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Congenital preauricular fistula is a type of congenital ear anomaly that affects the external ear. It is characterized by a small opening or fistula near the ear that is present at birth. This condition is relatively uncommon and affects approximately 1 in 10,000 individuals.

There are several different types of congenital preauricular fistulas, which can be classified based on their location, size, and shape. Some of the most common types include:

Simple preauricular fistula: This is the most common type of congenital preauricular fistula and is characterized by a single, small opening near the ear.
Multiple preauricular fistulas: In some cases, individuals may have more than one opening near the ear. These multiple fistulas can be located in different areas of the ear or grouped together in one area.
Branching preauricular fistulas: This type of congenital preauricular fistula is characterized by multiple openings that branch off from a single main opening.
Cystic preauricular fistulas: This type of congenital preauricular fistula is characterized by a small cyst or sac-like structure near the ear. The cyst may contain fluid or other materials and may need to be drained if it becomes infected.
Sinus tract preauricular fistulas: This type of congenital preauricular fistula is characterized by a deep opening that leads to a sinus tract or canal. The sinus tract may extend into the surrounding tissues and can be associated with other anomalies, such as deafness or facial nerve paralysis.

Causes

Potential causes of preauricular fistulas:

Genetics: Preauricular fistulas can be inherited as a dominant or recessive trait. If a person has a family history of preauricular fistulas, they may be more likely to have this condition themselves.
Environmental factors: Exposure to certain environmental toxins or chemicals during pregnancy may increase the risk of preauricular fistulas.
Teratogens: Certain drugs, such as thalidomide, that are known to cause birth defects may also increase the risk of preauricular fistulas.
Maternal infection: Infections during pregnancy, such as rubella or cytomegalovirus, can increase the risk of preauricular fistulas in the developing fetus.
Maternal age: Older mothers are more likely to have children with preauricular fistulas, perhaps due to an increased risk of chromosomal abnormalities.
Maternal smoking: Pregnant women who smoke are more likely to have children with preauricular fistulas, possibly due to decreased oxygenation of the fetus.
Low birth weight: Infants born with a low birth weight are more likely to have preauricular fistulas.
Prematurity: Preterm infants are more likely to have preauricular fistulas than full-term infants.
Multiple pregnancies: Women who have multiple pregnancies are more likely to have children with preauricular fistulas.
Ethnicity: Preauricular fistulas are more common in certain ethnic populations, such as African Americans and Asians.
Neural crest cells: Preauricular fistulas may be caused by a problem with the migration of neural crest cells during embryonic development.
Abnormal folding of the ear: Preauricular fistulas may result from abnormal folding of the ear during embryonic development.
Abnormal growth of the ear: Preauricular fistulas may be caused by abnormal growth of the ear during embryonic development.
Congenital heart defects: Children with congenital heart defects are more likely to have preauricular fistulas.
Cleft lip and palate: Children with cleft lip and palate are more likely to have preauricular fistulas.
Other craniofacial anomalies: Children with other craniofacial anomalies, such as craniosynostosis or microtia, are more likely to have preauricular fistulas.
Down syndrome: Children with Down syndrome are more likely to have preauricular fistulas.
William syndrome: Children with William syndrome are more likely to have preauricular fistulas.
Turner syndrome: Children with Turner syndrome are more likely to have preauricular fistulas.
Trisomy 13: Children with Trisomy 13 are more likely to have preauricular fistulas.

It is important to note that most preauricular fistulas are benign and do not cause any problems. However, in some cases, a preauricular fistula may become infected, leading to pain, swelling, and discharge. In these cases, surgical intervention may be necessary to treat the infection and prevent recurrence

Symptoms

Here is a list of symptoms associated with congenital preauricular fistula:

Presence of a small hole or tract near the ear
Small cyst or nodule near the ear
Swelling or redness near the ear
Discharge from the tract
Pain or discomfort near the ear
Hearing loss
Tinnitus (ringing in the ears)
Dizziness
Nausea
Vomiting
Vertigo
Unsteadiness or balance problems
Facial nerve weakness or paralysis
Facial asymmetry
Inability to close the eyelid completely
Drooling
Speech difficulties
Taste disturbances
Chronic ear infections
Increased risk of developing cholesteatoma (a type of noncancerous cyst in the ear)

Each of these symptoms is discussed in detail below:

Presence of a small hole or tract near the ear: This is the most obvious symptom of congenital preauricular fistula. The hole is usually located near the front or side of the ear and is typically small in size. The tract may be visible as a thin line or canal, and it may or may not be covered by skin.
Small cyst or nodule near the ear: Some individuals with congenital preauricular fistula may develop a small cyst or nodule near the hole. This is caused by the accumulation of fluid within the tract and is not usually painful.
Swelling or redness near the ear: In some cases, the area around the fistula may become swollen or red. This is often a sign of an infection and requires prompt medical attention.
Discharge from the tract: A discharge from the tract may be a sign of an infection and should be evaluated by a healthcare professional. The discharge may be clear, cloudy, or pus-like in appearance.
Pain or discomfort near the ear: Some individuals with congenital preauricular fistula may experience pain or discomfort near the ear. This may be caused by inflammation or infection within the tract.
Hearing loss: Hearing loss is a common symptom of congenital preauricular fistula. This may be due to the fistula being located near the ear canal or to the presence of fluid within the tract. In severe cases, hearing loss may be permanent.
Tinnitus (ringing in the ears): Tinnitus is a ringing, buzzing, or whistling sound in the ears that is not caused by an external source. It is a common symptom of congenital preauricular fistula and may be caused by hearing loss or damage to the ear.
Dizziness: Dizziness is a feeling of unsteadiness or imbalance that may be caused by a congenital preauricular fistula. It may be accompanied by vertigo, which is a spinning sensation.
Nausea: Nausea is a feeling of discomfort or sickness in the stomach that may be caused by a congenital preauricular fistula. It may be accompanied by vomiting.

Diagnosis

The following is a list of diagnostic tests and evaluations that can be used to diagnose a congenital preauricular fistula:

Physical Examination: The first step in diagnosing a congenital preauricular fistula is a physical examination of the ear. During this examination, the doctor will look for any signs of an opening near the ear or a small tract that may indicate a CPF.
Otoscopy: Otoscopy is a diagnostic tool that allows the doctor to examine the ear canal and eardrum. This test can help to identify any signs of a congenital preauricular fistula.
CT scan: A CT scan is an imaging test that uses X-rays and computer technology to produce detailed images of the ear and surrounding structures. This test can be used to diagnose a congenital preauricular fistula by showing the location and size of the fistula.
MRI: An MRI is another imaging test that uses magnetic fields and radio waves to produce detailed images of the ear and surrounding structures. This test can also be used to diagnose a congenital preauricular fistula.
Ultrasound: An ultrasound is a diagnostic test that uses high-frequency sound waves to produce images of the ear and surrounding structures. This test can be used to diagnose a congenital preauricular fistula by showing the location and size of the fistula.
Audiometry: Audiometry is a test that measures a person’s ability to hear different sounds at different frequencies. This test can be used to diagnose a congenital preauricular fistula by determining if there is any hearing loss or other hearing-related issues.
Tympanometry: Tympanometry is a test that measures the movement of the eardrum in response to changes in air pressure. This test can be used to diagnose a congenital preauricular fistula by determining if there is any fluid buildup in the middle ear.
Acoustic Reflectometry: Acoustic Reflectometry is a test that measures the reflectance of sound waves from the ear drum. This test can be used to diagnose a congenital preauricular fistula by determining if there is any fluid buildup in the middle ear.
Otoacoustic Emissions: Otoacoustic Emissions is a test that measures the sounds that are produced by the inner ear in response to a sound stimulus. This test can be used to diagnose a congenital preauricular fistula by determining if there is any hearing loss or other hearing-related issues.
Pure Tone Audiometry: Pure Tone Audiometry is a test that measures a person’s ability to hear different sounds at different frequencies. This test can be used to diagnose a congenital preauricular fistula by determining if there is any hearing loss or other hearing-related issues.
Speech Audiometry: Speech Audiometry is a test that measures a person’s ability to understand spoken words. This test can be used to diagnose a congenital preauricular fistula by determining if there is any hearing loss or other hearing-related issues.
Impedance Audiometry: Impedance Audiometry is a test that measures the movement of the eardrum in response to changes in air pressure. This test can be used to diagnose a congenital preauricular fistula by determining if there is any fluid buildup in the middle ear.

