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Disorders > Exam 1 > Flashcards

Exam 1 Flashcards

1
Q

What are variants of the pinna?

A

Size, position, width, length and angle

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2
Q

Darwin Tubercle/Notch

A

One normal variant of the external ear
Atavistic remnant; usually located in the posterosuperior helix as a cartilaginous protuberance usually on concave edge but may be convex
Autosomal dominant
Variable expressivity

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3
Q

Four mild malformation of the pinna

A

Lop ear, Stahls ear, prominent ear and cup ear

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4
Q

Syndromes associated with outer ear malformations

A

Down syndrome, other trisomies, Treacher Collins, BOR, chromosomal deletions, CHARGE, Goldenhar etc.

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5
Q

Stenosis

A

Narrowing of ear canal, may or may not have hearing loss
Common in children with down syndrome
Can be accompanied with atresia
Treatment- stint in cartilage portion

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6
Q

Anotia

A

No external ear and the most severe pinna malformation
No ear, no pinna, no canal
Unilateral is more common

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7
Q

Atresia

A

No ear canal
Birth defect
Usually accompanied by abnormalities of middle ear bones and external ear
Three forms
Can be genetic- may be a family Hx
Microtia (small ear) may also be associated
Can have max. conductive component (60 dB)
CNT tymps
Speech and language will develop normally if other ear is okay
Early amp is important- BAHA
Can have surgery to restore hearing but it is very complicated and can put the facial nerve at risk
Refer (if they have not been already)

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8
Q

Grade I atresia

A

Most mild form. Characterized be small external canal with most features being present, canal often looks stenotic, the tympanic bone and membrane are often developed but ossicular abnormalities are common

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9
Q

Grade II atresia

A

Moderate and most common form
Characterized by the pinna being present but being formed abnormally, a significant defect in canalization of the tympanic bone, the membranous external canal will be absent or ends in pinpoint fistula opening, a narrow bony canal, deformed ossicles and fused incus and malleus

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10
Q

Grade III atresia

A

Most severe form
Characterized by severely deformed or absent pinna otherwise called “peanut ear”, ossicles are typically an amorphous mass, undefined stapes
This form is often associated with a syndrome
Complete atresia

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11
Q

Microtia

A

External ear malformations

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12
Q

How many grades of microtia are there?

A

Three, with one being the mildest and three being most severe

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13
Q

What is more common, unilateral or bilateral atresia?

A

Unilateral

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14
Q

When does the six hillocks of his develop?

A

Week 5

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15
Q

What are the six hillocks of his?

A

Tragus, crus of helix, ascending helix, upper helix/scapha/antihelix, middle scapha/antihelix/helix, antitragus/lobule

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16
Q

Pinna development week 7

A

Moves dorsolaterally

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17
Q

Pinna development week 18

A

Separates from head

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18
Q

Pinna development week 20-22

A

Approaches mature shape

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19
Q

When is the pinna fully developed

A

Age 9

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20
Q

Preauricular tags and pits

A

Higher rates in individuals with hearing loss so it is important to look when doing an examination

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21
Q

Preauricular sinus

A

Abnormality of the otic hillocks, infected

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22
Q

Development of ossicles and middle ear week 4

A

First pharyngeal pouch endoderm extends outward to form the tubotympanic recess and approaches the 1st cleft ectoderm

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23
Q

Development of ossicles and middle ear week 5-6

A

Neural crest mesenchyme concentrates (ossicles) between cleft and pouch; cleft widens (future external auditory meatus)

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24
Q

Development of ossicles and middle ear week 15

A

Malleus and incus are adult size and shape

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25
Q

Development of ossicles and middle ear week 20

A

Tympanic cavity grows to enclose ossicles but still full of mesenchyme

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26
Q

Development of ossicles and middle ear week 26-30

A

Ossicles ossified

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27
Q

Development of ossicles and middle ear week 32

A

Stapes adult size and ossified

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28
Q

Development of ossicles and middle ear week 33

A

Early pneumatization (development process of the mastoid air cells)

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29
Q

Canalization

A

How to canal opens up, can halt at any time

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30
Q

Canalization week 7-8

A

Tympanic ring forms

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31
Q

Canalization end of month 2

A

External canal is a thick epithelial plug, migrated inward toward ossicles

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32
Q

Canalization week 21

A

Plug starts to recanalize (hollow) via apoptosis, forming the external canal

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33
Q

In what direction does canalization move?

A

Starts medially then moves laterally

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34
Q

What happens if canalization halts before it starts?

A

Total atresia but can have normal middle ear

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35
Q

What happens if canalization halts during development?

A

Patency of ear canal depends on when it is halted

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36
Q

Development of tympanic membrane at 5 weeks

A

Tubotympanic recess contacts ectoderm (which will form the outer layer)

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37
Q

Development of tympanic membrane at 10 weeks

A

Bony ring in place but is incomplete

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38
Q

Development of tympanic membrane at age 3

A

Changes in the face of it

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38
Q

Disorders of the external ear and ear canal that are not congenital (6)

A

Cerumen impaction/foriegn bodies/collapsing canals
External otitis
Tumors of ear canal
Abnormalities/lesions of the pinna
Results of traums
TM abnormalities: myringitis, tympanosclerosis, perfs

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38
Q

What are three ossicular abnormalities (one for each)?

