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Common Diseases of the Pinna and External Auditory Canal

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    The ear is morphologically divided into three parts:

    • External ear
    • Middle ear
    • Inner ear

    External Ear

    The external ear consists of the following components:

    • Pinna
    • External Auditory Canal (EAC)
    • Tympanic Membrane (TM)
    Pinna

    The pinna is composed of the following features:

    • Made of a thin yellow elastic cartilage covered with skin.
    • The skin is firmly adherent to the cartilage on the lateral surface and loosely adherent on the medial surface.
    • The cartilage is continuous with the cartilage of the EAC except between the root of helix and tragus, which is filled by fibrous tissue. This gap is called incisura terminalis.
    • The lobule of the pinna is devoid of cartilage.
    External Auditory Canal

    The external auditory canal possesses the following characteristics:

    • Formed by the temporal bone.
    • The EAC is "S"-shaped.
    • The canal is 24mm in length.
    • The outer 1/3rd of the EAC is cartilaginous and 8mm in length.
    • The inner 2/3rd is bony, 16mm in length.

    Haematoma of the Pinna

    Synonyms: Sanguineous tumours of the insane

    • Haematoma of the pinna is a collection of blood under the perichondrium of the pinna.
    • It is usually the result of blunt trauma that tears small blood vessels located between the perichondrium and the auricular cartilage.
    • The blood collects in the subperichondial plane, causing the elevation of the perichondrium.

    Causes

    • Common in boxers, wrestlers, and other combat athletes.
    • Road Traffic Injuries (RTI).
    • Microtrauma: Patients sleeping on a folded pinna position.
    • Bleeding disorders.
    • Clotting disorders.

    Clinical Features

    • Pain.
    • Bruises.
    • Swelling in conchal region.

    Complications if Not Treated

    • Perichondritis.
    • Necrosis of cartilage.
    • Cauliflower ear: Permanent deformity due to the organization of the hematoma (commonly seen in boxers and wrestlers, referred to as "boxer's/wrestler's ear").

    Treatment

    • Aspiration (aseptic technique): Done when the blood collection is small and recent.
    • Incision and drainage: The skin over the swelling is infiltrated with 2% xylocaine and incised. The incision is made parallel to the margin of the helix, and blood and clots are removed with a curette.
    • Pressure dressing: Applied to prevent re-accumulation.
    • Antibiotics.
    • Anti-inflammatory medications.

    Perichondritis

    • Inflammation/infection of the perichondrium and the cartilage of the pinna.
    • Very painful condition.
    • Can lead to necrosis and deformity of the pinna.
    • It may follow injury or hematoma.

    Causative Organisms

    • Staphylococcus aureus
    • Pseudomonas
    • Streptococcus

    Causes

    • Trauma:
      • Mechanical.
      • Thermal: Burns or frostbite.
      • Surgical.
    • Spread from superficial infection: Seborrheic dermatitis, psoriasis, SLE (Systemic Lupus Erythematosus), etc.
    Pathology

    Clinical Features

    • Warm sensation in the pinna.
    • Movement of pinna is painful.
    • Swelling of pinna, forward displacement, and prominence of the pinna.
    • Deformity of pinna in the late stage.
    • Red and puffy-looking pinna.
    • Edema of auricular skin.
    • Marked erythema and loss of normal contour.
    • Tenderness.
    • Late stage: Thickened skin, areas of necrosis and discharging sinuses, deformity.

    Investigation

    • Swab M/C/S (Microscopy/Culture/Sensitivity).
    • E&U + Cr (Electrolytes and Urea + Creatinine).

    Treatment

    • IV antibiotics:
      • Antipseudomonal antibiotics for 2 weeks:
      • Ciprotab, tazocin, ceftazidime, meropenum.
    • Topical antibiotic irrigation:
      • With indwelling catheter.
      • Irrigation with aluminum acetate.
    • I&D with curettage of the cartilage + pressure dressing:
      • Indicated if a subperichondrial abscess is already formed.
    • Reconstruction of pinna deformity - Plastic surgery.

    Complications

    • Septicemia.
    • Subacute bacterial endocarditis.
    • Necrotizing fasciitis of the neck.

    Prevention

    • Prompt drainage of auricular hematoma.
    • Cleaning of pinna laceration.

