Shared Flashcard Set

Details

Pathologies of the Auditory System
Exam Three Review
166
Audiology
Undergraduate 3
03/22/2011

Additional Audiology Flashcards

 


 

Cards

Term
Congenital hearing loss
Definition

mild to very profound

 

unilateral or bilateral

 

conductive, sensorineural, or mixed

Term
profound hearing loss occurance
Definition
1 in 1,000 live births
Term
mild/moderate occurance
Definition
3 in 1,000 live births
Term
Causes for hearing loss:
Definition

chromosomal/ genetic (endogenous)

 

or nongenetic/ external factors (exogeneous) 

Term
Exogenous
Definition

caused by inflammatory disorders, toxicity, noise, accident, or injury that inflicts damage on the auditory system

 

remember: ex-external gen-genes

Term
Endogenous
Definition

originate within the genetics of an individual.

 

transmitted from parents typically

 

remember: En=IN Gen=Genetics

Term
congenital
Definition
present at birth
Term
not all congenital disorders are hereditary, nor are all heredity disorders congenital...
Definition

child whose hearing mechanisms is damaged in utero by maternal rubella is born with hearing loss.

 

this is congenital but not hereditary. 

Term
autosomal dominant inheritance
Definition

1 parent passes the gene, not on the 'sex' gene

 

20% of cases of genetically caused (endogenous) deafness.

 

 

Term
Autosomal recessive inheritance
Definition

both parents pass the gene


parents are heterozygous, offspring homozygous or heterozygous. 


HOMO is more than a carrier (25% chance)


Accounts for as much as 80% of childhod deafness and most cases of genetic hearing loss

Term
X-linked inheritance
Definition

Sex linked. 


All daughters of trait carrying males are carriers

 

 Mothers with trait likely to pass on to son, making him demonstate characteristics of that gene.

type also called mother to son transmission. Similar to hemophilia gene.


2-3% of all cases of deafness. 

Term
Two categories of genetic deafness:
Definition

Non-syndromic

 

Syndromic

Term
occurance of Waardenburg Syndrome
Definition

1/4000

 

about 3% of childhood hearing loss

Term

waardenburg syndrome:

 

type of syndrome & charachteristics

Definition

autosomal dominance 


pigmentary animalies (white forelock, blue iries, partial albinism), cranofacial anomalies.


20-50% of suffers experience sensiorneural hearing loss

Term
hypertelorism
Definition

a characteristic of Waardenburg's

 

an abnormally increased distance between two organs or parts

 

 

Term
synophrys
Definition

a characteristic of Waardenburg's

 

a condition where the eyebrows grow together.

Term
Occurance of branchio-oto-renal syndrome
Definition
1/40,000 or 2% of children with profound hearing loss
Term
Type of and clinical characteristics of branchio-oto-renal syndrome
Definition

autosomal dominant

 

branchial abnormalities (ear pits and tags, cysts, fistulae) & renal abnormalities 

 

 

 

Term
Branchio-oto-renal syndrome's percentage exhibiting HL
Definition

75 % exhibit some kind of hearing loss

 

20% demonstrating conductive

20% SNHL

&

50% present with mixed HL

Term
prevalence of Treacher Collins Syndrome
Definition
Unknown
Term
type of disorder and clinical characteristics: Treacher Collins
Definition

autosomal dominant

 

craniofacial abnormalities (poorly developed malar bones, notching of eyelids, malformations of the external ear or canal, cleft palate)

Term
micrognathia
Definition

a characteristic of Treacher Collins

 

abnormally small mandible 

Term
Treacher Collins: types and percentage of HL
Definition
30% present with conductive HL, although SNHL may also be present
Term
Prevalence of Usher Syndrome
Definition

3.5/100,000 of childhood hearing loss

 

or about 10%

Term

Clinical characteristics & type of disorder:

Usher Syndrome

Definition

Autosomal Recessive

 

SNHL and retinitis pigmentosa

Term
Retinitis Pigmentosa
Definition

a characteristic of Usher's Syndrome

 

a group of disorders, often hereditary, that show progressive loss of retinal response, retinal atrophy, and clumping of the pigment with contraction of the field of vision.

