What is AIED?

Autoimmune inner ear disease (AIED) is an inflammatory condition of the inner ear. It occurs when the body’s immune system attacks cells in the inner ear that are mistaken for a virus or bacteria. AIED is a rare disease occurring in less than one percent of the 28 million Americans with a hearing loss.

How Does the Healthy Ear Work?

The ear has three main parts: the outer, middle and inner ear. The outer ear (the part you can see) opens into the ear canal. The eardrum separates the ear canal from the middle ear. Small bones in the middle ear help transfer sound to the inner ear. The inner ear contains the auditory (hearing) nerve, which leads to the brain.

Any source of sound sends vibrations or sound waves into the air. These funnel through the ear opening, down the ear canal, and strike your eardrum, causing it to vibrate. The vibrations are passed to the small bones of the middle ear, which transmit them to the hearing nerve in the inner ear. Here, the vibrations become nerve impulses and go directly to the brain, which interprets the impulses as sound (music, voice, a car horn, etc.)

Symptoms of AIED

The symptoms of AIED are sudden hearing loss in one ear progressing rapidly to the second ear. The hearing loss can progress over weeks or months. Patients may feel fullness in the ear and experience vertigo. In addition, a ringing, hissing or roaring sound in the ear may be experienced. Diagnosis of AIED is difficult and is often mistaken for otitis media until the patient develops a loss in the second ear. One diagnostic test that is promising is the Western blot immunoassay.

Treatment for AIED

Most patients with AIED respond to the initial treatment of steroids, prednisone and methotrexate, a chemotherapy agent. Some patients may benefit from the use of hearing aids. If patients are unresponsive to drug therapy and hearing loss persists, a cochlear implant maybe considered.

History of AIED

Until recently it was thought that the inner ear could not be attacked by the immune system. Studies have shown that the perisacular tissue surrounding the endolymphatic sac contains the necessary components for an immunological reaction. The inner ear is also capable of producing an autoimmune response to sensitized cells that can enter the cochlea through the circulatory system.

AIED Research

A multi-institutional clinical study, Otolaryngology Clinical Trial Cooperative Group (OCTCG) co-sponsored by the NIH and the American Academy of Otolaryngology – Head and Neck Surgery Foundation, is being conducted to measure the benefits and risks of treating AIED with two different immunosuppressive drugs: prednisone and methotrexate, a chemotherapy drug.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

¿Qué es la otoesclerosis?

El término otoesclerosis deriva del griego esclero (duro) y oto (oído). Describe la condición de un anormal crecimiento en los pequeños huesos del oído medio que llevan a la fijación del estribo. El hueso del estribo se debe mover libremente para que el oído trabaje correctamente y se pueda escuchar en forma adecuada.

La audición es un proceso complejo. En un oído normal las vibraciones sonoras son conducidas por el oído externo hacia el canal auditivo donde golpean la membrana del tímpano. Estas vibraciones producen el movimiento de la membrana que lo transfiere a los tres pequeños huesos del oído medio, el martillo, el yunque y el estribo. Cuando el hueso del estribo se mueve pone en movimiento los líquidos en el oído interno que empieza el proceso de estimular el nervio auditivo. Este nervio lleva la energía sonora al cerebro resultando en la interpretación del sonido escuchado. Cuando cualquier parte de este proceso está comprometida la audición se ve dificultada.

¿Quién sufre Otoesclerosis y por qué?

Se estima que el diez por ciento de la población de adultos caucásicos está afectada de otoesclerosis. La enfermedad es menos común en asiáticos y rara en afroamericanos. Las mujeres de mediana edad son las de mayor riesgo.

El síntomas destacado de la otoesclerosis, lenta progresión de pérdida auditiva, puede comenzar en cualquier momento entre las edades de 15 y 45 años pero usualmente empieza a principios de los 20.

Esta enfermedad puede desarrollarse tanto en hombres como en mujeres, pero es particularmente problemática en mujeres embarazadas quienes, por razones desconocidas, tienen una rápida disminución de su capacidad auditiva.

Aproximadamente el 60 por ciento de los casos de otoesclerosis son de origen genético. En promedio una persona que tiene un padre con otoesclerosis tiene un 25 por ciento de chance de desarrollar esta alteración. Si los dos padres tienen otoesclerosis el riesgo aumenta hasta el 50 por ciento.

Síntomas de Otoesclerosis.

La pérdida gradual de la audición es el síntoma más frecuente de la otoesclerosis. Frecuentemente individuos con otoesclerosis empiezan a notar que no pueden oír sonidos de baja frecuencia o susurros. Otros síntomas de esta alteración pueden incluir mareos, alteraciones del equilibrio o sensación de zumbidos o ruidos en los oídos conocidos como acúfenos.

¿Como se diagnostica la Otoesclerosis?

Debido a que muchos de los síntomas típicos de otoesclerosis pueden ser causados por otras patologías, es importante que sea examinado por un otorrinolaringólogo para eliminar otras posibles causas de estos síntomas. Luego de un examen de oído, el otorrinolaringólogo puede ordenar un examen de audición. Basado en los resultados de este examen el otorrinolaringólogo sugerirá las distintas opciones terapéuticas.

Tratamiento de la Otoesclerosis

Si la pérdida auditiva es leve, el otorrinolaringólogo puede sugerir una observación continua y un audífono para amplificar el sonido que llega a la membrana del tímpano. El fluoruro de sodio retrasa la progresión de la enfermedad y puede ser prescripto. En la mayoría de los casos de otoesclerosis un procedimiento quirúrgico llamado estapedectomía es el método más efectivo para restaurar y mejorar la audición.

¿Que es la estapedectomía?

