Outer and Middle Ear Disorders

Idiopathic disorders

arise spontaneously, unknown cause

Genetic disorders

due to alteration of genotype of individual

• can be syndromic: seemingly unrelated to the constellation of symptoms

  • all syndromes are genetic

Non genetic disorders

not related to the genotype of the individual

Genetic hearing loss with external and middle ear abnormalities will result in…

conductive or mixed hearing loss

Microtia

underdeveloped pinna (graded I-IV)

Stenosis

ear canal narrows to nothing, no tympanic membrane

Anotia

absence of ear canal entirely (grade IV)

Brachio-oto-renal syndrome (BOR)

Autosomal dominant with varied expressivity, chromosome 8

• 90% with HL

• 80% with pre-auricular pits

• 60% branchial cysts or fistulas (skin bulges out)

• 25% kidney abnormalities

CHARGE Association

C: coloboma (clefting of eye)

H: heart defects

A: atresia of choanae (opening of nasal cavity to nasopharyx

R: Regression; delayed growth and development

G: genital hypoplasia

E: Ear abnormalities (pinnae)

Treacher Collins syndrome

Phenotypes:

• Corners of eyes slope down

• depressed cheekbones

• eye clefting

• malformations of ossicles

Apert syndrome

Autosomal dominant or sporadic mutation

Phenotypes:

• developmental delays

• wide set eyes

• flat facial profile

• fused digits

Stickler syndrome

Autosomal dominant

Phenotypes (different types exist)

• midfacial flattening

• long extremities

• loose joints, arthritis in older age

• progressive myopia

Low percentage with SNHL

Vernix caseosa

white film that newborns have on body, can get trapped in ear canal and cause screen failure

Exostoses or osteoma

Non cancerous bony growths

Otitis externa

Inflammation/infection of the outer ear

Otitis media

inflammation of the middle ear

• can be with or without effusion (fluid)

Bullous myringitis

blister develops between layers of the TM often during upper respiratory infection

• rarely results in HL but is painful

Cholesteatoma

growth of skin from external ear that invades middle ear through a retraction pocket in the tympanic membrane

• bacteria is trapped, foul smelling drainage

• aural fullness

• requires surgery

Mastoiditis

Acute: inflammation of mucosa

Chronic: affects bone structure

Tympanosclerosis

Calcified scar tissue on the TM

• related to chronic otitis media

• can cause CHL

Identify the following pathologies:

a. healthy TM

b. acute otitis media

c. chronic serious otitis media

d. cholesteatoma

e. TM perforation

f. myringotomy with PE tube placement