    Erysipelas

    • It is an acute streptococcal lymphangitis and dermatitis that spreads rapidly.
    • It may follow a scratch.
    • It is due to the spread of β-Hemolytic streptococcus.

    Causes

    • Surgery for otitis media: Infected by β-Hemolytic streptococcus.
    • Contamination by trauma.

    Features

    • Fever, malaise.
    • Slowly advancing, redness, slightly tender, indurated area of skin with a sharp slightly elevated border that is redder than the older central area.

    Treatment

    • Penicillin injection.

    Keloid

    • Are firm, rubbery, shining fibrous nodular lesions that occur at the site of a healed skin injury.
    • Occur as a result of an overgrowth of granulation tissue (collagen type 3) being slowly replaced by collagen type 1.
    • Frequency of occurrence is 15 times higher in pigmented people.
    Ear lobe keloid

    Pathology

    • Histologically, keloids are characterized by a collection of atypical fibroblasts with excessive deposition of extracellular matrix components, especially collagen, fibronectin, elastin, and proteoglycans.
    • Generally, they contain relatively acellular centers and thick, abundant collagen bundles that form nodules in the deep dermal portion of the lesion.

    Treatment

    • Keloids are difficult to treat.
    • Small keloids are best left untouched, and the individual is advised to avoid earrings.
      1. Simple excision - Usually produces recurrence.
      2. Gel sheeting - Hydrogel and silicone scar sheets (to treat pain and itching).
      3. Intralesional triamcinolone injection - Once weekly for a month.
      4. γ-interferon injection - For one week after excision.
      5. Laser excision.
      6. Cryotherapy.

    Sebaceous Cyst

    Clinical Features

    • Occurs as a soft, slightly fluctuant round swelling in the dermis.

    Most Common Site

    • Loose skin behind the lobule of the auricle.
    • Concha.
    • Floor of the external auditory canal.

    Treatment

    • Enucleation: (with walls and content intact).

    Leprosy

    Clinical Features

    • Enlargement of the auricle.
    • Ulceration of auricle.
    • Discharge.

    Histology

    Foamy, lipoid-containing cells with round cell infiltration and the presence of acid-fast bacilli.

    Treatment

    • Dapsone: for 2 months.

    Relapsing Polychondritis

    • Is an autoimmune disease with autoantibodies against a component of cartilage, type II collagen, manifesting as intermittent episodes of inflammation of both articular and non-articular cartilages. It may involve cartilages of the trachea, ribs, larynx, nasal, and auricular cartilages.
    • Common in women.
    • Commonly occurs between 35-45 years of age.

    Treatment

    • Corticosteroids.
    • Analgesics.
    • Cyclosporin.

    Preauricular Abscess

    • Occurs from infection of a preauricular sinus.
    • Preauricular sinus is a congenital skin-lined tract present in front of the ear.
    • Infection can lead to an abscess.

    Treatment

    • Excision of the tract.
    Excision of a preauricular sinus tract
    Pre auricular sinus
    Preauricular abscess

    Cicatricial Stenosis (Acquired Meatal Atresia)

    Types

    • Diffuse.
    • Localized.
    • It usually occurs at the isthmus 5mm lateral to the T.M.

    Causes

    • External Trauma.
    • Mastoid surgery.
    • Keloid of EAC (External Auditory Canal).
    • Burns.
    • Radiation.
    • Neoplasia.

    Symptom

    • Conductive Hearing loss.

    Treatment

    • Surgical removal of fibrous tissue and reconstruction.

    Cerumen (Wax)

    • Cerumen: is a mixture of ceruminous and sebaceous gland secretions mixed with epithelial debris and dust particles in the EAC (External Auditory Canal).
    • It may be dry or wet.
    • Soft or hard.
    • The skin lining the lateral 2/3rd of EAC contains two glands: Ceruminous and sebaceous gland.

    Ceruminous gland: are modified sweat glands that produce white secretion that darkens with time on exposure to air. They open into the root canal of hair follicles.

    Functions

    • It contains a high concentration of lipid protecting the skin of the canal.
    • Also contains:
      • Lysozymes.
      • Immunoglobulins.
      • Antibacterial agents.

    Wax is a natural secretion, but it sometimes accumulates and hardens in the EAC.