Una estapedectomía es un procedimiento quirúrgico ambulatorio que se hace bajo anestesia local o general a través del canal auditivo bajo microscopio (no se hacen incisiones externas). Incluye la remoción del estribo inmovilizado y se lo reemplaza por una prótesis. Esta prótesis permite que los huesos del oído medio recuperen el movimiento que estimula el líquido en el oído medio y mejora y restaura la audición.

Las estapedectomías modernas se vienen realizando desde 1956 con una tasa de éxitos del 90 por ciento. En algunos raros casos (alrededor de 1 por ciento) el procedimiento puede empeorar la audición.

La otoesclerosis afecta ambos oídos en ocho de cada diez pacientes. Para estos pacientes los oídos son operados uno a la vez. El de peor audición primero.

¿Que se debe esperar luego de una estapedectomía?

La mayoría de los pacientes vuelven a su casa al otro día de la cirugía y son avisados para que descanses tranquilos sobre el oído no operado. Se prescriben antibióticos por vía oral. Algunos pacientes experimentan mareos los primeros días luego de la cirugía. La sensación del gusto puede estar alterada por algunas semanas o meses después de la cirugía pero usualmente regresa a la normalidad.

Luego de la cirugía los pacientes deben evitar el sonarse fuerte la nariz, nadar u otras actividades que puedan llevar agua al oído operado. Actividades normales (incluido el viaje en avión) son generalmente retomadas dos semanas después de la cirugía.

Se debe avisar al otorrinolaringólogo inmediatamente si ocurre algo de lo siguiente:

  • Pérdida súbita de la audición
  • Dolor intenso
  • Mareo prolongado o intenso
  • Cualquier síntoma nuevo relacionado con el oído operado.

Dado que el vendaje es colocado en el oído en el momento de la cirugía, la mejora auditiva no se notará hasta que este sea removido alrededor de una semana después. La membrana del tímpano curará rápidamente alcanzando el máximo nivel de mejora dentro de las dos semanas.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

The term otosclerosis is derived from the Greek words for “hard” (scler-o) and “ear” (oto). It describes a condition of abnormal bone growth around the stapes bone, one of the tiny bones of the middle ear. This leads to a fixation of the stapes bone. The stapes bone must move freely for the ear to work properly and hear well.

Hearing is a complex process. In a normal ear, sound vibrations are funneled by the outer ear into the ear canal where they hit the tympanic membrane (ear drum). These vibrations cause movement of the ear drum, which transfers the vibrations to the three small bones of the middle ear, the malleus (hammer), incus (anvil) and stapes (stirrup). When the stapes bone moves, it sets the inner ear fluids in motion, which, in turn, start the process to stimulate the tiny sensory hair cells in the inner ear, which connect with the auditory (hearing) nerve. The hearing nerve then carries sound information to the brain, resulting in hearing of sound. When any part of this process is compromised, hearing is impaired.

Who Gets Otosclerosis and Why?

It is estimated that ten percent of the adult Caucasian population is affected by otosclerosis. The condition is less common in people of Japanese and South American decent and is rare in African Americans. Overall, Caucasian, middle-aged women are most at risk.

The hallmark symptom of otosclerosis, slowly progressing hearing loss, can begin anytime between the ages of 15 and 45, but it usually starts in the early 20’s. The disease can develop in both women and men, but is particularly troublesome for pregnant women who, for unknown reasons, can experience a rapid decrease in hearing ability.

Approximately 60 percent of otosclerosis cases have a genetic predisposition. On average, a person who has one parent with otosclerosis has a 25 percent chance of developing the disorder. If both parents have otosclerosis, the risk goes up to 50 percent.

Symptoms of Otosclerosis

Gradual hearing loss is the most frequent symptom of otosclerosis. Often, individuals with otosclerosis will first notice that they cannot hear low-pitched sounds or whispers. Other symptoms of the disorder can include dizziness, balance problems or a sensation of ringing, roaring, buzzing or hissing in the ears or head known as tinnitus.

How is Otosclerosis Diagnosed?

Because many of the symptoms typical of otosclerosis can be caused by other medical conditions, it is important to be examined by an otolaryngologist (ear, nose and throat doctor) to eliminate these other causes. After an examination, the otolaryngologist may order a hearing test. The typical finding on the hearing test is a conductive hearing loss in the low frequency tones. This means that the loss of hearing is due to an inability of the sound vibrations to get transferred into the inner ear. Based on the results of this test and the exam findings, the diagnosis of otosclerosis can be made. The otolaryngologist will suggest treatment options.

Treatment for Otosclerosis

If the hearing loss is mild, the otolaryngologist may suggest continued observation or a hearing aid to amplify the sound reaching the ear drum. Sodium fluoride has been found to slow the progression of the disease and is sometimes prescribed. In some cases of otosclerosis, a surgical procedure called stapedectomy can restore or improve hearing.

What Is a Stapedectomy?

A stapedectomy is an outpatient surgical procedure done under local or general anesthesia. The surgeon performs the surgery through the ear canal with an operating microscope. It involves removing part or all of the immobilized stapes bone and replacing it with a prosthetic device. The prosthetic device allows the bones of the middle ear to resume movement, which stimulates fluid in the inner ear and improves or restores hearing.

Modern-day stapedectomy has been performed since 1956 with a success rate of approximately 90 percent. In rare cases (about one percent of surgeries), the procedure may worsen hearing.

Otosclerosis affects both ears in eight out of ten patients. For these patients, ears are operated on one at a time; the worst hearing ear first. The surgeon usually waits a minimum of six months before performing surgery on the second ear.

What Should I Expect after a Stapedectomy?

