Pediatric Ear Disease
Aural Atresia or Microtia (malformations of the ear)
Aural atresia is a condition that occurs in utero when the ear canal fails to form properly and instead of being open like a tunnel, the canal remains closed. This occurs more commonly on one side but may also occur bilaterally. Aural atresia is seen in about 1 in 10,000-20,000 births.
Microtia is a congenital deformity where the pinna (external ear) is underdeveloped or absent. A completely absent pinna is referred to as anotia. Atresia and microtia can occur together.
This condition causes the patient to have a conductive hearing loss. If this occurs bilaterally, the hearing loss can interfere with normal development and cause developmental delays. In these situations, the patient should be fitted with a bone conduction hearing device as soon as possible to avoid these delays.
After 6 years of age, surgical repair of atresia to open the canal may be considered. To assess if the patient meets criteria for surgical intervention, we will order a hearing test to see if the surgery could improve the patients’ hearing, as well as a CT scan of the head to further assess the middle and inner ear structures. If the patient qualifies, surgery may be appropriate and is a decision made by the parents and/or child. Cosmetic repairs can be made for microtia to replace external ear structures.
Tympanic membrane perforation
Tympanic membrane perforation is the rupture or presence of a hole in the eardrum. The eardrum acts as a barrier between the outer and middle ear and aids in the transmission of sound vibrations to the small bones of hearing. Tympanic membrane perforations can lead to hearing loss. The degree of hearing loss is related to the size and location of the perforation. Tympanic membrane perforations often occur in children after numerous sets of ear tubes, or after chronic ear infections.
Surgical repair is indicated in patients with associated hearing loss, frequent ear infections, or with patient preference. In children, often the perforation is watched until the child grows older to guarantee they will not have recurrence of eustachian tube dysfunction. Once surgery is indicated, Dr. Cristobal performs cartilage tympanoplasty harvested from the tragus.
EUSTACHIAN TUBE DYSFUNCTION
This is one of the most common ear related problems in young children. The Eustachian tube is a tube that sits behind your sinuses and connects the space behind the nose to the middle ear space behind the eardrum. Its job is to protect the middle ear by maintaining an equalized pressure within the middle ear and draining out middle ear fluids. Children commonly have problems with their Eustachian tube as they are often horizontally oriented rather than downward, as in adults. A poorly functioning Eustachian tube keeps it from allowing air into the middle ear, which can cause negative pressure or a buildup of fluid, eventually leading to middle ear effusions and hearing loss.
If medical management with allergy medications and nasal steroids is not enough, surgical intervention includes placement of pressure equalization tubes to prevent infection and hearing loss. Some children will need more than one set of tubes during their period of Eustachian tube dysfunction. In adults, pressure equalization tubes can be placed in the office by one of our trained providers.
ACUTE/Chronic Otitis Media
Acute otitis media is very common in children and is generally very easily treated with oral antibiotics. Chronic otitis media is a persistent infection of the middle ear characterized by fluid accumulation, drainage and/or pain lasting over 3 months. This infection is caused by the trapping of fluid behind the eardrum due to a malfunctioning Eustachian tube (drainage tract). Chronic otitis media can lead to hearing loss and/or eardrum perforation. It can also be a sign of a more complicated issue such as the presence of a skin cyst (cholesteatoma). Treatment of the infection may consist of ear-drops, topical powders, or oral medications.
In addition, it is very important to correct the cause of the problem by addressing the Eustachian tube. Eustachian tube dysfunction can be improved by allergy control or surgical intervention by placing pressure equalization tubes in the ear drums. In children, this is performed in the operating room. Once tubes are placed in the ear drum, topical antibiotic drops can be used to reach the middle ear space during an ear infection.
Attributed © Krames Staywell
MASTOIDITIS
Mastoiditis is inflammation often resulting from infection of the mastoid air cells located behind the ear. When it occurs as a complication of a middle ear infection (otitis media) it may be life threatening if untreated. Fortunately since the modern use of antibiotics as well as childhood vaccinations against H. influenzae, the number of cases of otitis media progressing to mastoiditis has greatly decreased, and the condition is now considered rare. Symptoms of mastoiditis can mimic those of a middle ear infection including fever, ear pain, and a conductive hearing loss. Others may experience tenderness, redness, or swelling behind the ear. Based on symptoms and physical exam findings, if the clinician suspects mastoiditis, the diagnosis can be confirmed with CT imaging of the temporal bone.
Surgical treatment may include making an incision in the eardrum (myringotomy) or placing a pressure equalization tube in the eardrum to allow drainage of the middle ear. Also, intravenous antibiotic treatment may be indicated to quickly and effectively eradicate the infection. Treatment warrants close observation. If mastoiditis persists despite proper management, further surgical intervention to drain the infected contents of the mastoid air cells may be indicated and a mastoidectomy will be performed. The goals is to prevent further spread of the infection or erosion of the bone leading to meningitis (inflammation of the lining of the brain).
