Audiology Test 3- Ear Pathologies

Front 1

Occurs when the abnormal gene is one of the ordinary paired chromosomes. That is, it is not one of the sex chromosomes.

Back 1

autosomal dominant inheritance

Front 2

with autosomal dominant inheritance only necessary for ______ parent to have the abnormal gene.

Back 2

one

Front 3

What percent of genetic deafness is autosomal dominant inheritance?

Back 3

20% of genetic deafness

Front 4

With autosomal dominant inheritance, the risk of having a hearing impaired child is ____%.

Back 4

50

Front 5

The parent with the abnormal gene __________________ exhibits the trait.

Back 5

frequently

Front 6

Occurs when each apparently normal parent carries one abnormal gene for the condition.

Back 6

Autosomal recessive inheritance

Front 7

With autosomal recessive inheritance Parents are ________________ (only one abnormal gene) and are carriers for the trait.

Back 7

heterozygous

Front 8

Autosomal recessive inheritance accounts for ______% of genetic deafness

Back 8

80

Front 9

Which type of inheritance is more common?

Back 9

Autosomal recessive inheritance

Front 10

Why is autosomal recessive inheritance more problematic?

Back 10

It’s not only passed on from generation to generation, but it skips generations as well

Front 11

Autosomal recessive inheritance hearing loss is caused when parent carries how many abnormal genes?

Back 11

one

Front 12

With autosomal recessive inheritance there is a____% probability of having a child who is homozygous (2 abnormal genes) and expresses the trait.

Back 12

25

Front 13

With autosomal recessive inheritance there is a ____% probability of having a child who is heterozygous and is a carrier

Back 13

50

Front 14

Inheritance that does incorporate the sex linked chromosomes

Back 14

X-linked (sex linked) inheritance

Front 15

X-Linked inheritance is a much less frequent occurrence, ______%

Back 15

2-3

Front 16

Gene locus determining this x-linked inheritance is on the ___ chromosome (sex chromosome)

Back 16

X

Front 17

Males have ____________ chromosomes while females have ______________ chromosomes

Back 17

1 X and 1 Y
2 X

Front 18

Affected fathers produce daughters who are _____________

Back 18

carriers

Front 19

If mother is a carrier there is a _____% probability that each son will be affected and a ____% probability that each daughter will be a carrier.

Back 19

50, 50

Front 20

Genetic Hearing losses can be _____________ or ______________

Back 20

nonsyndromic or syndromic

Front 21

Is nonsyndromic or syndromic genetic hearing loss more common?

Back 21

nonsyndromic

Front 22

Non syndromic accounts for ______ of all hearing losses

Back 22

2/3

Front 23

Connexin 26 hearing loss accounts for ______ of non syndromic hearing loss

Back 23

1/3

Front 24

_______________ hearing loss accounts for 1/3 of non syndromic hearing loss

Back 24

Connexin 26

Front 25

a protein important for hearing that is instructed by the GJB2 (Gap Junction Beta 2) gene

Back 25

Connexin 26

Front 26

When there’s a mutation in this GJB2 gene, it affects this protein which is necessary for __________ hearing

Back 26

normal

Front 27

_____ of non syndromic hearing losses are a result of Connexin 26 and a mutation in GJB2

Back 27

1/3

Front 28

Genetic hearing losses that are associated with anomalies other than hearing loss

Back 28

Syndromic

Front 29

There are approx _____ syndromes that are associated with hearing loss

Back 29

400

Front 30

very small or deformed pinna
(Anomalies of the External Ear)

Back 30

Microtia

Front 31

absence or abnormal closure of the external auditory meatus (ear canal).
(Anomalies of the External Ear)

Back 31

Atresia or Aural Atresia

Front 32

opening present but very narrow.
(Anomalies of the External Ear)

Back 32

Stenosis

Front 33

What are some examples of facial anomalies?

Back 33

Skin tags
Pre-auricular pits
Pre-auricular pit with fistula
Pre-auricular skin tags

Front 34

External ear anomalies that are complex

Back 34

Complex Craniofacial Anomalies

Front 35

External ear anomalies sometimes get quite complex and include several _______________.

Back 35

stigmata

Front 36

physical or mental marks that aid in diagnosis by indicating that a person has a particular syndrome

Back 36

stigmata

Front 37

There are hundreds of syndromes that contain complex craniofacial anomolies. Most of these include ______________ hearing loss (HL), but some have _______________ HL.

Back 37

conductive, sensorineural

Front 38

hearing loss present at birth

Back 38

congential hearing loss

Front 39

True or False? Congenital Hearing Loss can vary in its degree from mild to profound, may be unilateral or bilateral, and can be sensorineural, mixed or conductive

Back 39

True

Front 40

What do we use to determine the nature of a congenital hearing loss?

Back 40

case history

Front 41

T or F. Overall incidence of congenital hearing loss is unknown.

Back 41

True

Front 42

Why don't we know the overall incidence of congenital hearing loss?

Back 42

The more mild forms of hearing loss will go undetected. We are getting a better idea in recent years due to ABR & autoacoustic immitance.