Most patients return home the evening after surgery and are told to lie quietly on the un-operated ear. Oral antibiotics may be prescribed by the otolaryngologist. Some patients experience dizziness the first few days after surgery. Taste sensation may also be altered for several weeks or months following surgery, but usually returns to normal.

Following surgery, patients may be asked to refrain from nose blowing, swimming, or other activities that may get water in the operated ear. Normal activities (including air travel) are usually resumed two to four weeks after surgery.

Notify your otolaryngologist immediately if any of the following occurs:

  • Sudden hearing loss
  • Intense pain
  • Prolonged or intense dizziness
  • Any new symptom related to the operated ear

Since packing is placed in the ear at the time of surgery, hearing improvement may not be noticed until it is removed about one to three weeks after surgery. The ear drum will heal quickly, generally reaching the maximum level of improvement within two weeks.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

What is Hyperacusis?

Hyperacusis is a condition that arises from a problem in the way the brain’s central auditory processing center perceives noise. It can often lead to pain and discomfort.
Individuals with hyperacusis have difficulty tolerating sounds which do not seem loud to others, such as the noise from running faucet water, riding in a car, walking on leaves, dishwasher, fan on the refrigerator, shuffling papers. Although all sounds may be perceived as too loud, high frequency sounds may be particularly troublesome.
As one might suspect, the quality of life for individuals with hyperacusis can be greatly compromised. For those with a severe intolerance to sound, it is difficult and sometimes impossible to function in an everyday environment with all its ambient noise. Hyperacusis can contribute to social isolation, phonophobia (fear of normal sounds) and depression.

Prevalence and Causes of Hyperacusis

Many people experience sensitivity to sound, but true hyperacusis is rare, affecting approximately one in 50,000 individuals. The disorder can affect people of all ages in one or both ears. Individuals are usually not born with hyperacusis, but may develop a narrow tolerance to sound.
Other common causes include:

  • Head injury
  • Ear damage from toxins or medication
  • Lyme disease
  • Air bag deployment
  • Viral infections involving the inner ear or facial nerve (Bell’s palsy)
  • Temporomandibular joint (TMJ) syndrome

There are a variety of neurologic conditions that may be associated with hyperacusis, including:

  • Post-traumatic stress disorder
  • Chronic fatigue syndrome
  • Tay-Sach’s disease
  • Some forms of epilepsy
  • Valium dependence
  • Depression
  • Migraine headaches

Hyperacusis is seen in brain-injured children (due to the universal sensory sensitivity), some autistic children and some children with cerebral palsy).

Diagnosis of Hyperacusis

Individuals who suspect they may have hyperacusis should seek an evaluation by an otolaryngologist (ear, nose and throat doctor). The initial consultation is likely to include a full audiologic evaluation (with a hearing test), a recording of medical history, and a medical evaluation by a physician. Counseling about evaluation findings and treatment options may also be provided at that time.

Treatment for Hyperacusis

There are no specific corrective surgical or medical treatments for hyperacusis. However, sound therapy may be used to retrain the auditory processing center of the brain to accept everyday sounds. This involves the use of a noise-generating device worn on the affected ear or ears. Those suffering from hyperacusis may be uncomfortable with placing sound directly in their ear, but the device produces a gentle static-like sound (white noise) that is barely audible. Completion of sound therapy may take up to 12 months, and usually improves sound tolerance. Because social situations are often painfully loud for those with hyperacusis, withdrawal, social isolation and depression are common.

Hearing Loss

Hearing tests usually indicate normal hearing sensitivity and often register at minus decibel levels. Counter to what one might think, this does not mean that those with hyperacusis hear better than others. Instead, it is a clear indication of a problem in the way the brain processes sound. Hearing loss coupled with low tolerance to sound is another, termed recruitment, a condition where soft sounds cannot be heard and loud sounds are intolerable (or distorted). For example, a person with recruitment may have hearing loss below 50 decibels while at the same time sound above 80 decibels may be intolerable. The result is a narrow range of comfortable hearing.

Relation to Tinnitus

Hyperacusis is strongly associated with tinnitus, a condition commonly referred to as ringing in the ears. Nearly 36 million Americans suffer from tinnitus; an estimated one of every thousand also has hyperacusis. Individuals can have tinnitus and hyperacusis at the same time.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

One of the most common birth defects is hearing loss or deafness (congenital), which can affect as many as three of every 1,000 babies born. Inherited genetic defects play an important role in congenital hearing loss, contributing to about 60% of deafness occurring in infants. Although exact data is not available, it is likely that genetics plays an important role in hearing loss in the elderly. Inherited genetic defects are just one factor that can lead to hearing loss and deafness, both of which may occur at any stage of a person’s lifespan. Other factors may include: medical problems, environmental exposure, trauma and medications.

The most common and useful distinction in hearing impairment is syndromic versus non-syndromic.

Non-syndromic hearing impairment accounts for the vast majority of inherited hearing loss, approximately 70%. Autosomal-recessive inheritance is responsible for about 80% of cases of non-syndromic hearing impairment, while autosomal-dominant genes cause 20%, less than two% of cases are caused by X-linked and mitochondrial genetic malfunctions.

Syndromic (sin-DRO-mik) means that the hearing impairment is associated with other clinical abnormalities. Among hereditary hearing impairments, 15 to 30% are syndromic. Over 400 syndromes are known to include hearing impairment and can be classified as: syndromes due to cytogenetic or chromosomal anomalies, syndromes transmitted in classical monogenic or Mendelian inheritance, or syndromes due to multi-factorial influences, and finally, syndromes due to a combination of genetic and environmental factors.

