Part III Hearing Health Ear Problems

Anything that completely occludes the ear canal can cause hearing loss. Impaction with cerumen (wax) is common. It is frequently due to the use of cotton-tipped swabs in attempts to clean the ear. The ear has a natural self-cleaning mechanism. Ear skin normally sheds from the inside out. If you place a drop of ink on the eardrum and wait a few weeks, it will turn up near the opening of the ear. The ear canal is also shaped like a funnel, with the narrow end near the eardrum. Cotton swabs generally push wax deeper into the ear than it normally is (wax forms only in the outer segment of the ear canal), and packs it into a mass. As long as there is even a pinhole opening in the packed wax, hearing is usually good. However, when occlusion by wax is complete, a substantial hearing loss develops. This can be cured easily by removing wax either by using specially designed instruments, or irrigation with water. There are many over-the-counter wax control preparations but they often cause maceration of the skin and consequent external ear infections.

Many other problems can cause ear canal occlusion and hearing loss. They include infections with swelling that shuts the ear canal, foreign bodies in the ear, trauma, birth defects, tumors (including cancer), and other causes. The resulting hearing loss is conductive. That is, it interferes with sound conduction and is generally correctable. It causes a decrease in volume but does not generally produce distortion.

Temporary hearing loss is commonly caused by ear infections. Middle ear infections cause swelling of the mucosal lining in the middle ear, and often an accumulation of fluid (such as pus). When there is fluid behind the eardrum surrounding the ossicles, these structures cannot work properly; and hearing loss results. Infections are usually treated with antibiotics, sometimes with decongestants, and occasionally with drainage by lancing the eardrum (a procedure called myringotomy). If infections are severe or inadequately treated, they may cause permanent hearing loss by damaging the ossicles (incus, malleus, or stapes), causing fixation or stiffness of the ossicle, or perforating the eardrum. Most perforations (holes) heal spontaneously. When one does not, it can nearly always be repaired surgically. The procedure is usually relatively fast and is generally performed under local anesthesia.

Ear fluid not caused by infection accumulates commonly in children, and sometimes in adults. The condition is known as serous otitis media. This is the condition that leads to myringotomy and tube placement, one of the most common operations performed in the United States. It is useful to understand the mechanism behind serous otitis media. Usually, it is due to a malfunction of the eustachian tube which connects the ear with the back of the throat. The eustachian tube’s job is to keep the pressure in the middle ear approximately the same as that in the ear canal and outside world. The eustachian tube does so by opening momentarily when we swallow or yawn. If it fails to open because of swelling, allergy, structural-developmental reasons, the air already in the middle ear becomes trapped. Gradually, this air is resorbed into the bloodstream. The middle ear space is bony except for the eardrum.

As resorption occurs and less and less air is present, its disappearance creates a vacuum (negative pressure) which sucks the eardrum into the middle ear (retracted eardrum). When the negative pressure is great enough, it causes fluid to seep out of the blood vessels in the mucosa lining of the middle ear. This is the serous fluid that constitutes serous otitis media. The condition causes hearing loss, and sometimes frequently recurring ear infections. In some cases, the eustachian tube starts to work again spontaneously and the condition resolves. In others, decongestants may be helpful. When allergy is the cause, allergy treatments may improve the problem. When serious otitis media causes frequent infections (recurrent acute otitis media) some physicians recommend the long-term use of antibiotics although this treatment is currently controversial. The most widely accepted treatment for persistent middle ear fluid especially in children is myringotomy and tube placement. This ordinarily eliminates the hearing loss almost instantly, decreases the incidence of recurring infections, and prevents the reformation of fluid.

