside of the ear to the middle ear.

Microtia can affect one or both ears:

·       Unilateral microtia is when one ear is affected. This is the most common form. About 9 in 10 (90 percent) microtia cases are unilateral. It appears on the right side of the head twice as often as the left side.

·       Bilateral microtia is when both ears are affected. It happens in about 1 in 25,000 babies. 

There are four types of microtia:

1.    Type I microtia. The ear is slightly smaller than usual, but all other parts of the ear are healthy. This is the mildest form of microtia.

2.    Type II microtia (also called conchal type microtia). This is when a baby has a partial ear with a narrow or closed ear canal. A baby with Type II microtia has some hearing loss. Hearing loss is being partly or totally unable to hear sound in one or both ears. It can happen when any part of the ear isn’t working in the usual way. Hearing loss can affect a baby’s ability to develop speech, language and social skills.

3.    Type III microtia (also called lobular type microtia). This is the most common type of microtia. The ear looks like a small peanut with an ear lobe. The ear canal and ear drum are usually missing (called aural atresia). Most babies with Type III microtia have hearing loss.

4.    Type IV microtia (also called anotia). This is the most severe and rarest type. Anotia happens when both the inside and outside parts of the ear are missing. It can affect one or both ears, but it’s more common for babies to have anotia in only one ear. Babies with anotia have conductive hearing loss. This happens when there’s a problem in the outer or middle ear that slows or prevents sound waves from passing through. 

 

Hyperacusis

Hyperacusis is a rare disorder that causes ordinary sounds that are otherwise normal to most people to sound intolerably loud, and sometimes painful. People who suffer from the disorder may even perceive normal environmental sounds to be too loud and uncomfortable enough to avoid these situations (e.g. car engines, dishes clanging, shuffling paper, or even loud conversation). Many people with hyperacusis have normal hearing.

Hyperacusis can develop suddenly or gradually, occur in one or both ears and is often accompanied by tinnitus (ringing or buzzing in the ear); it can be caused by a number of factors. The most common relates to damage to the cochlea from exposure to loud noises such as those found in certain work environments, rock concerts, gunfire and fireworks. Other causes may include, drugs, Bell’s palsy, Lyme disease, Meniere's disease, Temporomandibular Joint Syndrome (TMJ), head injury, Posttraumatic stress disorder, postoperative surgery, head trauma (e.g. airbag deployment in cars) or autism.

Treatment is based on the notion of desensitization, and increasing sound intensity (loudness) from a low level gradually over time. Depending on the cause, hyperacusis may also get better over time. Specifically, in cases of trauma to the brain or auditory system, there is a chance that the sensitivity to sounds will become more tolerable. In all cases, professional counseling with qualified individuals can be beneficial.

Autoimmune Inner Ear Disease (AIED)

AIED is a rare condition of progressive hearing loss or dizziness (less than one percent of all cases of sensorineural hearing loss or dizziness) caused by antibodies or immune cells inappropriately attacking the inner ear. It occurs when the body's immune system attacks the inner ear cells that are mistaken for a virus or bacteria. Until recently it was not thought that the inner ear could be attacked by the body’s own immune system.

Diagnosis can be difficult because AIED can mimic other disorders exhibiting sensorineural hearing loss. In most cases, a reduction of hearing is accompanied by tinnitus (ringing, buzzing, hissing) that occurs over weeks or months. Variations of the condition are attacks of bilateral hearing loss and tinnitus that resemble Meniere’s disease, and episodes of dizziness in addition to abnormal blood tests for antibodies. Approximately 50 percent of patients with AIED have symptoms related to balance (dizziness or unsteadiness).

Steroid responsiveness is the most useful approach for diagnosis; typically a bilateral progressive sensorineural hearing loss that responds to steroids confirms the diagnosis. The standard treatments for autoimmune manifestations are drugs that suppress immune function. Before treatment is begun, it is imperative to understand, and never ignore, the side effects of immunosuppressive drugs. With the proper precautions, treatment can be safe and the results can be remarkable.