Variable expression of different aspects of syndromes is common. Some aspects may be expressed in a range from mild to severe or different combinations of associated symptoms may be expressed in different individuals carrying the same mutation within a single pedigree. An example of variable expressivity is seen in families transmitting autosomal dominant Waardenburg syndrome. Within the same family, some affected members may have dystopia canthorum (an unusually wide nasal bridge due to sideways displacement of the inner angles of the eyes), white forelock, heterochromia irides (two different-colored irises or two colors in the same iris), and hearing loss, while others with the same mutation may only have dystopia canthorum.

How Do Genes Work?

Genes are a road map for the synthesis of proteins, which are the building blocks for everything in the body: hair, eyes, ears, heart, lung, etc. Every child inherits half of its genes from one parent and half from the other parent. If the inherited genes are defective, a health disorder such as hearing loss or deafness can result. Hearing disorders are inherited in one of four ways:

Autosomal Dominant Inheritance: For autosomal dominant disorders, the transmission of a rare allele of a gene by a single heterozygous parent is sufficient to generate an affected child. A heterozygous parent has two types of the same gene (in this case, one mutated and the other normal) and can produce two types of gametes (reproductive cells). One gamete will carry the mutant form of the gene of interest and the other the normal form. Each of these gametes then has an equal chance of being used to form the offspring. Thus the chance that the offspring of a parent with an autosomal dominant gene will develop the disorder is 50 percent. Autosomal dominant traits usually affect males and females equally.

Autosomal Recessive Inheritance: An autosomal recessive trait is characterized by having parents who are heterozygous carriers for mutant forms of the gene in question but are not affected by the disorder. The problem gene that would cause the disorder is suppressed by the normal gene. These heterozygous parents (A/a) can each generate two types of gametes, one carrying the mutant copy of the gene (a) and the other having a normal copy of the gene (A). There are four possible combinations from each of the parents, A/a, A/A, a/A, and a/a. Only the offspring that inherits both mutant copies (a/a) will exhibit the trait. Overall, offspring of these two parents will face a 25 percent chance of inheriting the disorder.

X-linked Inheritance: A male offspring has an X chromosome and a Y chromosome, while a female has two copies of the X chromosome only. Each female inherits an X chromosome from her mother and her father. On the other hand, each male inherits an X chromosome from his mother and a Y chromosome from his father. In general, only one of the two X chromosomes carried by a female is active in any one cell while the other is rendered inactive. This is why when a female inherits a defective gene on one X chromosome, the normal gene on the other X chromosome can usually compensate. As males only have one copy of the X chromosome, any defective gene is more likely to manifest into a disorder.

Mitochondrial Inheritance: Mitochondrias, small powerhouses within each cell, also contain their own DNA. Interestingly, the sperm does not have any mitochondria, and consequently, only the mitochondria in the egg from the mother can be passed from one generation to the next. This leads to an interesting inheritance pattern where only affected mothers (and not affected fathers as their sperms do not have mitochondria) can pass on a disease from one generation to the next. Sensitivity to aminoglycoside antibiotics can be inherited through a defect in mitochondrial DNA and is the most common cause of deafness in China!

In the last decade, advances in molecular biology and genetics have contributed substantially to the understanding of development, function and pathology of the inner ear. Researchers have identified several of the various genes responsible for hereditary deafness or hearing loss, most notably the GJB2 gene mutation. As one of the most common genetic causes of hearing loss, GJB2-related hearing loss is considered a recessive genetic disorder because the mutations only cause deafness in individuals who inherit two copies of the mutated gene, one from each parent. A person with one mutated copy and one normal copy is a carrier but is not deaf. Screening tests for the GJB2 gene are available for at risk individuals to help them determine their risk of having a child with hearing problems.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

¿Qué es la otitis media?

Otitis media se refiere a la inflamación del oído medio. Cuando la infección ocurre esta condición es llamada “otitis media aguda”. La otitis media aguda ocurre cuando un resfrío, alergia o infección de las vías respiratorias superiores y la presencia de bacterias o virus llevan a la acumulación de pus y moco detrás de la membrana timpánica bloqueando la trompa de Eustaquio. Esto causa dolor de oído e hinchazón.

Cuando se forma líquido en el oído medio, la condición es conocida como “otitis media con efusión”. Esto sucede en una infección en recuperación o cuando una infección esta por ocurrir. El líquido puede permanecer en el oído por semanas hasta algunos meses. Cuando una descarga del oído persiste o se hace recurrente es llamada a veces infección crónica de oído medio. El líquido puede permanecer en el oído hasta tres semanas después de la infección. Si no es tratada, la infección crónica del oído puede tener consecuencias potenciales serias como pérdida auditiva transitoria o permanente.

¿Como afecta la otitis media la audición del niño?

Todos los chicos con infección de oído medio o líquido tienen un grado de pérdida auditiva. La pérdida promedio en oídos con secreción es de 24 decibeles, equivalente a usar auriculares (24 decibeles es el nivel aproximado de los silbidos leves). Líquidos más espesos pueden causar una pérdida mucho mayor, de hasta 45 decibles (el nivel de la conversación normal)

Su niño puede tener pérdida de la audición si no es capaz de entender ciertas palabras y habla a un volumen mayor del normal. Básicamente, un chico con pérdida auditiva debida a infecciones de oído medio escuchará sonidos poco claros y perderá de entender algunos diálogos en menor medida que aquellos con hipoacusias profundas. De todas formas las consecuencias pueden ser importantes – el paciente joven pueden perder en forma permanente la habilidad de entender en forma concisa el diálogo en ambientes ruidosos (como el aula de la escuela) llevando a un retraso en el aprendizaje de importantes habilidades de lenguaje.