Cholesteatoma of the ear
Cholesteatomas are an accumulation of dead tissue within the middle ear space. Eustachian tube dysfunction (blockage of the drainage path of the middle ear) creates an environment of decreased (negative) pressure within the middle ear, which causes the eardrum to retract. When the drum retracts, it can create a space for the pocketing of debris. This pocket can grow and become a nest of infection that erodes surrounding bones. Cholesteatomas are associated with symptoms of chronic ear drainage, pain, fullness, hearing loss, and dizziness. The retraction pocket of a cholesteatoma can be identified during an office visit but usually requires a CT scan for full evaluation and diagnosis.
In order to prevent further growth and progression of symptoms, treatment involves surgical removal. Following surgery, 30-50% of cholesteatomas recur due to continued Eustachian tube dysfunction. Therefore, it is important for continued follow-up appointments to be maintained in order to evaluate for re-occurrence. During surgery, it is often necessary to remove the bones of hearing due to erosion or involvement in the cholesteatoma. If this occurs, then a titanium ossicular prosthesis will replace the bones of hearing. In some cases of large cholesteatomas, it will be necessary to perform a second-look surgery during which the bones of hearing will be reconstructed with a prosthesis. After having a cholesteatoma, hearing may not return to “normal” but may be improved following its removal. Therefore, the primary goal of removal surgery is not to achieve baseline hearing but is to create a safe, infection free middle ear.
PEDIATRIC HEARING LOSS
CONGENITAL HEARING LOSS
Congenital hearing loss simply means hearing loss was present at birth. It can be caused from genetic disorders (hereditary) or from problems occurring during pregancy or birth.
Nongenetic factors could include:
Maternal infections such as Toxomoplasmosis, Rubella, Cytomegalovirus, and Herpes simplex virus (TORCH)
Maternal consumption of toxins, certain drugs, or alcohol during pregnancy
Maternal Toxemia
Gestational Diabetes
Anoxia (Lack of oxygen) in utero
Birth injuries
Prematurity
Low birth weight
Jaundice or hyperbilirubinemia
Genetic Factors are more commonly the cause of congenital hearing loss. Syndromes associated with hearing loss include:
Down Syndrome
Waardenburg Syndrome
Usher syndrome
Treacher Collins syndrome
Crouzon syndrome
Alport syndrome
Many of the causes of congenital hearing loss, also involve problems with other systems of the body. For this reason, when congenital hearing loss is suspected, in many cases the patient will receive an evaluation by a genetics specialist and ophthalmologist and will also need imaging of the kidneys and the brain.
Regardless of the cause of hearing loss, it is required that children are diagnosed with hearing loss by 6 months of age and are treated within the 1st year of life. To ensure this, in the United States, hearing screening is done just after birth. If a baby does not pass the hearing screen, they are immediately sent for further evaluation. Hearing plays a crucial part in normal growth and development and if present can cause speech delay as well as language and social problems. So if hearing loss is suspected it is important to seek care as soon as possible.
Treatment for congenital hearing loss can vary depending on the type and severity of hearing loss experienced. This could include hearing aids, a bone anchored hearing aid (BAHA), or implantation with a cochlear implant, as discussed above. Treatment will require a collaborative approach with routine monitoring of hearing, frequent adjustments of hearing device, and speech therapy.
Sensorineural (nerve) Hearing Loss
Sensorineural hearing loss occurs when there is damage to or dysfunction of the cochlear hair cells or cochlear nerve. This type of hearing loss if often permanent. This can lead to a decrease in perceived sound loudness, with or without deterioration of the clarity of the sound. This may manifest itself as difficulties with speech understanding, which are usually most noticeable in background noise. The causes can range from congenital (present at birth), infectious viral causes, autoimmune disease, or progressive hearing loss related to age, loud sound exposure, or genetics. Oftentimes, no exact cause can be determined.
Children with sensorineural hearing loss can have difficulties learning speech and may need speech therapy even after they are amplified. It is very important to provide either hearing aids or cochlear implants to children with early onset sensorineural hearing loss so they may begin speech and language acquisition as early as possible.
A cochlear implant is a hearing implant that is placed in the cochlea of the inner ear. Cochlear implants directly stimulate the nerve of hearing through an electrode array, thus bypassing the ear canal, eardrum, middle and inner ear. The inner piece is implanted surgically underneath the scalp and into the skull bone behind the ear, while a hearing aid-type outer piece is worn either over the ear, clipped to the clothing, or on an armband. This implant is used for patients who have severe to profound hearing loss in both ears and difficulty understanding speech.
Dr. Cristobal has performed cochlear implants in children of all ages and works closely with the Cook Children’s Hospital Audiology and Speech Therapy programs. He has successfully implanted the second youngest patient in the nation at 3 months of age.
For more information about cochlear implants for your child, please click the link below.
Central Auditory Processing Disorder
Central Auditory Processing Disorder (CAPD) is a term used to describe a processing disorder that may vary based on the perspective of the professional describing the problem. Symptoms include difficulty localizing sound, difficulty understanding spoken language in competing messages or background noise, taking longer to respond in oral communication situations, inconsistent or inappropriate responding, difficulty comprehending and following rapid speech or complex auditory directions. Patients with CAPD can have conductive, sensorineural, and mixed hearing loss, as well as auditory neuropathy. Appropriate audiological, as well as speech and behavioral tests should be administered by an experienced professional.
Treatment includes using a team approach to assess all needs. Depending on the needs of the individual, team members may include an audiologist, speech language pathologist, a teacher, parents, and a counselor.