Front 43

What is the prevalence of profound congenital hearing loss?

Back 43

1 per 1000

Front 44

Approx. ____ per ________ babies are born with some degree of hearing loss at birth

Back 44

2 to 3 per 1000

Front 45

Is congenital hearing loss hereditary?

Back 45

It can be hereditary or non hereditary. (There can be some genetic predisposition)

Front 46

A hearing loss that is hereditary in nature is also known as ______________________________________

Back 46

genetic or endogenous hearing loss

Front 47

A hearing loss that is non hereditary in nature is also known as __________________________________

Back 47

non genetic or exogenous hearing loss

Front 48

Many outer ear anomalies may also have _________ ear implications.

Back 48

middle

Front 49

Middle ear anomalies may include _________________ or ________________ of the ossicles.

Back 49

malformation or fusion

Front 50

_________________________ may be absent or just have a slit-like opening.

(Anomalies of the middle ear)

Back 50

Middle ear cavity

Front 51

What are some acquired external ear disorders?

Back 51

Trauma
Otitis externa – viral or bacterial
Fungus Infections
Herpes Infection
Collapsed canal – define and note condition, may result in signif. Conductive hearing loss
Foreign bodies
Wax impaction
Bony growths (exotoses)

Front 52

inflammation of the middle ear without reference to cause or pathogenesis

Back 52

otitis media

Front 53

rapid onset of signs and symptoms, e.g. otalgia and fever, of acute infection of middle ear

Back 53

acute otitis media

Front 54

inflammation of the middle ear with a collection of liquid in middle ear space

Back 54

Otitis Media with Effusion (OME)

Front 55

liquid in middle ear resulting from otitis media

Back 55

Middle ear effusion (MEE)

Front 56

Liquid in middle ear that is thin & watery

Back 56

serous

Front 57

Liquid in middle ear that is thick & viscous; mucus-like

Back 57

mucoid

Front 58

Liquid in middle ear that is puss-like

Back 58

purulent

Front 59

discharge from the ear

Back 59

otorrhea

Front 60

opaque or erythema tympanic membrane (TM) with no effusion.

Back 60

Otitis Media without effusion

Front 61

rapid, short onset of signs & symptoms: otalgia, otorrhea, fever, irritability, anorexia, vomiting, diarrhea; TM is bulging, opaque and has limited or no mobility to pneumatic otoscopy.

Back 61

Acute Otitis Media (AOM)

Front 62

patient relatively asymptomatic; lacks signs/symptoms of AOM.

Back 62

Otitis Media with effusion (OME)

Front 63

TM is opaque, retracted or bulging
Fluid level present is middle ear space
Can be serious enough to erode bone (ossicles, tegmen typmpani – which may cause meningitis)

Back 63

Otitis Media with effusion (OME)

Front 64

___________________ is the most common complication from otitis media.

Back 64

Hearing loss

Front 65

Hearing sensitivity as a result of otitis media may range from ______________ to _____________.

Back 65

normal to moderate (50 dB) HL

Front 66

What is treatment for hearing loss as a result of otitis media?

Back 66

Antibiotics and placement of PE (pressure equalization) tubes.

Front 67

Build up of spongifying bone on the osseous labyrinth

Back 67

Otosclerosis

Front 68

Otosclerosis is _____ times more common in women and may be exacerbated during pregnancy.

Back 68

2.5

Front 69

Otosclerosis occurs less frequently in ___________________ and _________________.

Back 69

African Americans and Asians

Front 70

The onset of otosclerosis is usually between age ____ – ____.

Back 70

20-40

Front 71

Is bilateral or unilateral otosclerosis more common?

Back 71

bilateral

Front 72

Does otosclerosis have a genetic predisposition?

Back 72

Yes

Front 73

What are some audiologic manifestations for otosclerosis?

Back 73

progressive conductive HL
Carhart Notch (max. @ 2000 Hz)

Front 74

How is otosclerosis most commonly treated?

Back 74

Most common approach is surgery to the stapes.

Front 75

_______________ is the removal of the stapes and a prosthesis is substituted for the stapes.

Back 75

Stapendectomy

Front 76

What are some other ossicular disorders?

Back 76

Fixation may occur with other ossicles besides the stapes
The ossicles may be disarticulated.

Front 77

Hearing losses that occur at or central to the inner ear are called ________________ hearing losses (SN HL).

Back 77

sensorineural

Front 78

Hearing losses that occur at or central to the ___________ are called sensorineural hearing losses (SN HL).

Back 78

inner ear

Front 79

So, in Sensorineural Hearing Loss ________________ responses are abnormal.

Back 79

AC and BC

Front 80

Is sensorineural hearing loss permanent or reversible?

Back 80

permanent

Front 81

The term Sensorineural is derived from the fact that in cochlear pathology there is damage to the _______________________ within the cochlea.

Back 81

sensory structures

Front 82

This destruction of the sensory structures within the cochlea eventually leads to some neural degeneration of _____________ nerve fibers, which are connected to the hair cells.

