Hearing Disorders Take 2

Front 1

The outer two-thirds or ________ portion of the ear canal contains two kinds of glands, ________ glands, and ______glands.

Back 1

cartilaginous
ceruminous glands
sebaceous glands

Front 2

T/F The bony ear canal contains glands and hairs.

Back 2

False. The bony ear canal does NOT contain glands or hairs.

Front 3

T/F It takes several months for the lining (dead skin and such) to reach the border of the cartilaginous portion of the ear canal.

Back 3

True!

Front 4

T/F Presence of earwax is always a problem.

Back 4

False. This is typically not a problem.

Front 5

Earwax can do the following:

Back 5

Protect
Lubricate the ear canal lining

Front 6

If enough skin flakes, hair gland secretions dust, and other foreign materails are present in the ear canal the result can be ______ ______.

Back 6

Earwax impaction!!! ewww.

Front 7

Impaction of earwax can cause the following:

Back 7

Sensation of obstruction
Mild conductive HL
Otalgia (pain)
Vertigo
Coughing

Front 8

Audiologist does what for earwax impaction?

Back 8

Cerumen management.

Front 9

Ear canal collapse is most likely to occur in what two populations?

Back 9

Infants and children <7 years
Elderly

Front 10

What does idiopathic mean?

Back 10

No known cause.

Front 11

What is an example of a growth in the ear canal?

Back 11

Exostoses - a cartilage capped bony projection arising from the ear canal wall.

Front 12

Exostoses can cause:

Back 12

Sensation of obstruction
Mild conductive HL
Otalgia (pain)
Vertigo
Coughing
Tinnitus

Front 13

What are the disorders of the middle ear that were covered in class?

Back 13

Eustachian tube dysfunction.
Otitis media (with effusion).
And other disorders (may be caused by OM):
mastoiditis
TM perforation
TM retraction
Tympanosclerosis
Cholesteatoma

Front 14

T/F the ET is open at rest and closed by the levator palitini and tensor veli palitini muscles during the acts of swallowing or yawning.

Back 14

FALSE. ET is closed at rest and opened when swallowing or yawning.

Front 15

The ET allows for what?

Back 15

Aeration of the ME
Pressure equalization of the ME
Optimal functioning of the ME mechanisms.

Front 16

What are the signs and symptoms of ET blockage/dysfunction?

Back 16

Feeling of blockage
Otalgia (pain) due to tension on the TM
Possible HL and tinnitus
Possible dizziness/balance problems

Front 17

What are some causes of ET dysfunction?

Back 17

Normal pediatric craniofacial configuration (shorter, more horizontal)
Poor function of muscles
ET walls more compliant in children (more susceptible to collapse)
Structural deformities
Inflamation and swelling of mucosal lining
Neoplasms (i.e. nasopharyngeal carcinoma)
Functional
Barotrauma (sudden change in air pressure)

Front 18

What is the Valsalva maneuver?
What is the toynbee maneuver?

Back 18

Valsalva: hold nose, close mouth and blow
Toynbee: close the mouth and jaw, hold the nose and swallow.

Front 19

What is Otitis Media?

Back 19

An inflammatory process of the ME cavity that often includes the mastoid.

Front 20

T/F Otitis Media is second only to the common cold as the most common inflammatory disease of childhood.

Back 20

True.

Front 21

T/F The most common cause of Otitis Media is windy weather.

Back 21

False. Most commonly due to ET dysfunction.

Front 22

What is effusion?

Back 22

Fluid.

Front 23

What are some signs and symptoms of OM?

Back 23

Otalgia (pain)
Otorrhea (discharge)
Fevers
Restless sleep, irritability and temperament disorders
Possible balance and hearing disorders.

Front 24

Classification of OM is typically based on ______ and _____ __ _____.

Back 24

Duration and Type of effusion.

Front 25

What are the classifications of OM?

Back 25

Duration:
Acute - 1-21 days
Subacute - 22 days - 3 months
Chronic - > 3 months
Effusion:
Serous - sterile fluid
Suppurative/Purulent - bacteria
Mucoid - thick, glue like, some bacteria

Front 26

Types of Otologic management of OM.

Back 26

Medication
Autoinflation
Myringotomy
ME air injection
Tympanostomy (pressure equalization)
Possible adenoidectomy or tonsillectomy

Front 27

Steps to Diagnosing OM

Back 27

Patient signs and symptoms/case history
Visual and otoscopic exam
Audiologic exam (immitance, pure tone, etc.)

Front 28

An infection/inflammation of the mastoid portion of the temporal bone is known as ____.

Back 28

Mastoiditis.

Front 29

Acute mastoiditis typically shows a _____ HL while chronic mastoiditis typically shows _____ HL.