The tiny tubes placed in the eardrum to keep the ear drainage hole open are not drainage tubes. They are ventilating tubes. They act in lieu of a functioning eustachian tube to maintain normal pressures within the middle ear. Hence, no vacuum forms, and the fluid is not created in the first place. This is why children with tubes don’t have constant problems with fluid draining out of their ears. Treatment of this condition is important for many reasons. In addition to eliminating disturbing hearing loss and frequent infections, there is evidence to suggest that such hearing loss in children interferes with learning, perhaps more than we might expect it to from the mild to moderate severity of the hearing loss in most cases. Middle ear effusion in adults deserves special mention, too. It is not an uncommon consequence following barotraumas, such as a poorly pressurized airplane descent. However, occasionally we find unexplained middle ear effusion in one ear of an adult. It is essential to determine the cause of this problem, and especially to rule out the presence of a mass (such as cancer) in the nasopharynx occluding the opening of the eustachian tube.

Otosclerosis is a common hereditary disease that deposits bone around the footplate of the stapes. This prevents normal bone transmission of sound from the eardrum to the inner ear, and consequently conductive hearing loss. This hereditary condition is present in about 10 percent of white Americans, and it causes significant hearing loss in about 1 percent. It occurs less commonly in Black and Asian people. Otosclerosis generally becomes apparent during early life, and the severity of the hearing loss is accelerated by pregnancy. The hearing loss can be cured by an operation called a stapedectomy, in which the stapes bone is replaced by an artificial bone. In the good hands of the surgeon, this procedure usually only takes fifteen or twenty minutes, and it is performed under local anesthesia. When all goes well, the hearing may return to normal.

Various birth defects may also involve the middle ear and cause hearing loss. Some are major and cause obvious external defects. Many involve only the inner ear structures and a CT scan or even surgery may be necessary to establish the diagnosis. In any case, most conductive forms of hearing loss are surgically treatable.

It is also important to recognize that the tumors of the middle ear may be responsible for hearing gloss. They may be malignant, such as rhabdomyosarcoma in children or squamous cell carcinoma in adults. It may also be benign, such as glomous tumors (which can also cause pulsating ear noises) or neuromas of the facial or acoustic (hearing and balance) nerves. Cholesteatoma is a common benign skin cyst that grows slowly and dissolves adjacent bone as it grows. It is a fairly common problem, especially in people who have had repeated ear infections; but it may also occur from birth in the absence of infections. It generally requires mastoid surgery, as discussed below.

There are many conditions that cause the delicate sensorineural mechanism to function abnormally. Otosclerosis discussed above, can also affect the cochlea and cause sensorineural hearing loss in some patients. In a small percentage, sensorineural hearing loss can be severe.

The natural aging process also causes sensorineural hearing loss. Beginning shortly after birth, we begin to lose hair cells and nerve endings in the basal turn of the cochlea (the region that hears very high frequencies). As this loss pattern progresses over a lifetime, sensorineural hearing loss develops. There are also other age-related causes of hearing loss including stiffening of the cochlear partition in the inner ear and loss of nerve endings in the acoustic nerve.

A fistula (opening) is an abnormal connection between the inner ear and the middle ear. The inner ear is filled with fluid, and the middle ear is filled with air. If a fluid leak occurs allowing the inner ear fluids out, hearing loss and dizziness commonly result. This kind of sensorineural hearing loss can often be cured by surgically repairing the fistula. Such leaks are usually caused by trauma. The trauma may be direct, such as a blow to the ear or a head injury in a car accident. However, it may also be the result of barotraumas caused by an airplane trip, a forceful sneeze, or lifting a heavy object.

Direct head trauma, particularly trauma severe enough to cause unconsciousness, can cause inner ear concussions and sensorineural hearing loss.

Ménière’s syndrome is a condition characterized by fluctuating sensorineural hearing loss (usually more prominent in the lower frequencies where we hear speech), episodic vertigo, fluctuating ear pressure, and tinnitus (usually of a seashell variety). It is due to endolymphatic hydrops, which is a swelling and fluid overload of the middle compartment of the inner ear. There are many treatable causes of Ménière’s syndrome. So, exhaustive evaluation in search of them is recommended. When all tests have revealed none of the known causes, the condition is classified as Ménière’s Disease.