Back 82

afferent

Front 83

This destruction of the sensory structures within the cochlea eventually leads to some neural degeneration of afferent nerve fibers, which are connected to the _________________.

Back 83

hair cells

Front 84

What is one of the most common characteristics of patients with SN HL?

Back 84

they hear speech, but cannot understand it (except for profound HL).

Front 85

defined as a more rapid growth in loudness in the ear with cochlear pathology than for the normal hearing ear.

Back 85

Loudness recruitment

Front 86

Loudness recruitment presents problems when trying to fit patient with a ________________________

Back 86

hearing aid

Front 87

Persons with Sensorineural Hearing Loss may have some ________________ abnormalities depending on severity and age of onset

Back 87

speech

Front 88

What are some speech abnormalities that may result from sensorineural hearing loss?

Back 88

Misarticulated speech, poor monitoring skills without hearing aid (i.e. may speak at levels that are inappropriately loud).

Front 89

When a cochlear pathology is caused by an infectious disease that is transmitted to infant by mother in utero. (Occuring during birth or from 28th week of gestation to 7 days after delivery)

Cause of Cochlear Pathologies by a Viral or bacterial disease

Back 89

pre-natal, congenital, or perinatal cochlear pathology

Front 90

When a cochlear pathology caused by a viral or bacterial disease occurs later in life


Cause of Cochlear Pathologies by a Viral or bacterial disease

Back 90

Postnatal cochlear pathology

Front 91

Many prenatal diseases comprise the ___________ complex, an acronym for these diseases.

Back 91

(S)TORCH
****** look up storch complex in the book

Front 92

Any disease in (S)TORCH complex is considered high risk for ____________________.

Back 92

hearing loss

Front 93

What are some examples of post natal diseases that may cause hearing loss?

Back 93

Mumps
Measles
Chicken Pox
Influenza
Meningitis
Herpes Zoster Oticus

Front 94

A cochlear pathology that is caused by a buildup of fluid (endolymph) in membranous part of inner ear

Back 94

Meniere's Disease

Front 95

What are some symptoms of meniere's disease?

Back 95

Progressive fluctuant HL; episodic vertigo; tinnitus (in a classic example of meniere’s disease tinnitus is a roaring that sounds like an ocean); fullness (ears feel full of pressure)

Front 96

Meniere's disease is classified by low frequency _______________________________ hearing loss

Back 96

sensorineural

Front 97

Is Meniere's disease unilateral or bilateral?

Back 97

May be unilateral or bilateral

Front 98

What are some of the treatment options for Meniere's disease?

Back 98

low salt diet or diuretic to help remove excess fluid from the body; no smoking to help with vertigo; common surgical procedure= endolymphatic sac decompression/endolymphatic shunt
Vestibular nuerectomy- cutting of the vestibular nerve to help control the vertigo
Labyrinthectomy- removal of the inner ear. Vestibular part no longer a problem, but you are deaf. Reserve for “burned out” ears that have been so badly damaged and are barely useable.

Front 99

Aminoglycosides (family of antibiotics that usually includes the mycin drugs) drugs that are usually given to people that are extremely ill, but these medications can cause permanent hearing loss

Back 99

Ototoxic Drugs

Front 100

T or F Person taking ototoxic drugs must be monitored closely so that if the drug begins to alter hearing, doctors can adjust the dosage of the medication

Back 100

True

Front 101

What are some factors to consider with ototoxic drugs (that will determine if hearing loss occurs and how much occurs)?

Back 101

dosage
patient susceptibility
toxicity of agent
normalcy of kidney function (kidneys rid the body of the drugs)

Front 102

Hearing loss due to the aging process

Back 102

Presbycusis

Front 103

Presbycusis Begins around__________ years

Back 103

40-50

Front 104

Is Presbycusis greater for men or women?

Back 104

Men-men tend to work in more noisier environments than women

Front 105

Presbycusis is a sloping HF __________________HL

Back 105

sensorineural

Front 106

occurs as a result of exposure to extreme levels of noise; may experience a temporary threshold shift which later returns to normal; eventually you can end up with a permanent threshold shift

Back 106

Noise-induced hearing loss

Front 107

usually is an accumulation of noise that causes hearing loss over the years

Back 107

Acoustic trauma

Front 108

refers to pathologies central to cochlea

Back 108

retrocochlear pathology

Front 109

A retrocochlear pathology usually involves some type of ____________ but may include other CNS disease (e.g. MS)

Back 109

tumor

Front 110

Retrocochlear pathology deals with the ___________ and _____________________________________

Back 110

acoustic nerve and structures within the central auditory nervous

Front 111

When hearing loss exists with retrocochlear pathology it is usually ___________ hearing loss

Back 111

usually HF SN

Front 112

What are some common complaints with retrocochlear pathology?

Back 112

Tinnitus affected side
dysequilibrium or gait problems
WR varies from normal to poor
Acoustic reflexes usually absent or elevated when affected ear stimulated
Acoustic reflex decay – positive
ABR abnormal

Front 113

hearing loss that does not have an organic basis

Back 113

nonorganic hearing loss