Tipos de hipoacusias

La hipoacusia conductiva es una forma de dificultad auditiva debida a una lesión el en canal auditivo externo o en el oído medio. Esta forma de hipoacusia es generalmente transitoria y se encuentra en personas de 40 años o menos. Infecciones de oído crónicas no tratadas pueden llevar a una hipoacusia conductiva; drenar el oído medio infectado a través de la membrana timpánica lleva de nuevo la audición a la normalidad.

La otra forma de hipoacusia es la neurosensorial, pérdida auditiva debida a una lesión en la rama auditiva del VIII par craneal o del oído interno. Históricamente esta condición es más prevalente en la edad media o pacientes mayores, sin embargo la exposición continua a música a un alto volumen puede llevar a la pérdida auditiva neurosensorial en adolescentes.

¿Cuándo se debe realizar un examen de audición?

Un examen de audición se debe llevar a cabo en chicos que tienen infecciones de oído frecuentes, pérdidas auditivas que duran mas de seis semanas o líquido en el oído medio por mas de tres meses. Hay una amplia gama de instrumentos para evaluar la audición del niño, la función de la trompa de Eustaquio y la movilidad de la membrana del tímpano. Ellos incluyen otoscopía, timpanometría y audiometría.

¿Pierden los niños su audición por otras razones aparte de la otitis media crónica?

Los niños pueden tener pérdidas auditivas temporarias por otras razones más allá de la infección crónica del oído medio y la disfunción de la trompa de Eustaquio. Ellas son:

  • Impactación de cerumen (tapón de cera compresivo)
    Otitis externa: Inflamación del canal auditivo externo, también llamado oído de nadador.
  • Colesteatoma: Una masa de tejido epitelial escamoso y colesterol en el oído medio, generalmente resultado de una otitis media crónica.
  • Otoesclerosis: Enfermedad del laberinto óseo en el oído que es más común en los adultos y caracterizado por la formación de hueso que lleva a la progresiva hipoacusia conductiva. Ocurre debido a la fijación del estribo (uno de los huesecillos del oído). Hipoacusia neurosensorial puede resultar cuando esta involucrado el conducto coclear.
  • Trauma: Un trauma del oído o la cabeza puede causar una pérdida auditiva transitoria o permanente.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

What is otitis media?

Otitis media refers to inflammation of the middle ear. When infection occurs, the condition is called “acute otitis media”. Acute otitis media occurs when a cold, allergy, or upper respiratory infection, and the presence of bacteria or viruses lead to the accumulation of pus and mucus behind the eardrum, blocking the Eustachian tube. This causes earache and swelling.

When fluid forms in the middle ear, the condition is known as “otitis media with effusion”. This occurs in a recovering ear infection or when one is about to occur. Fluid can remain in the ear for weeks to many months. When a discharge from the ear persists or repeatedly returns, this is sometimes called chronic middle ear infection. Fluid can remain in the ear up to three weeks following the infection. If not treated, chronic ear infections have potentially serious consequences such as temporary or permanent hearing loss.

How does otitis media affect a child’s hearing?

All children with middle ear infection or fluid have some degree of hearing loss. The average hearing loss in ears with fluid is 24 decibels – equivalent to wearing ear plugs. (Twenty-four decibels is about the level of the very softest of whispers.) Thicker fluid can cause much more loss, up to 45 decibels (the range of conversational speech).

Your child may have hearing loss if he or she is unable to understand certain words and speaks louder than normal. Essentially, a child experiencing hearing loss from middle ear infections will hear muffled sounds and misunderstand speech rather than incur a complete hearing loss. Even so, the consequences can be significant – the young patient could permanently lose the ability to consistently understand speech in a noisy environment (such as a classroom), leading to a delay in learning important speech and language skills.

Types of hearing loss

Conductive hearing loss is a form of hearing impairment due to a lesion in the external auditory canal or middle ear. This form of hearing loss is usually temporary and found in those ages 40 or younger. Untreated chronic ear infections can lead to conductive hearing loss; draining the infected middle ear drum will usually return hearing to normal.

The other form of hearing loss is sensorineural hearing loss, hearing loss due to a lesion of the auditory division of the 8th cranial nerve or the inner ear. Historically, this condition is most prevalent in middle age and older patients; however, extended exposure to loud music can lead to sensorineural hearing loss in adolescents.

When should a hearing test be performed?

A hearing test should be performed for children who have frequent ear infections, hearing loss that lasts more than six weeks, or fluid in the middle ear for more than three months. There are a wide range of medical devices now available to test a child’s hearing, Eustachian tube function and reliability of the ear drum. They include the otoscopy, tympanometer and audiometer.

Do children lose their hearing for reasons other than chronic otitis media?

Children can incur temporary hearing loss for other reasons than chronic middle ear infection and Eustachian tube dysfunction. They include:

  • Cerumen impaction (compressed earwax)
  • Otitis externa: Inflammation of the external auditory canal, also called “swimmer’s ear”.
  • Cholesteatoma: A mass of horn-shaped squamous cell epithelium and cholesterol in the middle ear, usually resulting from chronic otitis media.
  • Otosclerosis: This is a disease of the otic capsule (bony labyrinth) in the ear, which is more prevalent in adults and characterized by formation of soft, vascular bone leading to progressive conductive hearing loss. It occurs due to fixation of the stapes (bones in the ear). Sensorineural hearing loss may result because of involvement of the cochlear duct.
  • Trauma: A trauma to the ear or head may cause temporary or permanent hearing loss.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

Otitis media means “inflammation of the middle ear”, as a result of a middle ear infection. It can occur in one or both ears. Otitis media is the most frequent diagnosis for children who visit physicians for illness. It is also the most common cause of hearing loss in children. Although otitis media is most common in young children, it occasionally affects adults.

