CONGENITAL MALFORMATIONS OF EAR Flashcards

1
Q

Name the congenital malformations of external ear?

A

Preauricular sinus or cyst
Atresia of external auditory meatus
Complete or partial absence of auricle
Accessory auricle
Anomalies in size or shape of pinna

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2
Q

How is preauricular sinus formed?

A

Preauricular sinus forms due to failure of complete fusion between the first and second branchial arch elements in the auricle

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3
Q

Where is preauricular sinus situated?

A

External opening of preauricular sinus is situated between the tragus & crus helix

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4
Q

Symptoms of preauricular sinus or cyst?

A

Usually no symptoms but if it gets infected it produce symptoms like pain,swelling,redness and discharge
After treatment surgical excision is needed

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5
Q

What is atresia?

A

Atresia is characterised by non canalisation of external auditory canal
Often associated with microtia
There is conductive deafness
Absence of external auditory meatus
Treated by reconstructive surgery

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6
Q

How does anomalies of the pinna occurs?

A

Anomalies of the pinna occurs due to failure of development of the six auricular tubercles

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7
Q

What is anotia?

A

Anotia is the complete absence of pinna

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8
Q

How is pinna developed?

A

Pinna is developed from six tubercles from first and second arch,which fuse together

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9
Q

Accessory auricle?

A

Sometimes the arch doesn’t fuse and forms the accessory auricle

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10
Q

What is microtia?

A

Microtia is a small pinna

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11
Q

What is bat ear or Lop ear ?

A

Usually big ear ,outwardly projecting pinna

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12
Q

Congenital abnormalities of middle ear?

A

Treacher Collin’s syndrome
Abnormal ossicles
Congenital fixation of stapes

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13
Q

What do you know about treacher collins syndrome

A

Hereditary malformation of the lower face which is hypoplastic together with varying degree of developmental failure of the external and middle ear

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14
Q

What is the other name of treacher collins syndrome

A

First arch syndrome

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15
Q

What are the ossicles that are most commonly deformed?

A

Incus and malleus

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16
Q

Abnormal ossicles are fused to?

A

May be fused together or sometimes to the bony wall of middle ear cavity
Treated by removal of deformed ossicles followed by tympanoplasty

17
Q

What is congenital fixation of stapes?

A

In this footplate of stapes is congenitally fixed to the oval window or footplate may fuse into the window
Treated by performing stapes mobilization operation

18
Q

Congenital malformations of inner ear

A

Scheibe dysplasia
Mondini dysplasia
Bing siebenmann dysplasia
Michael dysplasia

19
Q

What is scheibe dysplasia?

A

Involves saccule and cochlea
70 % of cases of hereditary deafness

20
Q

Mondini dysplasia?

A

Cochlear duct is reduced to one and a half turns and organ of corti may be absent

21
Q

Bing siebenmann dysplasia?

A

Membranous labyrinth is underdeveloped

22
Q

Michael dysplasia?

A

There is total absence of both labyrinth