    Causes of Accumulation

    • Excessive secretion/production: Hereditary factors, emotional factors, mechanical stimulation.
    • Decreased drainage: Meatal stenosis.
    • Variation in chemical composition.
    • Defective shape of EAC (External Auditory Canal).
    • Dusty occupation.
    • Retention by stiff hair in the canal.
    • Habitual picking of the EAC pushes wax in where it stagnates.

    Presentation

    SYMPTOMS: All otological symptoms.

    • Symptoms:
      • Blocked feeling in the ear.
      • Conductive deafness.
      • Pain.
      • Vertigo, tinnitus.
      • Reflex cough.

    OTOSCOPY: Brownish-black or yellowish mass filling the EAC, tenderness.

    Treatment

    Methods of Wax Removal:

    • Aural Syringing.
    • Wax hook.
    • Curette.
    • Jobson-Horne probe.
    • Ring probe.
    • Suction.
    • Electrically controlled water jet.

    Plan: Wax Solvent 3-4 times daily for 3-5 days; then Syringing.

    Wax Solvents (Softening Agent):

    • Olive oil.
    • Arachis oil.
    • 5% Sodium bicarbonate drops.
    • 3% Hydrogen peroxide.
    • Sodium bicarbonate 5% in glycerine.
    • Soda bicarbonate.
    • Glycerine.
    • Waxolve (paradichlorobenzene/benzocaine/chlorbutol/Turpentine).
    • Plain water (has been used before).

    Indication for Syringing

    • Totally occlusive cerumen.
    • Impacted cerumen associated with pain.
    • Cerumen associated with hearing loss and tinnitus.

    Contraindication to Syringing

    • Perforated TM (Tympanic Membrane).
    • CSF-otorrhoea.
    • Active otitis media.
    • History of recent ear surgery.
    • Only hearing ear.

    For medicolegal reasons: Patient hearing should be re-examined after wax cleaning.

    Complications of Syringing

    • Reactivation of inactive otitis media/externa.
    • Rupture of TM (Tympanic Membrane).
    • Caloric stimulation of the labyrinth leading to vertigo.
    • Scaling of the meatus.

    Keratotic Obturant

    • Occurs due to abnormal desquamation of epithelium in the deep EAC (External Auditory Canal).
    • It is a severe painful condition.
    • Characterized by the presence of desquamated epithelial mass mixed with cerumen deep in the EAC.
    • It gradually causes bone erosion.

    Features

    • The desquamated epithelium causes bony erosion of the canal wall with destruction of surrounding tissue.
    • Otalgia.
    • Hearing impairment.
    • Scanty discharge.
    • Tinnitus.
    • On Examination:
      • White flaky desquamated skin of the canal.
      • Bony erosion with surrounding hyperemia and granulations.
    • The condition may be associated with chronic bronchitis or bronchiectasis.

    Treatment

    • Periodic removal.

    Furunculosis in the EAC

    Furunculosis is a Staphylococcal infection of the hair follicles in the cartilaginous part of the EAC (External Auditory Canal).

    Causes

    • Diabetes.
    • Habit of repeated cleaning of the EAC.
    • Dusty dry environment/climate.
    • Dysphagocytosis.
    • Hypogammaglobulinemia.

    Symptoms

    • Pain in the ear which is severe.
    • Pain on moving the jaw => furunculosis in the anterior wall of the EAC.
    • Pain on moving the pinna => furunculosis in the posterior wall of the EAC.
    • Ear discharge when the furuncle ruptures.
    • Hearing loss when the furuncle occludes the EAC.

    Signs

    • Prominent pinna.
    • Obliteration of the postauricular groove.
    • Tragal tenderness.

    Treatment

    • Cleaning of EAC.
    • Packing the EAC with 10% ichthammol glycerine/cellulose wick.
      • (Magnesium sulfate or 70-95% alcohol can be used).
    • Antibiotics.
    • Analgesics.
    • Ichthammol glycerin pack: This pack absorbs water and decreases tension.

    Foreign Body in the EAC

    Foreign bodies (FB) in the EAC (External Auditory Canal) are common in children and mentally retarded adults.

    • Children generally love to explore body orifices during play.
    • FB could present as an emergency when:
      • The FB is a live insect with unbearable irritation.
      • There's a potential risk of TM (Tympanic Membrane) perforation.
      • There's a button battery with the risk of releasing corrosive chemicals.