Is it serious?

Yes, because of the severe earache and hearing loss it can cause. Hearing loss, especially in children, may impair learning capacity and even delay speech development. However, if it is treated promptly and effectively, hearing can almost always be restored to normal. Otitis media is also serious because the infection can spread to nearby structures in the head, especially the mastoid. (see the symptoms list below). Immediate attention from your doctor is the best action.

How does the ear work?

The outer ear collects sounds. The middle ear is a pea-sized, air-filled cavity separated from the outer ear by the paper-thin eardrum. Inside the middle ear are three tiny ear bones. When sound waves strike the eardrum, it vibrates and sets the bones in motion that transmit to the inner ear. The inner ear converts vibrations to electrical signals and sends these signals to the brain. A healthy middle ear has the same atmospheric pressure as air outside of the ear, allowing free vibration. Air enters the middle ear through the narrow eustachian tube that connects the back of the nose to the ear.

What causes otitis media?

Blockage of the eustachian tube during a cold, allergy or upper respiratory infection, and the presence of bacteria or viruses lead to a build-up of pus and mucus behind the eardrum. This infection is called acute otitis media. The build-up of pressurized pus in the middle ear causes pain, swelling and redness. Since the eardrum cannot vibrate properly, hearing problems may occur. Sometimes the eardrum ruptures, and pus drains out of the ear. More commonly, however, the pus and mucus remain in the middle ear due to the swollen and inflamed eustachian tube. This is called middle ear effusion or serous otitis media. Often after the acute infection has passed, the effusion remains lasting for weeks, months or even years. This condition allows frequent recurrences of the acute infection and may cause difficulty in hearing.

What will happen at the doctor’s office?

During an examination, the doctor will use an otoscope to look at and assess the ear. The doctor checks for redness in the ear, and/or fluid behind the eardrum, and to see if the eardrum moves. These are the signs of an ear infection. Two other tests may also be performed:

  • Audiogram – tests if hearing loss has occurred by presenting tones at various pitches.
  • Tympanogram – measures the air pressure in the middle ear to see how well the eustachian tube is working and how well the eardrum can move.

How should medication be taken?

It is important that all the medications be taken as directed and that you keep any follow-up visits. Often, antibiotics to fight the infection will make the earache go away rapidly, but the infection may need more time to clear up. Other medications that your doctor may prescribe include an antihistamine (for allergies), a decongestant (especially with a cold) or both. Sometimes the doctor may recommend a medication to reduce fever and/or pain. Special ear drops can ease the pain. Call your doctor if you have any questions about your or your child’s medication, or if symptoms do not clear.

What other treatment may be necessary?

If your child experiences multiple episodes of acute otitis media within a short time, or hearing loss, or chronic otitis media lasts for more than three months, your physician may recommend referral to an otolaryngologist for placement of ventilation tubes, also called pressure-equalization (PE) tubes. This is a short surgical procedure in which a small incision is made in the eardrum, any fluid is suctioned out, and a tube is placed in the eardrum. This tube eventually will fall out on its own and the eardrum heals. There is usually an improvement in hearing and a decrease in further infections with PE tube placement.
Otitis media may recur as a result of chronically infected adenoids and tonsils. If this becomes a problem, your doctor may recommend removal of one or both. This can be done at the same time as ventilation tubes are inserted.

What are the symptoms?

In infants and toddlers, look for: pulling or scratching at the ear, especially if accompanied by other symptoms, hearing problems, crying, irritability, fever, ear drainage.

In young children, adolescents and adults look for: earache, feeling of fullness or pressure, hearing problems, dizziness, loss of balance, nausea, vomiting, ear drainage and/or fever.

Remember, without proper treatment, damage from an ear infection can cause chronic or permanent hearing loss.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

Feeling unsteady or dizzy can be caused by many factors such as poor circulation, inner ear disease, medication usage, injury, infection, allergies and/or neurological disease. Dizziness is treatable, but it is important for your doctor to help you determine the cause so that the correct treatment is implemented. While each person will be affected differently, symptoms that warrant a visit to the doctor include a high fever, severe headache, convulsions, ongoing vomiting, chest pain, heart palpitations, shortness of breath, inability to move an arm or leg, a change in vision or speech, or hearing loss.

What is dizziness?

  • Dizziness can be described in many ways, such as feeling lightheaded, unsteady, giddy or feeling a floating sensation. Vertigo is a specific type of dizziness experienced as an illusion of movement of one’s self or the environment. Some experience dizziness in the form of motion sickness, a nauseating feeling brought on by the motion of riding in an airplane, a roller coaster or a boat. Dizziness, vertigo and motion sickness all relate to the sense of balance and equilibrium. Your sense of balance is maintained by a complex interaction of the following parts of the nervous system:
    The inner ear (also called the labyrinth), which monitors the directions of motion, such as turning, rolling, forward-backward, side-to-side and up-and-down motions.
  • The eyes, which monitor where the body is in space (i.e., upside down, right side up, etc.) and also directions of motion.
  • The pressure receptors in the joints of the lower extremities and the spine, which tell what part of the body is down and touching the ground.
  • The muscle and joint sensory receptors (also called proprioception) tell what parts of the body are moving.
  • The central nervous system (the brain and spinal cord), which processes all the information from the four other systems to maintain balance and equilibrium.

The symptoms of motion sickness and dizziness appear when the central nervous system receives conflicting messages from the other four systems.

What causes dizziness?