A

Malleus head fixation (common)
Incus- no long process (common)
Stapes fixation (perilymphatic gusher)

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38
Q

Development of tympanic membrane at week 11-16

A

Increases in size

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38
Q

Three other middle ear abnormalities from development

A

Congenital cholesteatoma- epidermoids
High jugular bulb
Congenital PLF

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39
Q

Franks Sign

A

Creased lobule

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40
Q

Hypertrichosis Lanuginosa Acquisita

A

Fine hair
Associated conditions:
Medications- phenytoin, streptomycin, penicillamine, minoxidil
Porphyria (werewolf myth)
Pregnancy
Malnutrition/anorexia
Malignancy

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41
Q

Auricular Hematoma

A

Collection of blood between cartilage of pinna and connective tissue
Possible causes:
Trauma
Scarring or thickening of the cartilage
Treatment:
Surgical
Prevention (drain the blood)
Refer and avoid activities that may cause trauma

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42
Q

Cauliflower ear

A

Causes: repeated trauma, common in high impact head sports like wrestling and rugby
Hearing loss is a possibility if the canal is closed off
Refer

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43
Q

Neomycin allergy

A

Medicine allergy that can cause intense itching and/or pain
Only a small percent of patients will have a reaction
Refer

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44
Q

Keloids

A

Excessive scar tissue formation following trauma or surgery to pinna; benign; more common in racial groups with more skin pigment
HL may be present if it occludes the canal
Treatment: steroid injection, pressure, surgery in some cases
Refer

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45
Q

Carcinoma

A

Malignant lesion that looks like a sore
Refer
Avoid frequent sun exposure

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46
Q

Basal cell carcinoma

A

Very common, from sun exposure, may look pink or flesh-colored and its usually a nodule with rolled borders and telangiectasias

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47
Q

Squamous cell carcinoma

A

Looks like a sore but the presentation varies

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48
Q

External otitis

A

Infection in the skin of the external auditory canal; often called “swimmers ear”
Can be caused by both fungi and bacteria, worse in hot/humid environments and can develop following lacerations of the canal, may be a chronic issue, can be infectious so use gloves
Symptoms: ear pain (particularly if the tragus is palpated), otorrhea, itching, aural fullness, tenderness/pain, fever and cellulitis, mild CHL if there is swelling, localized furuncle, redness
Refer
May be from swimming in a dirty place (counsel)

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49
Q

Malignant (necrotizing) external otitis

A

Severe inflammation caused by bacterial agent that is most common in elderly and diabetics
Symptoms: progressive pain and drainage, severe otalgia especially at night, granulation tissue in the ear canal
Can spread through eardrum to middle ear space and mastoid system
Can cause massive bone destruction in external, middle and inner ear
Occasional facial nerve palsy
Treatment: IV antibiotics, hyperbaric treatment, control of blood glucose, debridement

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50
Q

Hyperceruminosis

A

Abnormal accumulation of cerumen caused by defective production or clearance
Cerumen blockage can also result from obstruction of the canal, overly tortuous canals, stenotic canals
Can become stenotic with age
Treatment: frequent removal
More common in nursing home/people who are institutionalized
May have many colors and appear dry or wet (this is determined by genetics and diet)
May complain of tinnitus, fullness but rarely vertigo, coughing and pain
Hearing may be sudden or fluctuate, flat 30-40dB CHL with complete occlusion, tymps should be normal (may have smaller volume)
Remove or refer

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51
Q

Keratosis obturans

A

Accumulation of corneocytes due to failure of lateral migration
Forms a plug that often adheres to the skin
Will complain of fullness
HL with full occlusion

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52
Q

Canal cholesteatoma

A

Accumulation of keratin, usually related to a lesion. Typically there is bone erosion, dull pain and drainage
Refer- may need a CT to see if eroded to the bone

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53
Q

Collapsing canals

A

Most common in the elderly and very young children
Can be fixed by using inserts rather than headphones
Will usually occur in people with very cartilaginous canals
May be unilateral
ABG in mid-high frequencies (10-50) especially at 6kHz

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54
Q

Herpes zoster oticus/Ramsay-Hunt

A

Reactivation of the varicella-zoster virus at the geniculate ganglion
Viral infection of the OE, ME and IE
Trigeminal in origin
Symptoms: vesicular rash, ear pain, hearing loss, tinnitus, eyelid palsy, facial palsy, vertigo, change/loss of taste, will see it on pinna, canal and sometimes TM
Mild SNHL, affects the facial and auditory nerve so reflexes will look weird
Treatment:
Vaccination
Steroids
Anti-viral
Refer

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55
Q

Bell palsy

A

Symptoms:
Decreased tearing
Hyperacusis
Taste disturbances, metallic
Weakness of the facial muscles
Poor eyelid closure
Posterior auricular pain
Otalgia
Acute onset of unilateral upper and lower facial paralysis
Tingling or numbness of the cheek/mouth
Blurred vision
Increased salivation on affected side

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56
Q

Upper motor neuron palsy

A

Only the lower half of the face is affected
Causes:
Stroke
MS
Tumor

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57
Q

Horners syndrome

A

Problem with sympathetic nerve supply to one side of face
Symptoms:
Miosis- constricted pupils
Ptosis- droopy eyelid
Anhidrosis- failure to sweat

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58
Q

Trigeminal neuralgia

A

Sharp unilateral facial pain, usually the right side
Triggers:
Chewing, drinking, talking, smiling
Causes:
Vascular compression, tumor, inflammation, idiopathic

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59
Q

Polyps

A

Thickened nodules of skin in canal

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60
Q

Osteomas

A

Bony tumors that are benign, smooth nodule of spongy bone covered with skin
Usually at the junction of bony and cartilaginous, unilateral, pedunculated, found in kids more commonly, can occlude the canal/block cerumen
Treatment:
Surgical removement

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61
Q

Exotsoses

A

Benign bony lesions deep in the EAC
Causes:
Exposure to cold water, wind, “surfers ear”
Treatments:
Surgical if the canal is blocked
Ear plugs prevent recurrence
Usually found in adults

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62
Q

Development of the inner ear shape and size week 8/9

A

Cochlea reaches 2.5 turns
Semicircular canals attain general form

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63
Q

When does the inner ear reach full size and shape

A

The canals reach at 17-19 weeks and the cochlea 16-19 weeks

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64
Q

Development of the organ of corti week 9/10

A

Sensory epithelium develops

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65
Q

Development of the organ of corti week 10-20

A

Stria vascularis develops

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66
Q

Development of the organ of corti week 11

A

IHC and OHC present, immature stereocilia develops

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67
Q

Development of the organ of corti week 14

A

Hair cells and stereocilia better developed, supernumerary hair cells, pillar cells, tunnel of corti

