Congenital malformation of the ear
Release time : 03/21/2025 09:30:02
After a hard-won pregnancy of ten months, expectant mothers and fathers surely hope to see a lively and healthy baby. However, congenital malformations have always been a concern for people.
Congenital deformities of the ear are a common condition. What exactly is a congenital deformity of the ear? What are the symptoms? What causes it, and how is it treated? Below, we will delve into these questions through an exhaustive explanation by MommyNet. The ear is one of the most direct organs for receiving external stimuli and information from the world around us. A problem with the ear can be incredibly distressing. Congenital deformities of the ear refer to abnormalities in the shape or structure of the ear that appear during the embryonic development process at birth or shortly thereafter. The symptoms of congenital deformities of the ear vary depending on the type and severity of the deformity. Some common symptoms include: 1. Hearing issues: Due to structural abnormalities in the ear, hearing may decline or completely go mute. 2. Eardrum stenosis or atresia: This can lead to buildup of earwax, infections, and even hearing loss. 3. Outer ear deformities: Conditions such as curvature or deformation of the outer ear can affect normal hearing. 4. Middle ear problems: Issues like perforation of the drum membrane, otitis media, etc., can lead to hearing loss and ear pain. 5. Inner ear issues: Conditions like labyrinthine effusion, vestibular dysfunction, etc., can affect balance and coordination. The etiology of congenital deformities of the ear is multifaceted, including genetic factors, environmental influences, and gene mutations during embryonic development. The specific cause may vary from individual to individual. The treatment of congenital deformities of the ear depends on the type and severity of the deformity. For some minor deformities, observation and regular check-ups can monitor their progression. In cases requiring treatment, interventions could include surgical correction, pharmacotherapy, or other medical interventions. In certain instances, hearing aids or other assistive devices might be needed to improve hearing and quality of life. In summary, congenital deformities of the ear are a common condition, but through timely diagnosis and treatment, symptoms can be effectively alleviated and patients' quality of life can be improved. If you or your child has concerns about congenital deformities of the ear, consulting a professional doctor for personalized treatment advice is recommended.
What exactly is a congenital deformity of the ear? In medical terminology, congenital microtia or congenital external and middle ear deformities are characterized by severe underdevelopment of the auricle, external auditory canal atresia or stenosis, and middle ear deformities, while the inner ear development is mostly normal. There is a certain degree of hearing through bone conduction.
Treatment requires full tympanoplasty and auditory function reconstruction surgery.
Common congenital anomalies of the ear are categorized into two types: 1. Congenital preauricular fistula.
The commonest type of congenital deformity is called the 'smiling' ear, which results from incomplete fusion of the first pharyngeal pouch during embryonic development.
The slit-like opening is usually located anteriorly and superiorly on the auricle, with the fistula branching. The lumen contains shed epithelial cells, and when pressed, produces a white, foul-smelling secretion.
In the case of purulent infection, an abscess may form that requires incision and drainage.
Recurrent suppuration with the potential for abscess formation or scarring, necessitating excision of fistulas and their branches following recovery.
If the condition is not caused by a medication, there is generally no need for treatment.
2. Congenital microtia and atresia of the external auditory canal.
Both often occur simultaneously, with the former being the first and second pharyngeal arches, and the latter resulting from an abnormal development of the first branchial groove.
It may also be accompanied by middle ear or even inner ear malformations.
Generally, such deformities can be classified into three categories: ① The shape of the auricle is still recognizable, and the part of the ossicular chain that is obstructed by atresia has not affected the middle ear.
The second type presents with a crescent-shaped cartilaginous protuberance of the auricle, a blocked external auditory canal accompanied by middle ear deformities, and sensorineural hearing loss. This type is the most common.
The auricle is missing, with only irregular protrusions remaining; the tympanic cavity is obstructed, accompanied by middle ear deformities, and involves the inner ear. The hearing is mixed deafness.
This type is relatively rare in clinical practice.
Symptoms of congenital deformities in the ear, which can be diagnosed at a glance, may include congenital malformations of the middle and inner ears. To ascertain the nature of hearing loss, it is necessary to conduct a hearing examination. CT scans can provide information on the condition of the external auditory canal and middle ear.
What are the symptoms of congenital deformities of the ear? The clinical characteristics of congenital microtia patients mainly involve the auricle, external auditory canal, and middle ear, while the inner ear is often unaffected.
In clinical practice, the most commonly used classification system is trichotomy: - **Grade I**: The size and shape of the auricle are altered, but important surface markers remain. There is narrowing of the external auditory canal, and in severe cases, it may become completely closed. - **Grade II**: This is the most typical presentation, characterized by vertically oriented earlobes resembling a sausage, with an external auditory canal that is obstructed. - **Grade III**: Only skin and cartilage remnants are present, and in severe cases, there may be a complete absence of the ear.