Circulation: If your brain does not get enough blood flow, you feel lightheaded. Almost everyone has experienced this on occasion when standing up quickly from a lying-down position. But some people have light-headedness from poor circulation on a frequent or chronic basis. This could be caused by arteriosclerosis or hardening of the arteries, and it is commonly seen in patients who have high blood pressure, diabetes or high levels of blood fats (cholesterol). It is sometimes seen in patients with inadequate cardiac (heart) function, hypoglycemia (low blood sugar) or anemia (low iron).

Certain drugs also decrease the blood flow to the brain, especially stimulants such as nicotine and caffeine. Excess salt in the diet also leads to poor circulation. Sometimes circulation is impaired by spasms in the arteries caused by emotional stress, anxiety and tension.

If the inner ear fails to receive enough blood flow, the more specific type of dizziness – vertigo – occurs. The inner ear is very sensitive to minor alterations of blood flow and all of the causes mentioned for poor circulation to the brain also apply specifically to the inner ear.

Neurological diseases: A number of diseases of the nerves can affect balance, such as multiple sclerosis, syphilis, tumors, etc. These are uncommon causes, but your doctor may perform certain tests to evaluate these.

Anxiety: Anxiety can be a cause of dizziness and lightheadedness. Unconscious overbreathing (hyperventilation) can be experienced as overt panic, or just mild dizziness with tingling in the hands, feet or face. Instruction on correct breathing technique may be required.

Vertigo: An unpleasant sensation of the world rotating, usually associated with nausea and vomiting. Vertigo usually is due to an issue with the inner ear. The common causes of vertigo are (in order):

  • Benign Positional Vertigo: Vertigo is experienced after a change in head position such as lying down, turning in bed, looking up or stooping. It lasts about 30 seconds and ceases when the head is still. It is due to a dislodged otololith crystal entering one of the semicircular balance canals. It can last for days, weeks or months. The Epley “repositioning” treatment by an otolaryngologist is usually curative. BPV is the commonest cause of dizziness after (even a mild) head injury.
  • Ménière’s disease: An inner ear disorder with attacks of vertigo (lasting hours), nausea or vomiting, and tinnitus (loud noise) in the ear, which often feels blocked or full. There is usually a decrease in hearing as well.
  • Migraine: Some individuals with a prior classical migraine headache history can experience vertigo attacks similar to Ménière’s disease. Usually there is an accompanying headache, but can also occur without the headache.
  • Infection: Viruses can attack the inner ear, but usually its nerve connections to the brain, causing acute vertigo (lasting days) without hearing loss (termed vestibular neuronitis). However, a bacterial infection such as mastoiditis that extends into the inner ear can completely destroy both the hearing and equilibrium function of that ear, called labyrinthitis.
  • Injury: A skull fracture that damages the inner ear produces a profound and incapacitating vertigo with nausea and hearing loss. The dizziness will last for several weeks and slowly improve as the other (normal) side takes over. BPV commonly occurs after head injury.
  • Allergy: Some people experience dizziness and/or vertigo attacks when they are exposed to foods or airborne particles (such as dust, molds, pollens, dander, etc.) to which they are allergic.

When should I seek medical attention?

Call 911 or go to an emergency room if you experience:

  • Dizziness after a head injury,
    Fever over 101° F, headache or very stiff neck,
  • Convulsions or ongoing vomiting,
  • Chest pain, heart palpitations, shortness of breath, weakness, a severe headache, inability to move an arm or leg, change in vision or speech, or
  • Fainting and/or loss of consciousness

Consult your doctor if you:

  • Have never experienced dizziness before,
  • Experience a difference in symptoms you have had in the past,
  • Suspect that medication is causing your symptoms, or
  • Experience hearing loss.

How will my dizziness be treated?

The doctor will ask you to describe your dizziness and answer questions about your general health. Along with these questions, your doctor will examine your ears, nose and throat. Some routine tests will be performed to check your blood pressure, nerve and balance function, and hearing. Possible additional tests may include a CT or MRI scan of your head, special tests of eye motion after warm or cold water or air is used to stimulate the inner ear (ENG – electronystagmography or VNG – videonystagmography), and in some cases, blood tests or a cardiology (heart) evaluation. Balance testing may also include rotational chair testing and posturography. Your doctor will determine the best treatment based on your symptoms and the cause of them. Treatments may include medications and balance exercises.

Prevention tips:

  • Avoid rapid changes in position
  • Avoid rapid head motion (especially turning or twisting)
  • Eliminate or decrease use of products that impair circulation, e.g., tobacco, alcohol, caffeine and salt
  • Minimize stress and avoid substances to which you are allergic
  • Get enough fluids
  • Treat infections, including ear infections, colds, flu, sinus congestion and other respiratory infections

If you are subject to motion sickness:

  • Do not read while traveling
  • Avoid sitting in the rear seat
  • Do not sit in a seat facing backward
  • Do not watch or talk to another traveler who is having motion sickness
  • Avoid strong odors and spicy or greasy foods immediately before and during your travel
  • Talk to your doctor about medications

Remember: Most cases of dizziness and motion sickness are mild and self-treatable. But severe cases and those that become progressively worse deserve the attention of a doctor with specialized skills in diseases of the ear, nose, throat, equilibrium and neurological systems.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery

How does the ear hear sounds?

The ear can be divided into three parts: 1) the external ear includes the pinna (outer, visible ear) and the ear canal, 2) the middle ear includes the tympanic membrane (ear drum) and the ossicles (middle ear bones), 3) the inner ear, which includes the cochlea (organ of hearing) and vestibule (organ of balance).
Sound waves enter the ear canal and cause a vibration of the tympanic membrane (ear drum) which is then passed through three tiny bones behind the ear drum in the middle ear space: the malleus (hammer), incus (anvil) and stapes (stirrup). The sound vibrations in the ossicles are then transmitted to the nerves and fluids in the cochlea (inner ear), which generates a nerve impulse that passes along the auditory nerve to the brain.