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68
Q

Development of the organ of corti week 19

A

Mostly mature appearance

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69
Q

Development of the organ of corti week 20-25

A

Modiolus ossification

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70
Q

Development of the spiral ganglion week 4

A

Statoacoustic ganglion developed

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71
Q

Development of the spiral ganglion week 5

A

Cochlea and vestibular ganglia distinct

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72
Q

Development of the spiral ganglion week 7-9

A

Fibers enter cochlear epithelium

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73
Q

Development of the spiral ganglion week 11

A

Synapses on the hair cells develop

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74
Q

Development of the spiral ganglion week 14

A

Efferents on IHC develop

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75
Q

Development of the spiral ganglion week 22

A

Afferent synapses morpholol are mature

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76
Q

Development of the spiral ganglion week 15-24

A

Myelination occurs

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77
Q

When does the saccule and utricle develop

A

Weeks 6-8

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78
Q

What are the four labyrinth anomalies

A

Michel aplasia, common cavity malformation, cochlear hypoplasia and Mondini IP2

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79
Q

Michel aplasia

A

Aplasia of all inner ear structures (no structures)
Caused by- thalidomide, Slippel-Feil, anencephaly
Will occur during the 4th week
HL- severe to profound, bilateral deafness, absent reflexes
HL can vary
Contraindication for CI!!!!
Refer

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80
Q

Common Cavity Malformation

A

Membranous labyrinth present but poorly differentiated
Severe-profound hearing loss
Will occur during the 4th week
If a CI is used, there will be a lot of leaks

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81
Q

Cochlear Hypoplasia

A

Rounded, small cochlea
Degree of hearing loss varies depending on membranous differentiation
Will occur in the 6th week
Can have a whole variation of hearing depending on membranous labyrinth
Turns could be 2 or fewer

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82
Q

Mondini IP2

A

Incomplete partition, most common bony anomaly, most often bilateral
Often associated with LVAS
Usually occurs around week 7
Cochlea will be about 1.5 turns
Absent interscalar septum middle and apical turns
At risk with perilymphatic fistula, meningitis
Associated with Waardenburg, DiGeorge, CHARGE and Pendred syndromes
Ask about family history and genetics
Usually severe to profound SNHL with some preservation of the high frequencies due to basal turn being still functioning
May be severe rising to mild SNHL, bilateral, congenital
Degree of SNHL depends on degree of malformation
SRT and WRS could be better than PTA due to good HF hearing
OAE will be absent where there is hearing loss
Reflexes will be absent where hearing loss
Refer
Surgery is the only treatment

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83
Q

Bing-Siebenmann malformation

A

Otherwise known as cochleosaccular dysplasia (did not grow the right way)
Bony but intact organ of corti has collapsed Reissners and abnormal stria vascularis
Usually profound SNHL
Associated with Jervell and Lange-Neilsen, Usher

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84
Q

Schiebe malformation

A

Most common membranous malformation
Partial/complete aplasia of ossicular chain, collapse of cochlear duct
Severe to profound SNHL
Associated with Jervell and Lange-Neilsen, Waardenburg, Refsum and trisomy 18

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85
Q

Alexander malformation

A

Dysplastic basal turn, otherwise normal labyrinth
Hereditary high frequency SNHL

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86
Q

Large vestibular aqueduct syndrome (EVA, LVA)

A

Most common anomaly seen in imaging
Associated with incomplete partition, Pendred, BOR, perilymphatic gusher, Mondini
Aqueduct that is 1.5mm or greater at midpoint
Can also have a large endolymphatic sac
3rd window theory- ABG
Similar symptoms to perilymph fistula
Needs to be diagnosed with CT or MRI
More common for kids and usually diagnosed during childhood, progressive (related to 250 Hz) and will fluctuate, cochlear in cause, fake ABG but normal tymps and reflexes, may be unilateral (more common) or bilateral
Usually mixed, primarily SNHL, possibly severe to profound CHL but may have extremely good bone at low frequencies
Characterized by sudden drops and fluctuations

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87
Q

Superior semicircular canal dehiscence (SSCD)

A

Seen in young children but incidence increases with age
Symptoms:
Tullio, Hennebert, gaze evoked tinnitus, hypersensitive to sound, autophony, conductive hyperacusis
May complain of feeling full in the ear, unsteadiness when sneeze or cough, onset of symptoms are not linked to a specific event
Usually unilateral with gradual onset
No evidence of TM or ossicular abnormality
CHL will be similar to otosclerosis but unlike an ME problem will have AR and tymps, DPOAEs if hearing loss allows, VEMP and WAI findings, case history
Will have fake ABG
Surgery to patch the SCC

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88
Q

What percent of hearing loss is genetic in prelingual Deaf children?

A

50%

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89
Q

What percent of hearing loss is non-syndromic in prelingual Deaf children?

A

70%

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90
Q

Genotype

A

The specific genetic constitution of an organsim

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91
Q

Phenotype

A

The observable properties of an organism; the expression of genes in traits or symptoms

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92
Q

Heterogeneity

A

Any of several genes can produce the same phenotype
Diverse in character/content

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93
Q

Autosomes

A

Chromosomes other than sex

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94
Q

Variant

A

Change in a genes DNA

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95
Q

Mutagen

A

Substance that causes a mutation

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96
Q

Spontaneous mutation

A

Change in bases during replication

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97
Q

Mendelian inheritance

A

Autosomal dominant, autosomal recessive and X-linked

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98
Q

Syndromic

A

Group of characteristics that co-occur

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99
Q

For a person to have a autosomal recessive disorder, what must they have?