Back 29

acute = conductive

chronic = mixed

Front 30

T/F TM perforations can spontaneosly heal.

Back 30

True.

Front 31

A perforation in the pars flaccida portion of the TM results in _____ HL, while a perforation in the pars tensa results in _____ HL.

Back 31

Pars flaccida = less HL,
Pars tensa = greater HL

Front 32

TM perforation may be due to the following:

Back 32

Barotrauma (change in pressure)
Acoustic trauma/explosions
Self inflicted injury
Vigorous nose blowing

Front 33

T/F TM perforation results in sensory hearing loss.

Back 33

False. Conductive hearing loss may take place.

Front 34

What is a myringoplasty?
What is a Tympnoplasty?

Back 34

Myringoplasty - procedure in which a tissue graft, usually of fascia or vein is used to close a perforation of TM
Tympanoplasty - reconstructive surgery of the ME

Front 35

T/F TM retraction is due to positive pressure formed in the cavity primary to ET dysfuncion.

Back 35

False. TM retraction is due to NEGATIVE pressure formed in the cavity SECONDARY to ET dysfunction.

Front 36

What are weakened portions of the TM which invaginate into the ME space called?

Back 36

TM retraction pockets.

Front 37

What is tympanosclerosis?

Back 37

A form of membrane thickening produced by formation of whitish calcification plaques on the TM and nodular deposits (dense connective tissue) in the mucosa of the ME.

Front 38

Tympanosclerosis may result in ______ HL, typically greater in the ____ frequencies.

Back 38

conductive, lower

Front 39

What is a cholesteatoma?

Back 39

A "pseudotumor", or keratoma, made up of accumulation of cellular debris/squamous epithelium/keratin.

Front 40

A perforation in the _____ _____ portion of the TM poses the most serious risk for developing a cholesteatoma.

Back 40

pars flaccida (attic perforations)

Front 41

T/F A cholesteatoma may be acquired or congenital.

Back 41

True.

Front 42

Moisture and bacteria may access the cholesteatoma resulting in:

Back 42

TM perforations and foul smelling otorrhea
Erosion of the ossicles and surrounding bones
Fistulas in the otic capsule/mastoid cell walls and resulting meningeal complications
Labyrinthine fistulas (holes) with sensory HL and vestibular problems
Erosion of facial nerve canal (VII) and resultant facial nerve problems (paralysis)

Front 43

T/F HL due to cholesteatomas may be conductive or sensory.

Back 43

True. It depends on the location of the cholesteatoma.

Front 44

What does congenital mean? How often does congenital HL take place?

Back 44

This means present at birth.
1/1000 births.

Front 45

What is the difference between aplasia and dysplasia?

Back 45

Aplasia - defective development or congenital absence of tissue or organ.
Dysplasia - abnormal tissue or organ development.

Front 46

Michel anamoly is ______ of the inner ear.

Back 46

Aplasia. AKA Michel's aplasia.

Front 47

Michel's anamoly occurs in __% of the profoundly deaf population. There is no _____ ear and in some cases no _____ nerve.

Back 47

1%
no INNER ear
VIII nerve

Front 48

What is Mondini Dysplasia?

Back 48

congenital anomaly of osseus and membranous labyrinth.

Front 49

What is Mondini Dysplasia characterized by?

Back 49

Aplastic cochlea (not there)
Deformity of the vestibule and semicircular canals
Partial or complete loss of auditory and vestibular function.

Front 50

What is the most common congenital inner ear anomaly?

Back 50

Scheibe dysplasia. 70% of cases.

Front 51

What is Scheibe dysplasia characterized by?

Back 51

cochleosaccular dysplasia
atrophy of the organ of corti
utricle and SSC are spared

Front 52

What percentage of Congenital SNHL is due to genetic factors?

Back 52

50-60%

Front 53

What are some non-genetic factors of congenital HL/deafness?

Back 53

Maternal infections
Anoxia
Ototoxins
Trauma
Prematurity/low birth weight

Front 54

What are some genetic factors that may cause congenital HL/deafness?

Back 54

chromosomal aberrations
mitochondrial defects/mutations
single gene defects/mutations
polygenic or multifactorial inheritance
(there are over 400 known causes)

Front 55

T/F Approximately 55% of genetic forms of HL are inherited in an autosomal RECESSIVE pattern.

Back 55

False. 70% are recessive.

Front 56

What percentage of genetic HL results from a dominant pattern of inheritance?

Back 56

15%

Front 57

What does syndromic refer to?

Back 57

A collection of associated abnormalities and symptoms.

Front 58

In the majority (60-70%) of genetic cases, HL is ________. (an isolated finding)

Back 58

non-syndromic

Front 59

An example of a genetic non-syndromic cause of HL is what?