Term

Ushers Sydrome

 

Type 1

Definition
congenital, bilateral, profound hearing loss and absent vestibular function
Term

Ushers Syndrome

 

Type 2

Definition
moderate, bilateral SNHL and normal vestibular function
Term

Ushers Syndrome

 

Type 3

Definition

progressive bilateral SNHL, variable vestibular dysfunction.

 

found primarily in the Norwegian population 

Term
Prevalence of Pendred Syndrome
Definition

Unknown but about 5% of congenital childhood hearing loss

 

 

Term

type of disorder & clinical characteristics:

Pendred Syndrome

Definition

Autosomal Recessive

 

Thyroid goiter and SNHL

Term
Pendred Syndrome & HL
Definition
Severe to profound hearing SNHL, and may be progressive
Term

Jervell and Lange-Neilson Syndrome

 

prevalence

Definition
Unknown, it is very rare.
Term

Type of disorder & clinical characteristics:

 

Jervell and Lange-Neilson Syndrome

Definition

Autosomal Recessive

 

SNHL and syncopal episodes

Term
syncopal episodes
Definition

a trait of Jervell and Lange-Neilson Syndrome

 

a syncope is a temporary suspension of consciousness caused by generalized cerebral ischemia.

Term
Jervell and Lange-Neilson Syndrome & HL
Definition
profound bilateral SNHL
Term
prevalence of Norrie syndrome
Definition
unknown
Term
type and clinical charachteristics of Norrie syndrome
Definition

Sex-linked

 

SNHL, congenital, or rapidly progressive blindness, pseudoglioma, opacification, and ocular degeneration.

Term
pseudoglioma
Definition

characteristic of Norrie

 

an inflammatory condition of the eye which resembles glioma of the retina and is marked by a circumscribed suppurative inflammation of the vitreous body

Term
opacification
Definition

characteristic of Norrie

 

the development of opacity (imperviousness to light rays), of the cornea or lens

Term
Norrie Syndrome & HL
Definition
One-third exhibit pregressive SNHL beginning in the 2nd or 3rd decade of life
Term
Prevalence of Alport Syndrome
Definition
Unknown, predilection for males.
Term
type and clinical characteristics of Alport
Definition

Sex-linked

 

SNHL and nephritis

Term
Nephritis
Definition

characteristic of Alport Syndrome

 

inflammation of the kidney/s

Term
Alport & HL
Definition
Bilateral progressive SNHL
Term
Autosomal dominant inheritance accounts for what percentage of genetic deafness?
Definition
20%
Term
With Autosomal dominant inheritance the risk of having a hearing impaired child is what %?
Definition
50%
Term

T or F:

 

With Autosomal dominant inheritance, the parent carrying the abnormal gene does not typically exhibit the trait.

Definition
False, the parent with the abnormal gene DOES typically exhibit the trait.
Term
Autosomal recessive accounts for what percentage of genetic deafness?
Definition
80%
Term
With autosomal recessive, there is a 50% chance  of having a heterozygous/ carrier child & a what percent chance of having a homozygous child.
Definition
25%
Term
X-Linked accounts for what percent of genetic deafness?
Definition
2-3%
Term
Affected fathers produced carrier daughters and affected mothers...
Definition
50% chance son will be affected and daughter will be a carrier.
Term
Microtia
Definition
very small or deformed pinna
Term
Atresia (or Aural atresia)
Definition
absence or closure of the external auditory meatus
Term
stenosis
Definition
opening to ear present but very small
Term
facial abnormalities (the 4 given)
Definition

skin tags

pre-auricular pits

pre-auricular pit with fistula

pre-auricular skin tags

Term

Complex craniofacial abnormalities:

 

external abnormalities s/t get complex but include marks that aid in diagnosis

Definition

hundreds of syndromes like this...