A

A double dose of the gene

100
Q

Polygenic traits

A

Determined by more than one gene

101
Q

Multifactorial traits

A

Determined by one or more genes as well as the environment

102
Q

Usher syndrome

A

Dual sensory impairment- will have a auditory, vestibular and visual presentation
Several subtypes with some being nonsyndromic during childhood before vision loss
Autosomal recessive

103
Q

Jervelle and Lange-Neilsen Syndrome

A

Signs and symptoms:
Syncope (fainting) most often elicited by emotion or exercise
Autosomal recessive
Due to cardiac issues, risk of sudden death without treatment is high
Congenital profound bilateral SNHL
Refer

104
Q

Pendred syndrome

A

Autosomal recessive
Can be congenital or late onset often progressive hearing loss (may be sudden or fluctuate) but is often bilateral
Associated with Mondini and EVA
Possible thyroid enlargement (goiter)
Testing- imaging, genetic testing, vestibular, counseling, issue of progression for treatment

105
Q

Pierre Robin sequence

A

At 7-10 weeks of development the jaw grows rapidly OR the jaw does not grow so the tongue cannot lie flat, it then rests at the back of the mouth and prevents the palate from closing causing a cleft palate.
Often respiratory and feeding problems
Isolated version of this- jaw of affected child is often able to grow and catch up to normal size but often is associated with other conditions
Mainly CHL due to OM auricular anomalies
Auricular anomalies in 75%

106
Q

Craniosynostosis

A

Premature fusion of neurocranial sutures
Craniofacial dysmorphologies
Usually nonsyndromic
Will involve hands and feet

107
Q

Muenke syndrome

A

May have normal cognitive function to mild ID, changes in extremities
SNHL is most common and CHL would be related to OME
Autosomal dominant

108
Q

Penetrance

A

The percentage of individuals who possess a dominant gene and express it

109
Q

Variable expressivity

A

A genotype producing a phenotype that varies among individuals

110
Q

Apert syndrome

A

Up to 80% of these patients will have hearing loss due to stapedial fixation, middle ear structural anomalies, OME
Will typically have cleft palate, flat “dished” face, syndactyly, ID about 50% of the time
Possible heart or kidney problems
Primarily sporadic but is related to paternal age effect
Autosomal dominant

111
Q

Crouzon

A

75% of these patients will have hearing loss in addition to stenosis, atresia, microtia
CHL is most common yet middle ear structural anomalies are rare, OME, SNHL more often than other syndromes

112
Q

Treacher Collins syndrome

A

Lower jaw and cheekbones are smaller than normal, lower eyelid coloboma (notch), downward sloping eyes
Outer ear abnormalities (microtia, ear tags, ear pits, atresia), CHL, rarely SNHL
Cleft palate 30% of the time
60% of the time its sporadic (related to the advanced age of the father)
Autosomal dominant
100% penetrance, variable expressivity
Maximum CHL (around 60)

113
Q

Hemifacial microsomia, Goldenhar syndrome, Oculo-Auriculo-Vertebral (OAV) spectrum

A

Facial asymmetry of varying degrees- vascular or neural crest disruption
Vertebral changes such as cervical spine and cranial base malformation, occasional heart and kidney issues
Ocular findings, may involve brain and other organs, usually no ID
Auricular abnormalities (a spectrum from mild morphologic changes of the pinna to complete anotia)
Microtia if the most common, ossicular malformation common, absence of the IAC (inner auditory canal) or inner ear abnormality infrequent
50% have CHL and/or SNHL

114
Q

Stickler syndrome

A

Hearing loss most common, specifically high frequency SNHL but CHL may also be common
Midfacial underdevelopment
Cleft palate
Eye problems- myopia, cataract and retinal detachment in childhood
Joint problems- arthritis by 40s
Hypermobile TM 46% of patients
Underidentified
Variable phenotypic expression
5 types
Mostly autosomal dominant

115
Q

Alport

A

X-linked recessive, autosomal dominant
Kidney function- hematuria in early childhood
Progressive hearing loss, eye involvement (except when autosomal dominant), rare aneursyms in males
Bilateral high frequency SNHL in late childhood
90% of patients are deaf by age 40
Less severe progress and outcome in females

116
Q

Branchio oto renal syndrome

A

Fistulas and cysts associated with 2nd brachial arch
Pits, tags, pinna abnormalities, ME and inner ear structural abnormalities, may be unilateral
May have small or abnormal kidneys, absent kidney, ultimately end stage renal disease possible
May not be identified
Often confused with Alport
Hearing loss in 95% of the patients
Malformed auricles
Second branchial cleft anomalies
Preauricular sinus
Renal abnormalities
AD

117
Q

Otosclerosis

A

Not a craniofacial syndrome
No effect on facial structure
Youngest presentation is around age 5
Autosomal dominant
Multifactorial

118
Q

Waardenburg

A

Four types
Dystopia canthorum, a broad nasal root, confluence of the medial eyebrows, heterochromia irides, a white forelock
Autosomal dominant/recessive
A neural crest defect
Hearing loss is not typically progressive, usually profound but can be mild in high frequencies, unilateral or bilateral SNHL (usually profound bilateral SNHL)
Expressivity is extremely variable
May be not identified
May have temporal bone abnormalities including EVA
Not associated with progressive hearing loss
Refer

119
Q

Otitis media

A

Inflammatory process of the middle ear

120
Q

When does the ET open?

A

During active processes so yawning, sneezing, swallowing or when eccessive pressure is applied from the nose

121
Q

When does the ET reach adult size and configuration?

A

Age 7

122
Q

ET purpose

A

Ventilation, drainage, protection, pressure equalization

123
Q

Patulous

A

Always opening (in reference to the ET)
May be due to anatomic structures, radiation, MS, radical head neck surgery

124
Q

Mastoid

A

Area of the skull that surrounds the ear; it is “honeycombed” with hundreds of air cells, air cells are lined with mucous membrane

125
Q

Role of chorda tympani

A

Sensory branch of the facial nerve, has a role in taste

126
Q

Acute otitis media (AOM)

A

History of acute onset, middle-ear effusion, signs and symptoms of middle ear inflammation
Complaints for adults- mild intermittent pain, fullness, popping, balance problems, clumsiness
Complaints for infants- ear rubbing, irritability, fever, headache, cough, rhinitis, listlessness, anorexia, vomiting, diarrhea, sleep interruption
Pacifier use can increase the likelihood of this
Otoscopy- moderate to severe bulging, new onset of otorrhea, ear pain
May have CHL

127
Q

Otitis media with effusion

A

Presence of fluid in middle ear, no signs or symptoms of acute ear infection, hearing loss may be present
Otoscopy- amber, grey, white, blue
Hearing loss- mild
Will see best air frequency at 2kHz

128
Q

Recurrent acute otitis media

A

Three or more episodes of AOM within 4 or 6 months in a 12 month period
Affects 10-20%, 40% of these have 6+ episodes

129
Q

Chronic otitis media with effusion

A

Persistent fluid behind intact tympanic membrane in the absence of acute infection

130
Q

Chronic suppurative otitis media

A

Persistent inflammation of the middle ear or mastoid cavity
Recurrent or persistent otorrhea through a perforated tympanic membrane

131
Q

At what ages does OM prevalence peak?