In patients with congenital microtia, there are over ten types of middle ear development disorders, mainly involving the deformities of the ossicles, tympanic membrane muscles, and facial nerves, which are closely related to the severity of external ear deformities.
In severe cases of congenital malformations of the external ear, they may present as one of the clinical manifestations of sequence syndrome, such as the ocular-ear-spinal sequence syndrome.
Patients with achondroplasia besides the presence of microtia also suffer from craniosynostosis (closure of the cranial sutures), skeletal dysplasia (dysmorphic ossification of the temporal, maxillary or mandibular bones), soft tissue deformities (preauricular tags or macroglossia), ocular defects (defects in the eyelid or conjunctival pouch), spinal anomalies, and congenital renal and cardiac malformations.
Congenital deformities of the ear undoubtedly constitute a regrettable aspect of life, and what causes them? I. Embryology The auricle originates from the first branchial arch (hyoid arch) and the second branchial arch (thyroid arch).
At the 5th week of embryonic life, a part of the cartilaginous arch will develop into the external ear. The ear is essentially formed between the 5th and 9th weeks of gestation.
Six concave prominences fuse to form a prominent auricle; the primary branchial cleft inwardly recesses to form the external auditory canal.
During the development phase of the auricle, embryos are susceptible to genetic or external factors that can lead to various congenital abnormalities in the ear.
II. Genetics Congenital aural atresia can occur independently or as part of a syndrome, with common combinations including: Treacher Collins syndrome, Goldenhar syndrome, Nager syndrome, Miller syndrome.
The loci of the susceptible gene for congenital aural atresia and its discrimination are currently hotspots and difficult points in genetic research, but so far no results have been obtained.
III. Epidemiology: The incidence of congenital aural atresia varies across different studies, and is related to ethnicity and geographic location.
The incidence rate in our country is 5.18 per 10,000.
The male-to-female ratio is 2:1, and ectopic uteri are more common in the right side, with bilateral anomalies accounting for about 10%.
The etiology of microtia is still unclear, generally believed to be the result of a combination of environmental and genetic factors. Environmental factors include exposure to viruses during early pregnancy (such as colds), excessive pregnancy-induced nausea, and toxic substances from home renovations, which may contribute to the development of microtia. Patients with a family history of microtia have an approximate inheritance rate of 2.9% to 33.8%.
Treatment of Congenital Deformities of the EAR: Living in this world, observing all directions, listening to everything, it's all very normal.
However, some individuals are born without such a capability that is so commonplace and natural to the majority; it is undoubtedly a cruel thing.
The treatment for congenital aural deformity mainly includes two aspects: one is the reconstruction of external auditory meatus, and the other is the reconstructive therapy of hearing.
Generally, the external auditory meatus is reconstructed before the hearing function is restored.
Hearing rehabilitation surgery often involves the destruction of the skin behind the ear, so it is necessary to perform the surgery after the reconstruction of the auricle.
I. Otoplasty of the Auricle 1. Operation Timing: The timing of auricular reconstruction surgery is critical, as it is one of the key determinants for achieving ideal surgical outcomes.
We believe that the age of 9, 10, and 11 years is optimal for ear reconstruction, taking into account the combined development of cartilaginous trachea, auricles, and psychological growth.
2. Reconstructive Methods: Ear reconstruction usually requires 2 to 3 surgeries.
There are two specific methods.
A method known as the Brent method, named after a U.S. doctor, was later significantly improved by Dr. Nagata, Yoshio, in Japan. This method is used worldwide most extensively and universally.
This method does not require skin expansion.
Another method is skin expansion, which typically requires three surgical procedures.
The first surgery involved the placement of a hydrostatic balloon (approximately 50-80 ml) in the postauricular area of the remaining ear, which was done through skin soft tissue expansion, with a hospital stay lasting approximately four days.
Starting 7 days after the surgery, a saline solution is administered once every other day, filling 50-80ml of saline solution over about 1 month. After completing the saline injection, rest for one month before returning to the hospital for the second surgery.
II. For patients with bilateral microtia and atresia of the external auditory canal, external meatoplasty surgery to enhance hearing may be considered.
However, for patients with unilateral microtia, my experience has been that partial tympanoplasty should be performed, with the depth being around 1cm, not entering the middle ear cavity. The local skin flap should be inverted towards the inside to cover it.
The medical portions of this text are for reference only.
Should there be any discomfort, it is advised to seek medical attention immediately. For accurate diagnosis and treatment, please consult with a licensed practitioner offline.