 

Most include conductive hearing loss, but some have SNHL as well.

Term
acquired external ear disorders
Definition

trauma,                otitis externa

fungus infections,       herpes infection

collapsed canal,      foreign bodies

wax impaction,       bony growth

Term
exotoses
Definition
bony growths
Term

many outer ear anomalies may also have middle ear implications.

 

Middle ear anomalies may include...

Definition

malformation of the ossicles or fusion of the ossicles.

 

Middle ear cavity may be absent or just have a slit like opening.

Term
otitis media
Definition

inflammation of the middle ear without reference to cause 

 

opaque or erythema tympanic membrance with no effusion (fluid)

Term
acute otitis media
Definition

rapid onset of signs and symptoms

(otalgia, otorrhea, fever, irritability)

 

TM is buldging, opaque, limited or no mobility to pneumatic otoscopy

 

an acute infection of the middle ear

 

 

Term
Otitis media with effusion (OME)
Definition

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


relatively asymptomatic, lacks symptoms of AOM


TM is opaque, retracted or buldging, can be serious enough to erode bone (ossicles, which may cause meningitis)

Term
Middle ear effusion
Definition
liquid in the middle ear resulting from otitis media.
Term
otorrhea
Definition
discharge from the ear
Term
Three types of middle ear fluid:
Definition

serous- thin & watery

mucoid- thinck & viscous; mucus like

purulent- puss like

Term
Most common complication from otitis media...
Definition

Hearing loss. 

 

Hearing sensitivity may range from normal to moderate HL @ 50 dB

Term
Treatment for otitis media
Definition

Antibiotics, in more serious cases placement of PE tubes

 

(pressure equalization)

Term
otosclerosis
Definition

build up of spongifying bone within the osseous labyrinth

 

bilateral more common than unilateral

 

genetic predisposition

Term
Occurance of otosclerosis
Definition

2.5 more common in women

(pregnancy can exacerbate the condition)

 

Occurs less frequently in Asians and African Americans

 

Onset usually b/w 20-40 years

Term

Otosclerosis, 

 

Audiologic manifestations

Definition

progressive conductive HL

 

Carhart notch (max @ 2000 Hz)

Term
Management of otosclerosis
Definition

most common approach is surgery to the stapes

 

surgical canidates usually exhibit an air-bone gap of at least 15 dB and have speech recognition scores of 60% or better

 

 

Term
Stapedectomy
Definition
removal of the stapes and a prothesis is substituted for the stapes
Term

fixation may occur with other ossicles besides the stapes

 

The ossicles may also be disarticulated

Definition
Term
hearing losses that occur at or central to the inner ear are called...
Definition
SNHL
Term
In SNHL which response(s) are abnormal?
Definition
Both the AC & BC responses are abnormal with SNHL
Term
Is SNHL a permanent condition?
Definition
Typically, SNHL is permanent in nature.
Term
How was the term sensioneural derived?
Definition

from the fact that in the cochlear pathology there is damage to the sensory structures within the cochlea.

 

This leads to neural degeneration of afferent fibers, which are connected the the hair cells

Term
One of the most common characteristics of SNHL.
Definition

Patients can HEAR speech but cannot understand it.

 

This is aside from profound HL of course.

Term
Loudness recruitment
Definition

With SNHL

 

defined as a more rapid groth loudness.

 

makes fitting with  a device more challenging 

Term

Persons with SNHL may have some speech abnormalities depending on severity and age of onset.