A

6-18 months and 4-5 years of age

132
Q

What is the incidence of OM by age 2?

A

90%

133
Q

What are typical complaints of OM for adults?

A

Usually with otalgia (pain), crackling/popping tinnitus, fullness

134
Q

Two subtypes of AOM

A

Bacterial and viral (cannot be treated with antibiotics if viral)

135
Q

Symptoms of AOM for infants

A

Ear rubbing, irritability, fever, headache, cough, rhinitis, listlessness, anorexia, vomiting, diarrhea, sleep interruption

136
Q

General symptoms for AOM

A

Mild intermittent ear pain, fullness, “popping”, hearing loss, problems with school performance, balance problems, clumsiness and gross motor issues

137
Q

Color of the TM for a patient with AOM

A

White usually, also yellow or red

138
Q

Color of the TM for a patient with OME

A

Amber diagnostic, also grey, white or blue

139
Q

Myringitis

A

Inflammation of the TM, sudden pain, blebs or vesicles visible

140
Q

Atelectosis

A

TM sucked in so much, it sits on structures; may look like a perf

141
Q

Purulent

A

Infected, drainage

142
Q

Chronic otitis media

A

Usually follows recurrent AOM
Usually results in CHL
Can be serous or mucoid

143
Q

Serous

A

Fluid

144
Q

Mucoid

A

Mucous

145
Q

Do you expect acoustic reflexes with otitis media

A

No- do not expect reflexes with CHL

146
Q

Acute Mastoiditis

A

May be due to untreated bacterial AOM
High fever, pain/tender behind ear, may not have acute pain (especially with adults)
Children under 2 (immature immune system)
Risks of meningitis, abcess, CN palsy
Less common in developed countries
Testing: otoscopy, tymps (may be flat B or not), pure tones (CHL), possible poor word recognition, CT scan and blood tests

147
Q

Meningitis

A

Relatively common of the intracranial complication of OM
Symptoms- fever accompanied by neck stiffness
Tests- CT or MRI, lumbar puncture
Treatment- antibiotics

148
Q

Abcess- brain or epidural

A

One of the most common intracranial complication of COM
Latter- temporal bone through veins or boney erosion in the tegmen tympani or bone by posterior cranial fossa or sigmoid sinus
Former- usually temporal lobe or cerebellum, likely direct spread of infection
Symptoms- headache, vomiting, lethargy, seizures, hemiparesis, cranial nerve palsies and aphasia
Test- CT, MRI
Treatment- antibiotics and/or surgery

149
Q

Cholesteatoma

A

Cystlike lesions of the temporal bone
May be caused by CSOM

150
Q

Labyrinthitis

A

Inflammation of any part of the labyrinth (via round window, erosion)
Vestibular and/or auditory symptoms (hearing loss, vertigo)
Temporary- assumption is that the cause was serous and mostly due to inflammation
Permanent- more likely due to suppurative, bacteria in the inner ear
Treatment- intravenous antibiotics

151
Q

Facial Paralysis

A

CSOM especially with cholesteatoma causes inflammation of the facial nerve which then triggers edema and nerve fiber compression causing this

152
Q

Biofilm

A

Bacterial slime

153
Q

Treatment for OME

A

Non-surgical- wait and watch, autoinflation, drugs
Surgical- PE tubes, adenoidectomy, tonsillectomy

154
Q

CT scan

A

Focuses on bone
White = bone
Black = air
Risk for children

155
Q

MRI

A

Better for soft tissue and neural structures
No radiation but more time consuming

156
Q

Perilymphatic gusher

A

Basically a fistula that when ruptured perilymph rushes out and causes severe hearing loss

157
Q

Alexander malformation

A

Least severe membranous labyrinth malformation

158
Q

Lumen

A

Opening of ET

159
Q

Third window theory

A

Air conduction will be impacted because the air will go through the extra space (thresholds will be high), bone will be normal
Energy is lost through the enlarged aqueduct

159
Q

Neuritis

A

Facial paralysis

159
Q

Common cavity malformation

A

Membranous labyrinth present but barely functioning

160
Q

Cochlear hypoplasia

A

Small aplasia, still a little development of the cochlea, degree of hearing loss depends on membranous labyrinth development

161
Q

Precision medicine

A

Looks at everything including, but not limited to, SES, gender, living area/conditions, genetics etc.
Will give better outcomes

162
Q

Otoferlin

A

A gene often seen in ANSD patients and it encodes for synaptic vesicle fusion to the membrane

163
Q

Karyotype

A

Picture of all the chromosomes in the body

164
Q

Epigenetics

A

Heritable, changes that affect the codes accessibility, happens in response to environmental conditions
Genetic code that is altered due to the environment

165
Q

Incomplete penetrance

A

People with the gene have the same phenotype

166
Q

Connexin 26

A

AR and non-syndromic, imaging will not help diagnose
Most common form of congenital deafness
Family history of deafness important here
Normal tymps and otoscopy
Down sloping audiogram with HL in the high frequencies are the worst
Severe to profound deafness
Early onset with minimal progression
Excellent results with CI
Not as frequent symptoms- progressive HL, mild to moderate deafness, asymmetry dominant inheritance and associated with skin disorders

167
Q

Mitochondrial linked disorders

A

Will impact all kids
Only goes through the mother
Disorders characterized by symptoms in tissues with high energy demands

168
Q

X-linked disorders

A

Typically only impact the male children
If it is recessive, the mother will never show the affects but will show up in males in the family
If it is dominant, anyone who gets the gene will have HL