Noise is an important cause of hearing loss. It is estimated that there are 7 to 10 million people in the American industry with noise-induced hearing loss, virtually all of which was preventable. In addition to industrial noise, recreational noise can damage hearing. Such noise is encountered commonly from gunfire, power tools, snow blowers, motorcycles, loud music (especially with insert earphones), and other causes. In some cases, the playing of musical instruments can damage hearing. This has been reported not only with loud, electrical rock and roll instruments but also with classical music performance such as violin playing and flute playing. One can minimize such problems by using ear protection whenever practical, such as during selected practice sessions.

Infections involving the inner ear and the hearing nerve can also produce deafness. Middle ear infections can spread to the inner ear causing loss of hearing, and usually dizziness. Viral infections may also involve the hair cells or auditory nerve causing hearing loss and even sudden total deafness.

Sudden deafness is a special problem. It can occur in one or both ears. It may be caused by a variety of problems and demands immediate, aggressive and comprehensive evaluation. Treatment is controversial, but there is at least some evidence that suggests that aggressive treatment may improve the chances for hearing recovery even after a sudden profound loss. In any case, even those of us who believe in treating sudden deafness aggressively agree that once the condition has been present for more than 2 to 3 weeks, even the most aggressive treatments do not work. So, prompt attention is essential.

Autoimmune inner ear disease (AIED) is much more common than most physicians believe. In autoimmune disorders, the body makes antibodies against the patient. For example, in rheumatoid arthritis, we make antibodies against our joints. In psoriasis, we make antibodies against our skin; in multiple sclerosis, against our nerve sheaths; in thyroiditis, against our thyroid glands; and there are many other autoimmune disorders, including diabetes mellitus. In autoimmune inner ear disease, antibodies are produced against our own inner ears. AIED can cause hearing loss, tinnitus, dizziness, ear fullness, and other symptoms. They may occur in combination or alone. Classically, AIED is thought to be associated with sudden deafness, or rapidly progressive, asymmetric sensorineural hearing loss. However, it can cause hearing loss of any pattern, and it can also cause tinnitus or dizziness in the absence of any measurable hearing loss. AIED can affect adults or children.

Neural problems may also produce hearing loss. Among the more common are acoustic neuroma, multiple sclerosis, autoimmune sensorineural hearing loss (in which the body attacks its own ear), and ototoxicity. Ototoxicity is hearing loss caused by a substance that gets into the body. Most often the substance is a medication, particularly certain antibiotics and diuretics. However, other toxins (such as lead) may also cause hearing loss.

An acoustic neuroma is a common tumor of the acoustic nerve. It generally starts on the balanced division of the nerve, in the auditory canal, the bony channel through which the nerve courses as it goes from the ear to the brain. Acoustic neuromas grow, often at varied rates, and can compress the brainstem and other structures including the facial nerve. Ordinarily, these tumors are surgically curable. They are managed best by neurotologists, inner ear sub-specialists with particular interest and expertise in the treatment of this specific tumor. Neurotologists often work in collaboration with a neurosurgeon. In the majority of cases, it is possible to remove acoustic neuromas without serious injury, and without facial paralysis (which is always a risk since the tumor has to be peeled off a compressed facial nerve). In some tumors diagnosed early, it is even possible to preserve hearing (despite the fact that the tumor has its roots in the acoustic nerve).

A great number of other problems can cause hearing loss, particularly sensorineural hearing loss, and many of them are very common conditions, and that most people (including physicians) are not aware of their association with hearing loss.

Mumps is the most common cause of one-sided total deafness in the United States. Frequently, the child and family are not aware of the hearing loss until years later.  Usually, the balance system is normal. Other childhood infections may also affect hearing, particularly by destroying the eardrum and damaging the middle ear bones.  Scarlet fever is notorious for creating such problems.