    Other Classifications

    • Living: Insects, maggots.
    • Non-living:
      • Hygroscopic: Peas, grains.
      • Non-hygroscopic: Stones, plastic pieces.

    Symptoms

    • Animate FB (Living) may cause obstruction of the EAC, conductive deafness, and damage to the ear.
    • Vegetable FB easily swell and cause inflammation and pain in addition to obstruction.
    • Non-vegetative FB may be present in the EAC for a long time before causing symptoms.

    Clinical Features

    • Otalgia (ear pain).
    • Hearing impairment.
    • Trauma to EAC wall or TM (Tympanic Membrane).
    • Imaging when FB has migrated to the middle ear.

    Signs

    • Otoscopy to visualize FB:
      • Type, location, shape.
    • Check for any associated injury to the canal wall or TM.
    • Examination under GA (General Anesthesia) may be indicated in an uncooperative child.

    Treatment

    • In case of an irritating live insect in the EAR:
      • First, apply oily drops to suffocate the insect and render the situation non-emergency.
    • Removal with an instrument or syringing depending on the nature of the FB.
    • Surgical removal via a post-auricular approach when the FB is impacted beyond the isthmus or has migrated into the middle ear.
    • Removal may be done under GA (General Anesthesia) for an uncooperative child.
    • Insect should be killed by suffocating/drowning.
    • Instill either:
      • Spirit.
      • Alcohol.
      • Salt water.
      • Oil into the EAC and remove the FB.

    Otitis Externa

    Otitis externa is the inflammation of the skin lining the EAC (External Auditory Canal).

    Classification

    • Acute or Chronic.
    • Localized/Diffuse.
    • Infective.
      • Bacterial:
        • Localized (furuncle).
        • Generalized (infiltrative/desquamative).
      • Erysipelas.
      • Fungal (otomycosis).
      • Viral:
        • Herpes simplex.
        • Herpes zoster.
        • Bullous myringitis.
    • Reactive:
      • Eczematous.
      • Seborrhoid dermatitis.
      • Atopic dermatitis.
      • Psoriasis.
      • Lupus erythematosus.
      • Benign otitis externa.
      • Malignant otitis externa.

    Aetiology

    • Age: All ages.
    • Water entering the ear (contaminated).
    • Damp climate.
    • Scratching.
    • Discharge from the middle ear (CSOM - Chronic Suppurative Otitis Media).
    • Diabetes (Malignant Otitis externa).

    Pathology

    Localised:

    • A furuncle is a staphylococcal infection affecting a hair follicle or a sebaceous gland.

    Generalised otitis externa:

    • Staphylococcal, streptococcal, or gram-negative bacillary infection.

    Symptoms

    • Pain: More pronounced at night due to recumbent posture.
    • Otorrhoea (ear discharge).
    • Hearing impairment.
    • Tinnitus (ringing in the ears).
    • Itching.
    • Trismus (difficulty in opening the mouth).

    Signs

    • Swelling: Localised or diffuse.
    • Discharge.
    • Tenderness.
    • Trismus (difficulty in opening the mouth).
    • Granulation.

    Differential Diagnosis

    • Acute otitis media.
    • Acute mastoiditis.
    • Wax (cerumen).
    • Keratosis obturans.
    • Otomycosis.
    • Referred otalgia.

    Treatment

    Local:

    • Ototopical drug application with wick dressing:
      • 10% ichthammol in glycerin.
      • Flavin in spirit.
      • Otomed ear drop (antibiotic ear drop).
      • Aural toilet.
      • Incision and drainage for furuncle (do not cut deep to cartilage).

    General:

    • Oral antibiotics.
    • Analgesic.
    • Treat underlying cause, e.g., Diabetes.

    Wick Dressing:

    Packing the ear with a gauze strip soaked in drug.

    • It is very soothing.
    • The wick becomes wet by capillary action.
    • Effect of drug persists longer.

    Prevention

    • Keep the ear dry.
    • Avoid scratching.

    Otomycosis

    It is an OE (Otitis Externa) caused by fungus.

    • Common in the rainy season.
    • Common in the tropics.
    • Causative organisms: Aspergillus niger, Monilial, Candida.
    • Secondary bacterial infection may follow.
    • Treatment should be for a long time, typically more than 2 weeks, as fungus tends to invade deep layers of the skin.
    Otomycosis

    Aetiology of Otomycosis

    • Moisture.
    • Swimming.
    • Prolonged use of Antibiotic ear drops.
    • Inoculation by dirty habits.