 

May include:

Definition
misarticulated speech, poor monitoring skills without hearing aid (i.e. inappropriate loudness levels)
Term
Causes of cochlear pathologies...
Definition

high risk factors (which may require newborn to be placed in NICU)


viral or bacterial disease, either pre or postnatal


Meniere's Disease, ototoxic drugs, presbycusis

Term
hyperbilirubinemia
Definition

jaundiced appearance

 

bilirubin exceeds 6 to 8 mg/dL

 

liver malfunciton

Term

Persistent pulminary hypertension in newborn 

 

(PPHN)

Definition

severe pulinary complications

 

hypoxemia (subnormal oxygenation of blood)

 

25x greater risk of HL than other NICU babies

Term
asphyxia
Definition

'severe depression at birth'

 

interruption of oxygen to the brain

 

damage can be to inner ear or auditory portions of the brain

Term
viral or bacterial disease
Definition

can be cause for cochlear and retrocochlear pathology


frequently transmitter to infant in utero


can be pre-natal, congenital, preinatal (from 28th week of gestation to 7 days after delivery), or post natal (later in life)

 

Term
high risk factors may include:
Definition

 

hyperbilirubinemia, persistent pulminary hypertension in newborn (PPHN), asphixia

Term
Postnatal diseases that affect hearing
Definition
mumps, measles, chicken pox, influenza, meningitis, Herpes zoster oticus
Term
Meniere's Disease: basic facts
Definition

affects membranous inner ear

 

low frequency SNHL 

 

may be unilateral or bilateral

Term
Classic symptoms of Meniere's Disease:
Definition

progressive fluctuant HL, episodic vertigo, tinnitus, fullness

 

typically a low frequency SNHL that fluctuates with 'episodes'

Term
aminoglycosis
Definition

family of antibiotics that usually includes the mycin drugs.

 

ototoxic drugs

Term
ototoxic drugs
Definition

Drugs that have a harmful effect on the organs/nerves concerned with hearing and balance

 

aminoglycoside antibiotics, which include the mycin drugs (ie streptomyicn)

Term
Factors to consider with ototoxic drugs:
Definition
dosage, patient susceptability, toxicity of agent, normalcy of kidney function
Term
Presbycusis
Definition

hearing loss due to aging


more predominant in men


begins around 40-50 years


Sloping HF SNHL

Term
retrocochlear pathologies refers to:
Definition

pathologies central to cochlea


usually involves some type of tumor but may include other CNS diseases (ie MS)


audiogram varies in degree


when HL is present it is typicalls SNHL

Term

common complaints of retrocochlear pathology:

 

(six)

Definition

tinnitus on 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

Term
babies born each year with hearing loss
Definition
12,000
Term
all infants should be screened for hearing
Definition
1993, NIH
Term
average age of hearing loss in children
Definition
2 ½ to 3 years and many not until 5 or 6 years 
Term
Language ability of a child is established by what age?
Definition
18 months, 80% of the time
Term

Deafness:

 

lost productivity cost

Definition
2.5 billion
Term

Deafness:

 

cost of education

Definition
121.8 billion
Term

deafness:

 

cost of entitlements

Definition
2 billions
Term

Social:

 

Personal cost...

Definition
communication, isolation, and lack of leisure activities
Term

Deafness:

 

Educational cost

Definition
graduate with 3rd-4th grade reading level
Term

Deafness:

 

Occupational

Definition

hard core unemployment:

 

30% unemployment rate

Term
5 points of value for a screening
Definition

-importance of disorder

-prevalence of disorder

-acceptance criteria for diagnosis

-available disgnostic and treatment facilities

-cost-effectiveness 

 

Term
Selecting an acceptable screening test:
Definition

Selected test should be the most sensitive or effective to detect condition set out to identify 

 

Good screening should be acceptable, reliable, valid, and cost-effective.

Term
Purpose of screening
Definition

to examine a population of individuals and identify those with a disorder vs. those without a disorder.