169
Q

Otosclerosis

A

Multifactorially mediated metabolic bone disease
Autosomal dominance penetrance
Variable expressivity
Affects only the human otic capsule and ossicles
Progressive CHL
Tymps are variable
Typically stapes fixation
Can invade the cochlea in advanced cases
Most common in caucasian population and most common in the 30s
More common in women
Uncommon in children
Heredity is a factor
Genetically heterogeneous
Declining due to less measles presence
Bluish cast to whites of eyes (this is seen in other bone diseases)
Schwartze sign/risin sun (reddish blush near the promontory
Tinnitus 50% of the time
Dizziness has been reported but is not common
Paracusis willisii (speech is easier to understand in noise than in quiet)
Difficulty hearing while chewing
Excellent word discrimination
Bone will decrease at 2k
Acoustic reflexes typically absent
Diagnosis/treatment:
CT, surgery confirmation and history

170
Q

Cochlear otosclerosis

A

Affects the round window and invades the cochlea to cause SNHL that progresses overtime
Rare

171
Q

Carhart notch

A

Bone conduction thresholds decreased at 2k, 500 and 1k
Persistent ABG at 500 may be due to malleus/incus fixation
Air conduction thresholds may or may not be normal
Mechanical artifact possibly due to shift in resonant frequency produced by immobile oval window
After surgery there is recovery of about 8 dB

172
Q

Candidates for stapedectomy

A

Air bone gap of 15 or more
Speech discrimination score of 60% or more
Absence of anatomic or medical contraindications, risk of gusher
usually operate one one ear at a time if bilateral

173
Q

Outcomes of stapedectomy

A

Possible:
Permanent facial nerve injury, perf, vertigo, obliterative otosclerosis, CSF leak, floating footplate, subluxation of incus/malleus joint, tinnitus, hyperacusis

174
Q

Malleus/incus fixations

A

Presentation is similar to otosclerosis
Normal otoscopy but with pneumatic otoscopy will not see malleus move
ABG
Diagnosis during exploratory surgery

175
Q

Ossicle fixation

A

Recurrent OME, ossicular discontinuity due to incus necrosis or ossicular chain fixation due to tympanosclerosis
Poorly developed mastoid air cells is a risk factor
Congenital stapes fixation

176
Q

Paget Disease

A

Irregular breakdown and formation of bone, affects spine, skull and other bones
Symptoms:
Headache, pain, deformed bone otherwise asymptomatic
Diagnosis through alkaline phosphatase
Rare
CHL and/or SNHL
Vertigo
Some reports of low frequency CHL with high frequency SNHL

177
Q

Osteogenesis imperfecta

A

Otherwise known as brittle bone syndrome
1/20000
Genetic
Affects the collagen formation
AD and variable expressivity
Most severe form if incompatible with life- fractures in utero
Blue sclera
Four subtypes
Progresses from SNHL in kids to mostly mixed in older ages
Chance of having significant ABG

178
Q

Cholesteatoma

A

Invasive progressively enlarging tumor of the middle ear
Benign
Cyst like lesion that contains keratin and decaying materials
Often starts at tympanic membrane perf when epithelial layer sheds skin and forms a mass
Highly invasive, can erode the bone
May develop anywhere in the temporal bone but typically in the middle ear space
Can be acquired, congenital or iatrogenic
Signs and symptoms:
Painless smelly drainage, maybe dizziness, pain progressive unilateral CHL, retraction of TM, perforation, granulation tissue, unresponsive to antibiotics, possible epithelial debris in the canal, in otoscopy will see white mass medial and retraction pocket and perf, sometimes dizziness
Tymps will consistent with perf, may showed reduced mobility due to mass
Absent reflexes
Diagnose with CT

179
Q

Congenital Cholesteatoma

A

Present at birth
Detected in childhood
More common in males
Occurs in children with no history of perf, otorrhea, otologic procedures
Presents as a white mass medial to an intact TM

180
Q

Hemotympanum

A

Blood behind the TM due to trauma
Can take 4 weeks to resolve

181
Q

Barotrauma

A

Change in pressure
Symptoms:
Pain, hearing loss, tinnitus, vertigo

182
Q

Glomus Tumor

A

Benign highly vascularized tumors
Originate from cells called paraganglia that are found in the middle ear
Two types
May have hoarseness, tongue paralysis, vocal weakness, shoulder drop, aspiration
Pulsatile tinnitus is the characteristic symptom
Tymp- saw tooth pattern
Otoscopy- reddish/blueish mass behind TM
Reflexes- contralateral decay
CHL if the tumor is large enough to interfere with ossicular vibration

183
Q

Glomus tympanicum

A

Glomus bodies on Jacobsons nerve that crosses promontory or Arnolds nerve
Signs:
Reddish/bluish mass behind an intact TM, pulsatile tinnitus, late non-auditory symptoms (hoarseness, aspiration, tongue paralysis, shoulder drop, vocal weakness, facial nerve paralysis)
Sawtooth tymps

184
Q

Glomus jugulare tumors

A

Paraganglia in the jugular bulb under the middle ear
Signs:
Pulsing tinnitus, CHL, swallowing difficulties, possible dizziness
Slow growth
May eventually invade the brain/brainstem

185
Q

Wegeners granilomatosis

A

Systemic disease of connective tissue
Characterized by infalmmatory reactions around blood vessels
OME but effusion does not resolve with antibiotics or PE tubes
Treatment in steroids and immunosuppressant therapy

186
Q

TB

A

Bacterial infection that can cause an infection in the middle ear
TM will be red and look thickened
Effusion can block ossicles

187
Q

Syphilis

A

Can involve the middle ear, mastoid and inner ear
Can cause fibrous tissue in the middle ear
Fluctuating low frequency SNHL

188
Q

Immotile cilia syndrome

A

AR
Flat TM, bilateral CHL
Will not move as effectively
Will affect other parts of body

189
Q

What factors would qualify a hearing loss as nongenetic congenital? (4)

A

Infectious, chemical, physical and maternal

190
Q

Perinatal timeline

A

About 4 months before birth to 1 month after birth

191
Q

Perinatal hearing loss causes (7)