    Symptoms:

    • Itching.
    • Pain.
    • Impaired hearing.
    • Tinnitus.
    • Trismus (difficulty in opening the mouth).

    Signs:

    • Cotton-like growth.
    • Black specks.
    • Wet newspaper-like debris.
    • Other signs of otitis externa.

    Treatment of Otomycosis

    Local:

    • Stop any antibiotic ear drop.
    • Ototopical treatment:
      • Antifungal ear drop/wick dressing
      • E.g., Flucamed, 1% Clotrimazole.
      • 1% Gentian Violet.
      • Locacortin Vioform.
      • Nystatin.
      • Otomed.

    Systemic:

    • Antibiotic.
    • Antipruritic.
    • Analgesic.

    Herpes Simplex

    Similar to lesions occurring elsewhere.

    Zoster

    Herpetic vesicles occurring in the EAC. May be associated with facial nerve paralysis (Ramsay-Hunt syndrome).

    Herpes Simplex
    Herpes Simplex

    Bullous Myringitis

    It is a viral infection characterized by hemorrhagic vesicles on the external surface of the tympanic membrane.

    Clinical Features:

    • Blood-stained otorrhea (ear discharge).
    • Severe pain.
    • Conductive hearing loss.

    Treatment: As in otitis externa. Mainly symptomatic. No attempt should be made at puncturing the vesicles.

    Bullous Myringitis
    Bullous Myringitis

    Eczematous Otitis Externa

    It is allergic dermatitis of the EAC.

    Clinical Features:

    • Irritation.
    • Oedema.
    • Weeping eczema.
    • Fissuring and scaling.
    • Secondary infection.
    • Stenosis/fibrosis.

    Treatment:

    • Ototopical steroid.
    • Antihistamine.
    • Antibiotic (local & systemic).
    • 10% silver nitrate for fissuring.
    • Dilation of stenosis/plastic surgery.

    Seborrhoeic Otitis Externa

    A form of seborrhoeic dermatitis.

    Clinical Features:
    • Itching.
    • Secondary infection.

    Treatment:

    • Aural toileting.
    • Shampoo wash.

    Myringitis

    Infection of the external surface of the TM.

    Treatment is as OE.

    Traumatic Otitis Externa

    Malignant Otitis Externa

    It is a fulminating severe form of OE.

    Seen in elderly, immuno-compromised, diabetic patients.

    Very painful.

    The condition behaves like a malignant process and causes destruction of the canal, preauricular and postauricular tissue, and may involve and cause palsy of cranial nerves (CN VII, IX, X, XI).

    Causative agent: Pseudomonas aeruginosa.

    Treatment: antibiotics, debridement, treat predisposing factors.

    Aural Myiasis

    Aural myiasis refers to the presence of maggots in the external auditory canal (EAC).

    Predisposing factors for aural myiasis include poor hygiene, chronic suppurative otitis media (CSOM), and a low standard of living.

    Treatment involves the removal of the maggots.

    Exostoses (Surfer's Ear)

    Exostoses, also known as surfer's ear, are benign broad-based bony overgrowths in the bony external auditory canal (EAC).

    These growths are common in swimmers, particularly in the young age group and males. They often occur in individuals with a history of repeated exposure to cold water.

    Exostoses are commonly seen in areas where the tympanomastoid and tympanosquamous suture lines meet in the ear canal.

    Patients with exostoses are typically asymptomatic.

    Exostoses

    EAC Osteoma

    EAC Osteoma is a benign pedunculated bony overgrowth that can occur in the bony external auditory canal (EAC).

    It is typically a unilateral condition and may present with various symptoms such as:

    • Asymptomatic growth
    • Itching or irritation in the ear
    • Conductive hearing loss

    Diagnosis often involves a CT scan to assess the extent of the osteoma.

    Treatment depends on the symptoms. If the osteoma is asymptomatic, no treatment may be necessary. However, if it causes discomfort or hearing loss, surgical removal of the osteoma may be recommended.