 

Every population includes both groups

(disordered and non-disordered)

Term
Within each group there exists two subgroups:
Definition

Disordered (includes true positives and false negatives)

 

Non-disordered (false positives and false negatives)

Term
Any screening test should be compared to...
Definition
a gold standard
Term
Success of a screening test depends on two factors:
Definition

sensitivity: proportion of the population with the disease who have a positive test

 

Specificity: the proportion of the population without the disease who have a negative test finding.

Term
prevalence
Definition
proportion of individuals in the population who have the disorder
Term
positive predicitve value
Definition
the probability of disease in a patient when the test is abnormal
Term
negative predicitve value
Definition
the probability of a patient not having the disease when the test is negative 
Term

Types of screening programs:

 

newborns

Definition
universal newborn hearing screening
Term
Myringitis
Definition
otitis media without effusion
Term
otitis media with effusion is the most prevalent disease in childhood
Definition
76-95% of children has a bout before the age of 6.
Term
groups prone to middle ear disease...
Definition
children who attend daycare, had a case before 18 months (issues with recurrency), cleft palate, Down's, inner city children, as are Native Americans
Term
muscle responsible for opening and closing Eustachian tube
Definition
tensor veli palatini
Term
type of hearing loss associated with otitis media 
Definition
usually conductive but sensorineural can also occur 
Term

average amount of HL with otitis media with effusion...

 

Can range from 0-50 dB HL but...

Definition

speech frequency range (500-2000Hz) is 25 dB HL.

 

bone conduction averages 3dB HL 

 

producing an air-bone gap of approx 22 dB

Term
sensorineural hearing loss usually stems from what in the case of otitis media?
Definition

COM

 

chronic otitis media

Term
medical complications stemming from otitis media
Definition
cholesteatoma, perforations or retractions of tympanic membrane, tympanosclerosis, adhesive otitis media, and facial paralysis
Term
cholesteatoma
Definition

keratinizing (to become callous like) of the epithelium and the accumation of shedding cellular debris within the middle ear 

 

or other portions of the temporal bone.

 

can be associated w/ AOM or COM

Term
perforation of the typanic membrane causes hearing loss as a result of
Definition
reducing the areal ratio between the membrane and oval window, and the direct coupling of sound waves to the round window
Term
tympanosclerosis
Definition

white shale like plaques on the tympanic membrane, deposits onto the ossicles.

 

usually with COM, from residual material.

 

stiffens chain, causes conductive HL at low frequencies

Term
adhesive otitis media
Definition

aftereffect of chronic inflammation of middle ear cleft & mastoid

 

thinkening of membrane causes ossicle fixation, ossicular discontinuation, and hearing loss.

Term
myingotomy
Definition

surgical procedure

 

an incision is made in an inferior quadrant in the tympanic membrane

 

this is used to remove or allow fluid to drain from middle ear space

 

disease must be persistant for at least 3 months prior

Term
tympanoplasty
Definition

radical approach, is impairment of middle ear structure is involved

 

 

Term
retrograde degeneration
Definition
refers to the destruction of connecting anatomic structures located more central to the structure that was initially destroyed
Term
electronystagmography
Definition

nonauditory special test, tests the vestibular system

 

may also performed by an audiologist

Term
high frequency sensorineural hearing loss suggests damage to...
Definition
basal portion of the cochlea
Term
low frequency sensorineural hearing loss to...
Definition
apical region 
Term
hisk risk register is...
Definition
a checklist of conditions known to exhibit a higher than normal prevalence of hearing loss.
Term
high risk register includes...
Definition
illness that cause newborns to be admitted to the NICU for 48 hours or greater, familiar history of hearing loss, infections in utero, craniofacial abnormalities, and stigmata or other findings associated with a syndrome known to cause any type of HL.
Term

many of the prenatal diseases are categorized as a part of ________.

 

Which stands for _____________.

Definition

(s)TORCH

 

Syphilis, toxoplasmosis, rubella, cytomepalovirus, and herpes simplex.