A

Hyperbilirubinemia, anoxia, trauma, prematurity, NICU, ECMO, other teratogens

192
Q

Hyperbilirubinemia

A

Neonatal jaundice that often occurs 2-4 days postnatal
Build up of bilirubin
Yellow whites of eyes and skin
If extreme excess- kernicterus which is when bilirubin accumulates in the brain, neurological damage including cerebral palsy, hearing loss, ANSD
Prematurity is a risk factor
Temporary HL and permanent HL, specifically mild to severe SNHL

193
Q

Toxoplasmosis

A

From undercooked meat, unpasteurized goats milk, infected cat litter, unwashed fruits/veggies
A risk for a weakened immune system
Prenatal exposure to active infection
Ocular, neurological, cardiac effects
About 10-15% in US have antibodies, can reoccur with immundeficiency (AIDS)
Congenital 1/10000 in US (higher in Europe, Poland and 9/1000 in Brazil)
If it occurs in the first trimester fetal death will occur but in the 3rd will have disease
Early treatment seems effective
Hearing loss may range from mild unilateral to bilateral profound; congenital and delayed onset hearing loss
Ocular, neurological and cardiac effects

194
Q

Congenital Toxoplasmosis

A

85% infected infants are asymptomatic at birth
HL may range from mild unilateral to bilateral profound
Congenital and delayed onset HL
Highly variable
Associated with cognitive issues and late appearing learning disabilities (limited data)

195
Q

Syphilis

A

In utero fetal death is 50%
23.1 cases per 100000 births/year, 90% with asymptomatic syphilis
Effects- notched incisors, corneal scarring, flat SNHL, blindness, deafness, facial deformity, nervous system problems including neuropathy
Diagnosed through rapid plasma reagin, VDRL, FTA-abs
Treatment through penicillin
Late onset HL the rapidly progressive, may fluctuate
May have CHL from ossicles
Low word discrimination
Tullio or Henneburt

196
Q

Otosyphilis

A

HL- flat SNHL, late onset, progressive, may fluctuate, SSNHL, CHL, vertigo, Hennebert, Tullio, low discrimination, may look like Meniere
37% onset in childhood, 51% between 25 and 35 years of age and 12% later in life
More likely profound and bilateral or high frequency rapidly progressing to profound bilateral in young kids
Highly variable, progressive, often asymmetric in adults

197
Q

Rubella

A

Mostly eliminated in the US
Virus, mild disease, may be asymptomatic
1st trimester infection serious for fetus- 90% growth retardation, ID, HL, cataracts, heart and other defects, miscarriage, dual sensory impairment
2nd semester 40% HL
Usually flat severe bilateral SNHL but may be any mild to profound and can be unilateral and/or asymmetric
Typically congenital, can be late onset
Otoscopy, tymps should be normal, no reflexes
Usually flat severe bilateral SNHL but can be mild or profound

198
Q

Cytomegalovirus (CMV)

A

Asymptomatic or mild-flu like symptoms, may be latent throughout life and may reactivate
Estimate of prevalence of congenital infection in industrialized countries is 0.7%
Person to person transmission or through fluids
Effects when congenital- asymptomatic or have jaundice, splenomegaly, thrombocytopenia, intrauterine growth retardation, microcephaly, retinitis, HL
Symptomatic are at greater risk for HL and more severe HL
HL is mostly late onset, can be progressive and fluctuating
Asymptomatic may have moderate to profound bilateral HL
Symptoms are variable

199
Q

What is symptomatic CMV associated with?

A

Greater risk of HL, more severe HL, vision loss, microcephaly, ID

200
Q

What is associated with asymptomatic CMV

A

Moderate to profound bilateral hearing loss by age 6
Vestibular issues (late walking very common, head movement, abnormal calorics)

201
Q

Testing for CMV

A

Congenital- must test at or immediately after birth
Postnatal- not uncommon and does not have same effects
Blood spot, saliva, urine

202
Q

Herpes simplex virus (HSV)

A

Cold sores, genital herpes
Infection usually occurs during birth
Very rare cause of HL but can be unilateral or bilateral, severe to profound SNHL
May interact with other factors

203
Q

Postnatal meningitis

A

Can be viral or bacterial
Symptoms- rapid, high fever, stiff neck, vomiting, headache, rashes, sensitivity to light
Type will vary if there is HL
HL may be mild to profound
HL may resolve when disease resolve
Inflammation of the membranes of the brain and spinal cord
Suppurative labyrinthitis damages the organ of corti
Loss of the spiral ganglion cells
It is important to monitor for fluctuations in the first year
May be ossification of the cochlea
Older adults are mostly affected by this

204
Q

Zika

A

Microcephaly
Variable
Associated with West Nile, Herpes zoster

205
Q

Aminoglycosides

A

Used for serious bacterial infections
May cause cochlear, vestibular toxicity depending on the meds
Symptoms- high frequency HL, tinnitus, dysequilibrium, oscillopsia, aural fullness

206
Q

Loop diuretics

A

Removes water from the body which is good for heart failure, hypertension, edema and renal failure
Likely to affect stria vascularis
Usually reversible side effects

207
Q

Cisplain (antineoplastic agent)

A

Incidence is high and may be delayed and affected by dose
OHC degeneration
Bilaterally symmetric SNHL usually high frequency and irreversible, tinnitus often initial

208
Q

NSAIDS

A

Often will cause tinnitus more commonly then HL

209
Q

Quinine

A

Used to treat restless leg syndrome
Symptoms- tinnitus, HL (usually SNHL), vertigo, headache, nausea, and vision loss

210
Q

Sildenafil

A

Viagra
Sudden hearing loss

211
Q

X-ray

A

Electromagnetic, attenuated by structures, image on film, quick
Sometimes used for intraoperative/post-operative of CI electrode placement
Better for bone but not usually used in audiology

212
Q

CT scans

A

Sums multiple x-rays, primarily used for assessing bony skull base pathology (dysplasia of bony labyrinth, congenital external/middle ear anomalies, SSCD)
Shows up as bone (white), air space (black), tissue (grey)

213
Q

MRI (magnetic resonance imaging)

A

Uses electromagnetic properties of hydrogen nuclei to produce cross sectional images of internal body structures, best for soft tissues (excellent contrast), can show if CN VIII is absent or present
Primarily used for membranous labyrinth, IAC and intra-axial structures
Cannot have metal in body for this

