Congenital Abnormalities of the Ear - Types, Diagnosis, and Treatment

Introduction:

Congenital abnormalities of ears are the defects in the shape, size, position, and function associated with the ear present since birth. Our ear is divided into three parts that are outer, middle, and inner ear. Different types of deformities are associated with various parts of the ear. Congenital abnormalities of the ears can affect one or both ears, resulting in structural and functional defects of the ear. Read the article to learn more about different types of congenital abnormalities of the ears, its diagnosis, and management.

What Is the Prevalence of Congenital Abnormalities of the Ears?

The prevalence of congenital (birth) abnormalities associated with the ears is around five percent of the total population.

What Is the Incidence of Congenital Abnormalities of the Ears?

• The total incidence of congenital ear deformities is around one in 3800 births.

• The total incidence of congenital ear deformities associated with the external ear is around one in 1000 births.

• Congenital ear deformities can affect one or both ears and are more common in the right ear.

What Are the Congenital Deformities Associated With the Ears?

Different congenital deformities associated with the ears are:

Microtia or Anotia - It is the congenital deformities affecting the external ear resulting in underdeveloped, abnormally shaped ears (microtia) and complete absence of the ears (anotia).

Microtia Is Classified Into Four Types:

Prominent Ears: It is of concern to the patient in terms of physical appearance (cosmetic). Some factors like wearing eyeglasses are the problems that need to be resolved by treating prominent ears.

Cupped Ears: Tightened or constricted ear rims are called cupped or lop ears. It can vary from mild to severe cases based on the extent of the involvement. Hearing dysfunction is seen in the case of inner ear involvement. Cupped ear deformities are classified as:

1. Type 1: It is characterized by minor deformity affecting the helix of the ears only.

2. Type 2: It is characterized by moderate deformity affecting the ear's helix, antihelix, and crura (anterior part of antihelix).

3. Type 3: It is characterized by severe deformities of the pinna resulting in reduced height and width of the ears.

Preauricular Cyst: Preauricular cysts are congenital deformities in front of the ears on the facial skin. It is present as a hole or pit in front of the ear. Pus drainage is seen in the case of an infected cyst. Pain, swelling in the front of the ears, and pus discharge are the other symptoms presented by the preauricular cyst. Incision and drainage, and fistulography with surgical dissection are the treatment of choice. Antibiotic coverage after the surgery is recommended by the doctor.

External Ear Malformations: Pinna (projecting part of the external ear) malformation varies from mild to severe cases. Pinna malformation is classified as:

1. Grade One Dysplasia: In this, most parts of the pinna (projecting part of the external ear) can be recognized. It is characterized by slight deformities of the external ear. Reconstruction and repair are required occasionally.

2. Grade Two Dysplasia: Only a few parts of the pinna are recognizable. Reconstruction and repair of the pinna are partially required using skin or cartilage.

3. Grade Three Dysplasia: It is associated with a severe malformation of the pinna. None of the structures of the pinna is visible. Total reconstruction and repair of the pinna are needed in these cases.

External Ear Canal Malformation: Narrowing of the external auditory canal (EAC) is seen in newborns. External auditory canals stenosis (narrowing) is classified into three types:

1. External Auditory Canal (EAC) Stenosis Type A: Narrowing of the external ear canal along with an intact skin layer.

2. External Auditory Canal (EAC) Stenosis Type B: Narrowing of the external ear canal with bony canal atresia at the medial part.

3. External Auditory Canal (EAC) Stenosis Type C: It is characterized by complete atresia (closing) of the ear canal. Healing loss and middle ear deformities are present, which need immediate treatment.

How Can We Diagnose Congenital Abnormalities of the Ears?

Different techniques to diagnose congenital abnormalities of the ears are:

• History and Physical Examination: Complete history of the patients for any family history with genetic ear deformities is recorded. A physical examination of the ears is done to rule out structural abnormalities (shape, size, and position).

• Prenatal Screening: Prenatal ultrasonography is recommended to rule out structural defects of the ears, like the partial or complete absence of the external ear.

• Magnetic Resonance Imaging (MRI) Scans: These scans help to detect any defects present in the middle or inner ears, like a preauricular cyst or external auditory canal stenosis (narrowing of the canal). It also helps to detect abnormalities in the ears' shape and size.

• Auditory Screening: Auditory or hearing assessment is recommended for all newborn children to rule out hearing loss deformities in the early stages. It is done using an auditory brainstem response (ABR) test. In this procedure, three small sensors are placed on the baby's head and neck. The soft earpiece is placed in the ear, and soft sounds are played to note the response.

How Can We Treat Congenital Ear Deformities?

Various treatment techniques are:

• Reconstruction of Ears: Reconstruction surgery for ears is performed before four years of age. Medpor (high-density polyethylene implant) and cartilaginous framework are used for the reconstruction of ears for achieving normal contours of the ears. It is done to treat underdeveloped or abnormally shaped ears.

• Cosmetic Surgery: It is performed for the patient concerned about the prominent ears. It is done at the age of five to six years after the ear has developed to its full size.

• Hearing Loss Treatment: Bone-anchored hearing devices or headbands are given to older children for clear and sound hearing.

• Speech and Language Development: After the needful treatment is completed, patients are referred to a speech-language pathologist to guide the children in developing social communication and language skills.

Conclusion:

Congenital abnormalities of the ears affect the patients' and their parents' quality of life. It has a significant impact on both the normal structure and function of the ears. Therefore, auditory screening and physical examination of the ears for all newborns are recommended for prompt diagnosis and management of congenital abnormalities of the ears.