Term
syphilis & HL
Definition

severe to profound bilateral HL

 

configuration and degree vary

 

prevalence of HL: 35%

Term
toxoplasmosis and HL
Definition

moderate to severe bilateral SNHL, may br progressive

 

prevalence of HL: 17%

Term
rubella and HL
Definition

profound bilateral SNHL

 

cookie bite audiogram is common

 

may be progressive

 

prevalence of HL: 20-30%

Term
Cytomegalovirus (CMV) and HL
Definition

mild to profoud bilateral SNHL, may be progressive 

 

prevalence of HL: 17%

 

approx 20% die

Term
herpes simplex & HL
Definition

moderate to severe unilateral or bilateral SNHL

 

prevalence of HL: 10%

Term
bacterial meningitis
Definition

most common consequence is HL

 

severe to profound SNHL 10%

another 16% experiencing transient conductive HL

 

a postnatal infection

Term
mumps
Definition

one of the common cuases of unilateral SNHL

 

mild high-frequency impairment to profound

 

a postnatal infection

Term
herpes zoster oticus
Definition

burning pain near ear, loss is usually a bilateral high frequency HL

 

postnatal

Term
most often a tumor is involved, although this is not always the case (for example with multiple sclerosis)
Definition
retrocochlear pathology
Term
retrocochlear pathology that occurs at the first-order afferent nerve fibers typically produce...
Definition

high frequency SNHL

 

(either unilaterally or asymmetric between the two ears)

Term
no organic cause to the hearing loss
Definition
nonorganic hearing loss, pseudohypoacusis, functional hearing loss, psychogenic deafness
Term
who exhibits a disorder
Definition
A + C
Term
those who do not exhibit a disorder
Definition
B + D
Term

within the group with an auditory disorder,

 

there are two sub groups:

Definition

1. those with the disorder who test positive

(true positives) A

 

2. those with the disorder who test negative

(false negatives) C

Term

Within the group without an auditory disorder,

 

there are two subgroubs:

Definition

1. those without the disorder who test positive

(false positive) B

 

2. those without the disorder who test negative

(true negatives) D

Term
the results of the gold standard are ...
Definition
universally accepted as proof that the disease or disorder is either present or absent.
Term
In the case of hearing loss, what serves as the gold standard for typical screening...  
Definition
pure-tone audiometry
Term
In the case of hearing loss, what serves as the gold standard for screening for middle ear disease... 
Definition
usually pneumatic otoscopy or electroacoustic immittance
Term

Gold Standard Equation:

 

Sensitivity

Definition

sensitivity

100* [A/(A+C)]

 

100*

[true positives/

(true positives+ false negatives)]

Term
specificity refers to 
Definition
proportion of individuals without the characteristic correctly identified by the test
Term

Gold Standard Equation:

 

Specificity

Definition

100* [D/(B+D)]

 

100*

[true negative/

(false positives + true negatives)]

Term
the best screening tool is one that provides the highest degree of ___________ and ____________.
Definition

sensitivity & specificity

 

Term

Gold Standard Equation:

 

Prevalence

Definition

100*

[(True positive+false negatives)/

(A+B+C+D)]

 

A+B+C+D = total population

Term
positive predictive value
Definition
the probability of an individual having the disorder when the test is positive
Term
negative predictive value
Definition
the probability of not having the disorder when the test is negative
Term
unlike sensitivity and specifity, predictive values are________________ on the prevalence of the disorder in the population being tested.
Definition
 strongly dependent
Term

advantages of ABR for newborn screenings

 

(there are three main ones) 

Definition

A. use of less intense stimuli, detects milderforms of HL

 

B. ability to detect both unilater & bilateral

 

C. physiologic measurement that depends on sensory response.

Term
limitations to ABR in infant screenings
Definition

A. cost & sophistication of instrumentation

 

B. use of an acoustic click

 

C. presence of ABR doesn't mean they can hear

Supporting users have an ad free experience!