214
Q

PET scan (position emission tomography)

A

Uses radioactive tracer to identify active parts of the brain or tumors (including glomus tumors)

215
Q

Etiology

A

The cause, set of causes or manner of causation of a disease or condition

216
Q

Pathophysiology

A

The disordered physiological processes associated with disease or injury

217
Q

Comorbid

A

Presence of one of more additional disorders co-occurring with a primary disease

218
Q

Variable expressivity

A

Phenotype expressed to different degree among people with the same genotype

219
Q

Epidemiology

A

Study and analysis of patterns, causes and affects of health and disease conditions in defined populations

220
Q

Why is it important to recognize pinna variants? (4)

A

Can be associated with hearing loss and other syndromes
Funny looking ear can have a big effect on kids (social, emotional etc.)
There might be an effect on the transfer function
Might be challenging to give hearing aid (think atresia III)

221
Q

Hairy tragus

A

Normal variant
Generally found in older men

222
Q

Hairy pinna

A

Normal variant
Coarse hair
Usually in the lower helix

223
Q

Pressure atrophy

A

Normal variant
Can get from glasses or hearing aids

224
Q

Is prominent ear associated with HL or other audiological difficulties?

A

No hearing loss or difficulties are reported

225
Q

Is cup ear associated with HL or other audiological difficulties?

A

No hearing loss or difficulties are reported

226
Q

Is lop ear associated with HL or other audiological difficulties?

A

Possibly…is associated with some syndromes so may be associated with hearing loss but may not be the cause

227
Q

Is stahls ear associated with HL or other audiological difficulties?

A

Otherwise known as Spock ear
Significant delineation of anti-helix
Possibly… is associated with certain syndromes so may be associated but not the cause

228
Q

Tags and pits

A

From hillock of his (so present at birth)
Occurs in high rates in individuals with HL especially with some syndromes
Refer

229
Q

Frostbite and burns

A

Refer

230
Q

Aplasia

A

Nothing grew, nothing happened, no structure, failure of an organ or tissue to develop or function normally

231
Q

Hypoplasia

A

Congenital condition
Incomplete development or underdevelopment of an organ or tissue

232
Q

What is a possibility for every bony anomaly?

A

It can affect the facial nerve

233
Q

Cystic cochleovestibular malformation (pseudo mondini)

A

Cystic cochlea lacking septae and the modiolus
Poorer hearing than classic mondini

234
Q

Vestibular anomalies

A

Aplasia or dysplasia of lateral canal most common (happens late in development)
SNHL, mixed or CHL
Associated with CHARGE syndrome
May be present with cochlear anomalies

235
Q

PSCD (posterior semicircular canal dehiscence) and LSCD (lateral semicircular canal dehiscence)

A

Clinically rare
Associated with a high-riding jugular bulb and fibrous dysplasia
Possible mild CHL to significant CHL

236
Q

Prematurity

A

Low birth weight (under 6 pounds)
Co-existing factors- ototoxic, hyperbilirubinemia, in utero infection, craniofacial anomalies, syndromes, low APGAR score, mechanical ventilation for over 5 days, bacterial meningitis, family history of hereditary childhood SNHL, endocranial hemorrhage, hypoxic ischemic encephalopathy, convulsions, sepsis, GI surgery need, persistent pulmonary hypertension, intraventricular hemorrhage, patent ductus arteriosis ligation, lower SE

237
Q

ECMO HL

A

Higher risk for progressive/delayed SNHL

238
Q

TORCH

A

Toxoplasmosis
Other- syphilis, varicella, parvovirus, HIV
Rubella
CMV
Herpes simplex
Teratogens (such as FAS, dilantin, isoretinoin, thalidomide)

239
Q

HIV

A

Hearing loss can occur
May be unilateral or bilateral, progressive, sudden
Can be CHL, SNHL or mixed
Greater risk for otitis media

240
Q

Varicella

A

Rare now due to vaccine
Tinnitus is common and vertigo in 1/3 of cases
Unilateral mild-moderate SNHL
May cause sudden SNHL but that is rare

241
Q

Measles

A

Rare now due to vaccine
Bilateral moderate to profound SNHL

242
Q

Mumps

A

Rare due to vaccine
May cause profound HL that is often unilateral

243
Q

What is the most common cause of intrauterine infection

A

CMV

244
Q

What is the most common cause of non-genetic congenital SNHL in developed countries

A

CMV

245
Q

Autosomal dominant inheritance

A

50% change of having a baby with HL, only need one copy

246
Q

Autosomal recessive inheritance

A

Both copies of gene needed to have deafness element
Chance for offspring is 25%
Complete penetrance and expressivity

247
Q

Syndromic auditory syndromes (15)

A

BOR, Treacher Collins, Apert, Congenital stapes fixation, Alport, Jervell & Lange-Neilson, Pendred, Waardenburg, Crouzon, Goldenhar, Stickler, Hunter, Usher, Down Syndrome, Pierre Robin, Wolfram

248
Q

COMPT

A

Color
Other conditions (fluid level, perf, otorrhea etc.)
Mobility of TM
Position
Translucency

249
Q

Perf TM

A

Often trauma related
Will see it in otoscopy, tymps will have big volume
CHL increases as size of perf increases, largest losses at lowest frequencies
Should heal without intervention but in a big enough perf may need a patch
Monitor, antibiotic drops or steroid drops maybe

250
Q

Ossicular discontinuity

A

Case Hx may be- insertion of foreign body to the external ear canal, trauma, cholesteatoma, chronic OME
Tymp- Ad
Acquired CHL, may have SNHL too
Can have max CHL
Maybe get reflexes

251
Q

Temporal bone fracture

A

May present with facial nerve with facial paresis or paralysis
Case Hx may include black eye, bruising and blood behind the ear; can be from a car accident or blunt trauma
Usually young males
SNHL if there is a transverse fracture and CHL if longitudinal
Balance disturbances
Tinnitus
Vertigo
CSF leak through fracture lines

Disorders (3 decks)

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