    Neoplasms in the External Auditory Canal (EAC)

    Neoplasms in the EAC are relatively rare but can include various types:

    • Multiple exostoses: These are benign bony growths in the EAC.
    • Rodent ulcer: Referring to a basal cell carcinoma, a type of skin cancer.
    • Squamous cell carcinoma: Another type of skin cancer that can affect the EAC.

    Treatment options for neoplasms in the EAC may include:

    • Surgery: Depending on the type and extent of the neoplasm, surgical removal may be considered.
    • Chemotherapy: In some cases, chemotherapy may be used as part of the treatment plan.
    • Radiotherapy: Radiation therapy may also be employed for certain types of neoplasms.

    Organogenesis occurs – 3rd – 8th week gestation

    Otic placode appears 24-25 days

    External auricle appears 5-6 weeks

    Sound perception 24-26 weeks

    Anomalies of the pinna

    The pinna arises from a series of 6 tubercles which develop on the 1st and 2nd branchial arches around the primitive meatus at about the 6th week IUL.

    The development is complete by the 4th IUL.

    The arrest of development may result in various deformities

    Size of pinna

    • Anotia: absence of the pinna.
    • Microtia: deformed or abnormally small pinna.
    • Macrotia: abnormally large pinna.

    Position of pinna

    • Synotia: auricle are joined beneath the mandible. (ear below the level of the maxilla)
    • Malotia: ear placed downwards and forwards ventrocaudal displacement
    Anotia
    Microtia

    Other Auricular Anomalies

    • Preauricular tag and accessory auricles: These are small masses seen anterior to the tragus.
    • When it consists of only skin and fat, it is called a skin tag.
    • When it contains cartilage also, it is termed accessory auricle.
    • Polyotia: More than two well-formed auricles.
    • Telephone ear deformity: Excessive correction of the middle third segment of the ear in otoplasty.

    Accessory Auricle

    Accessory auricle is a small elevation of skin, often containing cartilage, located just in front of the tragus or the helix. It is usually asymptomatic.

    Treatment: Surgical excision can be considered if the patient desires it.

    Preauricular Sinus

    Preauricular sinus develops from the 1st and 2nd branchial arches due to improper fusion of the auricular tubercules. It is a blind tract lined by squamous epithelium and occurs in the region of the auricle, usually near the tragus and roof of the helix.

    Usually bilateral, preauricular sinuses are present at birth. There may be some sebaceous discharge from the punctum. The sinus may get infected repeatedly and may form an abscess. Treatment is usually not necessary and may be left untreated. However, excision of the tract may be considered if it becomes infected.

    Collaural Fistulae

    Collaural fistulae are a type of first branchial cleft anomaly. They are characterized by a skin tract that extends between an opening in the floor of the external auditory canal (EAC) and an opening located behind the angle of the jaw, anterior to the sternocleidomastoid muscle. In some cases, the tract may pass deep to the facial nerve.

    Symptom: Patients may experience discharge from an opening found in the neck.

    Sign: An external fistulous opening in the neck is typically observed along a line from the tragus to the hyoid bone.

    Investigation: Radiopaque sinugram imaging may be performed to confirm the diagnosis.

    Treatment: The typical treatment for collaural fistulae involves surgical excision.

    Hypertrichosis

    Hypertrichosis is characterized by excessive hairiness of the ears and is typically observed in men. This trait is inherited in a Y-linked manner. The usual site of excessive hair growth is along the margin of the helix. Hypertrichosis is particularly common in individuals of Indian descent.

    Harbula hirci

    Harbula hirci refers to the excessive growth of hair on the tragus, which is a secondary sex characteristic. The term "tragus" is derived from the Greek word "tragos," meaning "goat's beard."

    Darwin’s Tubercle

    Darwin’s Tubercle is a small elevation or cartilaginous protuberance in the posterior superior part of the Helix. It is most commonly located along the concave edge of the posterosuperior margin of the helix and projects anteriorly. This feature is homologous to the tip of the ear in mammals.

    Darwin's Tubercle is an inherited condition caused by an autosomal dominant gene that exhibits variable expressivity. Notably, this atavistic remnant represents the apex of the anthropoid ear, suggesting a common ancestry between humanity and apes, which is why it is referred to as "Darwin's" tubercle.

    Variations

    In some individuals, the antihelix is more prominent than the helix. The lobule may also be absent or adherent to the side of the head.

    Wildermuth’s Ear is characterized by an abnormal protrusion of the pinna with the absence of the antihelix.