Treatment

Preauricular fistulas are usually harmless and do not cause any significant medical problems. However, some people may choose to treat the fistula for cosmetic reasons or because it causes discomfort or irritation. Here is a list of treatments for preauricular fistulas, along with a detailed explanation of each one.

Observation: In many cases, preauricular fistulas do not cause any problems and do not require any treatment. If the fistula is small and does not cause any discomfort or irritation, it may be best to simply observe it and not treat it.
Cleaning the fistula: If the fistula becomes infected or irritated, it can be cleaned with an antiseptic solution, such as hydrogen peroxide or saline. This can help to prevent infection and reduce inflammation.
Surgical closure: In some cases, surgical closure of the fistula may be recommended. This procedure involves closing the fistula by sewing the edges of the skin together. This can be done under local or general anesthesia, depending on the size and location of the fistula.
Laser therapy: Laser therapy can be used to close the fistula by sealing the edges of the skin together. This is a non-invasive procedure that does not require incisions or stitches.
Dermabrasion: Dermabrasion is a procedure that uses a rotating brush to remove the top layer of skin. This can be used to smooth out the appearance of the fistula and make it less noticeable.
Microdermabrasion: Microdermabrasion is a gentler form of dermabrasion that uses fine particles to remove the top layer of skin. This can be used to improve the appearance of the fistula without causing significant discomfort.
Chemical peel: A chemical peel can be used to remove the top layer of skin and improve the appearance of the fistula. This can be done with various types of chemicals, such as glycolic acid or salicylic acid.
Cryotherapy: Cryotherapy is a procedure that uses cold temperatures to freeze the skin and remove the top layer. This can be used to improve the appearance of the fistula and reduce inflammation.
Electrocautery: Electrocautery is a procedure that uses heat from an electric current to seal the edges of the fistula. This can be used to close the fistula and prevent infection.
Radiofrequency ablation: Radiofrequency ablation is a procedure that uses radiofrequency energy to destroy the tissue in the fistula. This can be used to close the fistula and prevent infection.
Injection of filler: Injection of filler, such as collagen or hyaluronic acid, can be used to fill in the fistula and make it less noticeable.
Fat transfer: Fat transfer is a procedure in which fat is taken from another part of the body and injected into the fistula to fill it in and make it less noticeable.
Radiofrequency ablation: Radiofrequency energy can be used to heat and destroy tissue, allowing the fistula to be closed. This procedure is typically performed under local anesthesia and can be done in an outpatient setting.
Microdebridement: A small instrument can be used to remove infected or damaged tissue from the fistula, allowing it to heal from the inside out.
Cryotherapy: Liquid nitrogen can be used to freeze and destroy tissue, allowing the fistula to be closed. This procedure is typically performed under local anesthesia and can be done in an outpatient setting.
Electrodesiccation: High-frequency electrical energy can be used to destroy tissue and close the fistula. This procedure is typically performed under local anesthesia and can be done in an outpatient setting.
Photodynamic therapy: A special light-activated drug can be used to destroy tissue and close the fistula. This procedure is typically performed under local anesthesia and can be done in an outpatient setting.
Injection of bioactive agents: Bioactive agents, such as growth factors or stem cells, can be injected into the fistula to promote healing and closure.
Surgical marsupialization: The fistula can be surgically opened and the edges of the opening sutured to the surrounding tissue to allow it to heal from

Medications

Antibiotics: If a preauricular fistula becomes infected, antibiotics can be prescribed to help clear the infection. The type of antibiotics used will depend on the specific cause of the infection. If the fistula becomes infected, antibiotics may be prescribed to help clear the infection. Antibiotics can be taken orally or applied topically to the fistula.
Steroids: Steroids, such as cortisone, can be applied topically to the fistula to reduce inflammation and itching.
Pain relief: Over-the-counter pain medications, such as acetaminophen or ibuprofen, can help to relieve any discomfort associated with a preauricular fistula.
Topical creams: Antibiotic ointments or creams can be applied directly to the fistula to help prevent or treat infection.
Warm compresses: Placing a warm compress on the affected area can help to relieve pain and reduce swelling.
Drainage: If there is a build-up of fluid within the fistula, draining the fluid can help to relieve pressure and reduce the risk of infection.
Steroid injections: Corticosteroid injections can be used to reduce swelling and inflammation in and around the fistula.

References