There are many. Syphilis is among the most important. Commonly, it has been acquired either at birth, or a long time before hearing symptoms occur (20, 30, or more years). It is especially important because it is a treatable and sometimes curable, form of sensorineural hearing loss. It may present as Ménière’s syndrome, sudden deafness, or sensorineural hearing loss of any pattern (typically slightly asymmetric with somewhat poor discrimination). Routine syphilis tests, such as those obtained for marriage licenses, are generally normal; but special tests for latent syphilis are positive. The disease is not contagious at that stage; but if it is not recognized and treated, the hearing loss may progress and even become total.

Lyme disease can cause similar problems. This increasingly common infection is caused by a spirochete (as is syphilis) carried most commonly by ticks. Lyme disease often causes a rash and joint pain, but these may be minor enough to escape notice. Appropriate blood tests should be obtained for people with sensorineural hearing loss of unknown origin, and for patients with other unexplained nerve problems, such as facial paralysis.

Numerous other systemic infections including herpes, cytomegalovirus (CMV), measles, mononucleosis, varicella, mycoplasma, influenza, and fungal diseases may cause hearing problems as well.

Much sensorineural hearing loss is believed to be due to vascular insufficiency involving either the inner ear or related areas of the brain. This situation is encountered with generalized cardiovascular disease, hypertension, and other similar conditions. It may also be present with hypercoagulable states, in which the blood tends to sludge and clot excessively. Polycythemia, a condition in which there are too many blood cells, is one example.

Hearing loss is one of the most common consequences of meningitis, especially bacterial or fungal meningitis. Anyone who has had meningitis should have a hearing test upon recovery.

AIDS is associated with ear infections and neurological damage. Conductive and sensorineural hearing loss both occur in association with AIDS. AIDS is also associated with tumors in the head and neck that can cause hearing loss.

Tuberculosis and other granulomatous conditions such as sarcoidosis and Wegener’s granulomatosis, now referred to as granulomatosis with polyangiitis, have all been associated with hearing loss. The problem may be due to the disease itself, or to the medications used to treat the disease (such as streptomycin). Tuberculosis is increasingly common, especially among AIDS patients and those who come in contact with them.

Family history is extremely important. Hearing loss is often hereditary. When it runs in families from generation to generation, hearing loss is usually following a hereditary pattern called “autosomal dominant.” However, the absence of a family history does not mean that hearing loss is not genetic. “Autosomal recessive” inheritance is common. It means that neither parent has hearing loss, but both carry a gene that causes it. On average, hearing loss will be present in one child out of four.

Arthritides (inflammation of joints) and vasculitis (inflammation of blood vessels) commonly are associated with hearing loss. These include conditions such as rheumatoid arthritis, lupus erythematosus, giant cell arteritis, and others. The hearing problem is probably related to vascular abnormalities associated with these diseases.

It is well recognized that allergic problems in children cause eustachian tube dysfunction and middle ear effusion. However, in some cases, allergies may also cause inner ear problems such as Ménière’s syndrome. In such patients, allergy treatment usually results in the resolution of ear complaints.

Some conditions associated with high blood pressure (such as hypolipoproteinemia—extremely elevated cholesterol and triglyceride) are also associated with sensorineural hearing loss. In general, it appears that people with high blood pressure have a higher incidence of hearing loss. They may also be more prone to noise-induced hearing loss than others.

Hypothyroidism (underactive thyroid) is common. About 50 percent of patients with low thyroid function, severe enough to cause myxedema (a special kind of swelling), have hearing losses. Moreover, about 3 percent of patients with Ménière’s syndrome have hypothyroidism; and in some, control of the thyroid disease eliminates the Ménière’s syndrome symptoms.  

Many of the things that damage the kidney also damage the cochlea. The glomerulus of the kidney and the stria vascularis of the cochlea are quite similar. They are often damaged by the same drugs, for example. High-frequency sensorineural hearing loss is common in people with severe renal disease. Often, it is not possible to tell whether both were caused by the same etiology, or whether hearing loss is secondary to the renal disease and its treatment.