What are the types of hearing loss?

Hearing loss can be divided into two types: Conductive Hearing Loss, which is essentially a mechanical problem with the conduction of sound vibrations, and Sensorineural Hearing Loss, a problem with the generation and/or transmission of nerve impulses from the inner ear to the brain. Mixed hearing loss refers to a combination of these two types. The preliminary classification of hearing loss as conductive or sensorineural can be determined by a physician using a tuning fork in the office. A formal audiogram, or hearing test, is the best way to determine the type and degree of hearing loss. The distinction between these two types of hearing loss is important because many cases of conductive hearing loss can be improved with medical or surgical intervention. An otolaryngologist, also called an ear, nose and throat (or ENT) doctor, can determine the specific diagnosis and treatments for either type of hearing loss and perform surgical treatments, if necessary.

What can cause Conductive Hearing Loss?

Conductive hearing loss may result from diseases that affect the external ear or middle ear structures. Some of the causes of conductive hearing loss include:

Problems with the External Ear

  • Cerumen (ear wax) obstruction: Ear wax can be identified by a medical examination and can usually be removed quickly. This condition may actually be aggravated by cotton tipped applicators (Q-tips) that many patients use in an attempt to clean their ears.
  • Otitis Externa: Often referred to as “swimmer’s ear”, an infection of the ear canal may be related to water exposure. Although the most common symptoms of otitis externa are pain and tenderness of the ear, conductive hearing loss can also occur if there is severe swelling of the ear canal.
  • Foreign body in Ear Canal: This is also readily identified on examination and can usually be cleared in the office. Occasionally, a brief anesthesia is required for this procedure in children. Common foreign bodies include beads and beans in children and cotton or the tips of cotton-tipped applicators in adults. Uncommonly, the foreign object is a live bug such as a cockroach which can cause itching, pain and noise.
  • Bony lesions of Ear Canal: These are benign growths of bone along the walls of the ear canal resulting in a narrowing of the ear canal which may then lead to frequent obstruction from a small amount of wax or water. These bony lesions can generally be managed with vigilant cleaning of ear wax to prevent obstruction. In rare cases these lesions require surgical removal.
  • Atresia of the Ear Canal: Complete malformation of the external ear canal is called atresia. It may be seen along with complete or partial malformation of the pinna (outer ear) and is noted at birth. It is rarely associated with other congenital abnormalities and is most often only on one side (unilateral). Management of congenital aural atresia is complex. Surgical treatment may be beneficial to either reconstruct the ear canal in select cases or to implant a device that vibrates the bone of the ear directly.

Problems with the Middle Ear structures

  • Middle Ear Fluid or Infection (otitis media): The middle ear space may be filled with fluid instead of air. Otitis media is divided into three types: acute otitis media, serous otitis media (middle ear fluid) or chronic otitis media. Acute otitis media occurs rapidly, is painful and may cause fever. Serous otitis media often follows an acute otitis media infection or may occur on its own. Both conditions are common in children and are related to an inability to ventilate the middle ear space due to poor Eustachian tube function (the channel which connects the middle ear space with the nasal passage). Otitis media may be treated medically or with a myringotomy with tube insertion (also known as an M&T or ear tube surgery). In most adults, an M&T surgery may be performed in the office. In children, a brief general anesthesia is usually required. Chronic otitis media is associated with damage to the ear drum or ossicles (middle ear bones), and frequently requires surgery.
  • Tympanic Membrane Atelectasis or Retraction (collapse of the ear drum): Poor Eustachian tube function may also result in excessive negative pressure behind the ear drum causing the ear drum to collapse onto the middle ear bones. Severe retraction of the ear drum may necessitate ear tube surgery or a surgery to rebuild the ear drum (tympanoplasty).
  • Tympanic Membrane Perforation: A hole in the ear drum due to infections or trauma may result in hearing loss as the sound vibrations are not effectively captured by the damaged ear drum. A tympanoplasty is the surgical repair of the ear drum. Generally, this is an outpatient surgery performed by an otolaryngologist with a very high success rate (over 90%).
  • Cholesteatoma: This may develop when the ear drum collapses to the point that the outer skin of the ear drum grows into the middle ear and becomes trapped. In spite of the ending of the word cholesteatoma, this is not a tumor but a benign collection of skin that can cause destruction of the middle ear structures and, if left untreated, more serious problems. This is almost always a surgical disease and may require a staged surgical approach (more than one surgery) in order to safely remove the cholesteatoma and restore hearing by repairing the damaged middle ear bones.
  • Damage to the Middle Ear Ossicles: This may result from trauma, infection, cholesteatoma or a retracted ear drum leading to conductive hearing loss. Surgical reconstruction of the ossicular chain is often successful in restoring hearing in these cases.
  • Otosclerosis: This is an inherited disease in which the bone around the stapes bone hardens and the stapes fails to vibrate effectively. The conductive hearing loss slowly progresses in early adulthood. It affects women more often than men and affects slightly less than 1% of the population overall. This condition may be treated with a hearing aid or with a stapedectomy surgery which is highly effective in restoring hearing in most cases.

Many types of hearing loss can also be ameliorated with the use of conventional hearing aids. In addition, many implantable hearing devices are available for various types of hearing loss. An otolaryngologist can determine the specific cause of the hearing loss, advise patients of their treatment and rehabilitative options, and help patients achieve the best possible hearing outcome and hearing related quality of life.

© 2016 American Academy of Otolaryngology – Head and Neck Surgery