Back 59

Connexin 26 (CX26)
"gap junction defect"
This is responsible for about 50% of congenital non-syndromic HL

Front 60

In CX26 _____ ions are not being recycled because the ___ _____ are not functioning properly. This results in HL.

Back 60

potassium ions
gap junctions

Front 61

T/F Certain mutations of the Cx26 gene occur in higher frequency in certain populations such as Caucasians.

Back 61

True.

Front 62

What are the clinical features of Cx26?

Back 62

Typically congenital
Mild to profound SNHL.
Can be progressive or non-progressive.

Front 63

What are some common forms of Autosomal Recessive Syndromic SNHL?

Back 63

Usher
Pendred
Jervell and Lange-Nielson

Front 64

What is the most common eye/ear disorder?

Back 64

Usher syndrome. 3.5/100 000 about 3-10% of all children with severe to profound SNHL

Front 65

What are clinical findings for Hearing Loss associated with Usher Syndrome?

Back 65

Bilateral SNHL
Moderate to profound
Typically congenital
Can be progressive

Front 66

What are the clinical findings for Visual Deficits associated with Usher Syndrome?

Back 66

Retinitis pigmentosa
Nightblindness
Tunnel vision
Onset early teen's to 20's
Visual deterioration to total blindness in adult life in about 50% of cases

Front 67

What are some common forms of Autosomal DOMINANT syndromic SNHL?

Back 67

Waardenburg Syndrome
Branchio-Oto-Renal Syndrome
Neurofibromatosis Type II
Stickler Syndrome
Epstein Syndrome
Treacher Collins

Front 68

T/F Waardenburg Syndrome always results in SNHL.

Back 68

False. Only HL in about 25% of cases.

Front 69

What are some characteristics of Waardenburg Syndrome?

Back 69

Partial albinism (white forelock)
Laterally positioned medial canthi
Different coloured eyes or bright blue eye colour.
Hearing loss may occur
-mild to profound
-unilateral or bilateral

Front 70

What is a type of X-linked syndrome?

Back 70

Alport Syndrome.

Front 71

T/F If a man has the X-linked form of Alport syndrome all of his daughters will also have it, but none of his sons will.

Back 71

True, since it is on the X chromosome (the father would pass on a Y to his sons and X to daughters)

Front 72

T/F All children born with genetic hearing loss are born to parents with normal hearing.

Back 72

False. Most are born to normal hearing parents but not ALL.

Front 73

T/F. The risk rate for SNHL is for every child.

Back 73

True.

Front 74

What are some selected congenital non-genetic SNHL?

Back 74

Viral Infections
- rubella
- CMV
- Herpes
- HIV
Protozoal and other organism infections
- toxoplasmosis
- syphilis
(TORCHS Syndromes)

Front 75

What does TORCHS stand for?

Back 75

TOxoplasmosis
Rubella
Cytomegalovirus (CMV)
Herpes Simplex Virus
congenital Syphilis

Front 76

T/F CMV is the largest and most complex member of the Herpes family of DNA viruses.

Back 76

True.

Front 77

CMV affects most cell types but has special impact on the following:

Back 77

Epithelial cells
Ependymal cells lining the ventricles
*Organ of Corti!!!!!
*Neurons of the VIII cranial nerve!!!!!!

Front 78

What percentage of people in the US will acquire CMV by age 40?

Back 78

50-85%

Front 79

How is CMV contracted?

Back 79

Person to Person contact
Transplants and transfusions
Mother to unborn child
Mother to newborn child
Respiratory (airborne - most common)
Saliva
Infected urine
Sexy time
Breast feeding

Front 80

What accounts for most congenital cases of CMV?

Back 80

The mother having a primary infection (first time) while pregnant.

Front 81

What is the incidence of congenital CMV?

Back 81

40 000 neonates a year.

Front 82

What are possible sequela go along with congenital CMV?

Back 82

Neurologic problems
Visual deficits
Physical development
Motor impairment
Seizure disorder
Developmental differences
Learning delays
Hearing loss

Front 83

What characteristics of HL go along with congenital CMV?

Back 83

SNHL
May be unilateral or bilateral
May be congenital or delayed onset
May be mild to profound
Frequently progressive
Unpredictable configuration

Front 84

T/F Asymptomatic CMV results in 2-7 times as many SNHL's as does symptomatic CMV.

Back 84

True.

Front 85

Congenital Asymptomatic CMV has to have virologic confirmation within what time frame?

Back 85

3 weeks.

Front 86

T/F CMV infection later in life is not as dangerous.

Back 86

True.

Front 87

Are we able to predict time of HL or rate or degree of progression in CMV?

Back 87

NO.

Front 88

How can congenital CMV be prevented?

Back 88

Practice "universal precaution" or good hygiene techniques during 1st pregnancy.