    Lop Ear is a more severe variant of bat ear.

    Outstanding Ears

    Outstanding ears are the most common cosmetic deformity of the pinna. They are inherited by an autosomal dominant gene with complete penetrance but variable expressivity.

    Treatment: Although it may be asymptomatic, surgery may be indicated if there is

    • Major deformity
    • Intense emotional discomfort
    • Cosmetic disfigurement
    (posterior view- Bat Ear).

    Microtia

    Microtia is characterized by gross hypoplasia of the pinna with a blind or absent external auditory canal. It ranges from having no recognizable features on the side of the head to an incompletely formed auricular appendage. Microtia is typically bilateral, although the degree of the deformity may be different on the two sides.

    Children born with microtia should have their hearing tested soon after birth. If hearing loss is present, they should be fitted with a hearing aid as quickly as possible.

    External Auditory Canal

    The EAC develops from the 1st branchial cleft lying between the 1st and 2nd branchial arches. The ventral arch cartilage forms the mandible (1st arch) and the hyoid bone (2nd arch).

    Anomalies of structures associated with these arches give rise to multiple deformities, such as Treacher-Collins syndrome and Pierre-Robin syndrome.

    Classification

    • Minor Aplasia: The external auditory meatus is narrow, the TM is functional, the pinna is either normal or with minor deformity. There may be ossicular fixation.
    • Major Aplasia: There is usually microtia with EAC atresia and fixation of the malleus and incus. Stapes is usually normal.
    • Atresia: The EAC is atretic, the tympanic cavity is small, and the mastoid bone is not pneumatized. These are usually associated with abnormalities of the middle ear.

    Features

    • Complete Atresia: The external auditory canal is completely closed or absent.
    • Shallow Depression: There may be a shallow depression or absence of the normal canal.
    • Changes in the Curvature of the Canal: The curvature of the canal may be altered in cases of atresia.
    No meatal opening

    Congenital Syndromes Associated with Microtia and Pinna Deformities

    • Tricher-Cholin's Syndrome: This syndrome is characterized by microtia and may include various facial and cranial abnormalities.
    • Otomandibular Syndrome of Konigmark and Gorlin: This syndrome involves ear and jaw abnormalities, including microtia.
    • Branchio-Otic Dysplasia: A syndrome characterized by ear and branchial arch abnormalities.
    • Lacrimoauricular Dentodigital Syndrome (LAADD): A rare syndrome involving ear, lacrimal duct, dental, and digital abnormalities.
    • Down Syndrome: Associated ear abnormalities may include a small pinna, poorly developed lobe, EAC atresia, and ossicular chain defects.

    Potter's Syndrome

    Potter's syndrome is characterized by:

    • Low-Set Pinna: The ears are positioned lower than normal on the head.
    • Renal Agenesis: This syndrome is often associated with kidney abnormalities, including renal agenesis.

    Congenital Tumors of the External Ear

    • Lymphangioma: Lymphangiomas consist of a number of intercommunicating spaces containing lymph. Treatment typically involves excision with diathermy.
    • Dermoid Cyst:
      • These cysts develop where two ectodermal areas fuse during development.
      • Sequestration of a portion of the ectoderm may occur, leading to the formation of a cystic tumor.
      • The cyst contains epithelial debris and hair.
      • It is located deep to the deep fascia.
      • Common site: near the anterior border of the ascending limb of the helix.
      • Treatment usually involves excision.
    • Haemangioma: Hemangiomas are the most common tumors of childhood and consist of a mass of dilated capillaries. They occur in three forms:
      1. Capillary Hemangioma
      2. Compact Hemangioma
      3. Cavernous Hemangioma
    Capillary (straw berry) haemangioma

    Cavernous Hemangioma

    • Consists of a number of intercommunicating blood spaces.
    • Has a dark red color that tends to disappear on pressure with a glass slide or intensify with crying.
    • Surface is irregular and lobulated.
    • Typically increases in size in the first year of life.
    • Shows a tendency to regress and disappear after the fifth year.

    Predisposing Factors

    • Drugs: Thalidomide, phenytoin, folic acid antagonist
    • Radiation
    • Viruses

    Principle of Management

    1. History
    2. Physical examination
    3. ENT examination
    4. Imaging
      • MRI
      • CT-Scan

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