Cancers that involve the ear and the brain can cause hearing loss, of course. However, cancers elsewhere may also be related, particularly because many of the treatments for cancer produce hearing loss. Chemotherapy agents are often ototoxic. Radiation may also cause hearing loss if the ear is included in the radiation field. Patients receiving chemotherapy or radiation therapy should have an audiogram before treatment is begun, and usually during and after treatment.

Diabetes is one of the most common diseases in the United States. Although estimates vary from study to study, it appears that about 40 percent of diabetics have hearing loss. It is usually sensorineural, progressive, bilateral (both ears), and most severe in the high frequencies. However, Ménière’s syndrome may also be caused by diabetes, and sudden deafness has been reported. In general, diabetic hearing loss is felt to be caused by the same kind of microvascular changes that cause diabetic eye disease.

The relationship has been controversial, but it is probably that there is a significantly increased incidence of hearing loss in patients with glaucoma. The association is especially prominent in patients with narrow-angle glaucoma.

About 7 to 9 percent of Black Americans carry the sickle cell trait. About 1 in 400 has sickle cell disease, and 20 to 25 percent of patients with sickle cell disease have sensorineural hearing loss. Sudden deafness has also been reported in conjunction with the sickle cell crisis. Remarkably, in some cases, even total deafness resolves after the crisis is over.

There is a very important condition called Jervell and Lange-Nielson syndrome. It is a hereditary condition that accounts for approximately 1 percent of all cases of hereditary deafness. It is characterized by sensorineural hearing loss (often severe) and fainting. Whenever this association exists, an electrocardiogram should be obtained immediately. The fainting is due to heart arrhythmias that may cause sudden death. Diagnosis and placement of a pacemaker may be life-saving.

There are literally entire books on hereditary syndromes and hearing loss. The syndromes involve defects in virtually any part of the body.

Extensive discussions of tinnitus (ear noises) and dizziness are beyond the scope of this text and are covered in other publications. However, it is important to be aware that they commonly occur in association with hearing loss. It is also important to recognize that, contrary to the opinion of many physicians, neither tinnitus nor dizziness is necessarily incurable. Like hearing loss, thorough evaluation may lead to important and/or treatable causes for these conditions, and they should not be ignored.

The first principle and treatment of hearing loss is an accurate diagnosis. When a specific treatable cause is found, such as hypothyroidism or inner ear syphilis, appropriate treatment is instituted. If the hearing loss is due to mechanical causes such as chronic ear infections or ossicular dysfunction, it can generally be restored surgically.  Alternatively, amplification (a hearing aid) is always an option. For most people with sensorineural hearing loss, amplification is the best of the only option.

Virtually no condition in medicine can have as profound an effect on the quality of life as even moderate hearing loss in some people. Hearing loss makes even routine communication difficult. High-frequency hearing loss often involves loss of ability to hear consonants such as s, f, t, and z, even though vowels can be heard normally.  Consequently, people hear but cannot make out what is being said. This may result in frustration, marital discord, withdrawal from social activities, and depression. People lose the ability to take in the sounds that make life worth living, like bird songs, the rustling of leaves, and the voices of children. In general, these infringements on quality of life can be overcome through medical or surgical treatment or amplification.

When hearing loss occurs early in childhood, its devastating consequences are obvious than when it occurs insidiously late in life. A substantial hearing deficit in infancy interferes with the normal process of psychological and emotional development. Severe hearing loss makes learning a mammoth task for the child, and frustration or isolation frequently results. The personality distortion that results from this sequence of events affects the person and his family throughout their lives. Even more mild forms of hearing loss early in life can cause great difficulties, including poor attention and bad grades in school. Frequently, such children are considered “not too bright,” before anyone realizes that a hearing loss is present. When it is corrected, the changes in the child’s performance, attitude, and interactions are often remarkable.