Term
|
Definition
mild to very profound
unilateral or bilateral
conductive, sensorineural, or mixed |
|
|
Term
profound hearing loss occurance |
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
chromosomal/ genetic (endogenous)
or nongenetic/ external factors (exogeneous) |
|
|
Term
|
Definition
caused by inflammatory disorders, toxicity, noise, accident, or injury that inflicts damage on the auditory system
remember: ex-external gen-genes |
|
|
Term
|
Definition
originate within the genetics of an individual.
transmitted from parents typically
remember: En=IN Gen=Genetics |
|
|
Term
|
Definition
|
|
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
|
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
|
|
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
|
Definition
a characteristic of Waardenburg's
an abnormally increased distance between two organs or parts
|
|
|
Term
|
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
|
|
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
|
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
|
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
|
Definition
congenital, bilateral, profound hearing loss and absent vestibular function |
|
|
Term
|
Definition
moderate, bilateral SNHL and normal vestibular function |
|
|
Term
|
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
|
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
|
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
|
|
Term
prevalence of Norrie syndrome |
|
Definition
|
|
Term
type and clinical charachteristics of Norrie syndrome |
|
Definition
Sex-linked
SNHL, congenital, or rapidly progressive blindness, pseudoglioma, opacification, and ocular degeneration. |
|
|
Term
|
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
|
Definition
characteristic of Norrie
the development of opacity (imperviousness to light rays), of the cornea or lens |
|
|
Term
|
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
|
Definition
characteristic of Alport Syndrome
inflammation of the kidney/s |
|
|
Term
|
Definition
Bilateral progressive SNHL |
|
|
Term
Autosomal dominant inheritance accounts for what percentage of genetic deafness? |
|
Definition
|
|
Term
With Autosomal dominant inheritance the risk of having a hearing impaired child is what %? |
|
Definition
|
|
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
|
|
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
|
|
Term
X-Linked accounts for what percent of genetic deafness? |
|
Definition
|
|
Term
Affected fathers produced carrier daughters and affected mothers... |
|
Definition
50% chance son will be affected and daughter will be a carrier. |
|
|
Term
|
Definition
very small or deformed pinna |
|
|
Term
Atresia (or Aural atresia) |
|
Definition
absence or closure of the external auditory meatus |
|
|
Term
|
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
|
Definition
|
|
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
|
Definition
inflammation of the middle ear without reference to cause
opaque or erythema tympanic membrance with no effusion (fluid) |
|
|
Term
|
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
|
Definition
liquid in the middle ear resulting from otitis media. |
|
|
Term
|
Definition
|
|
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
|
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
|
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
|
|
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
|
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
|
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
|
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
|
Definition
family of antibiotics that usually includes the mycin drugs.
ototoxic drugs |
|
|
Term
|
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
|
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
|
|
Term
all infants should be screened for hearing |
|
Definition
|
|
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
|
|
Term
Deafness:
cost of education |
|
Definition
|
|
Term
deafness:
cost of entitlements |
|
Definition
|
|
Term
|
Definition
communication, isolation, and lack of leisure activities |
|
|
Term
Deafness:
Educational cost |
|
Definition
graduate with 3rd-4th grade reading level |
|
|
Term
|
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
|
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
|
|
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
|
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
|
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
|
|
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
|
|
Term
medical complications stemming from otitis media |
|
Definition
cholesteatoma, perforations or retractions of tympanic membrane, tympanosclerosis, adhesive otitis media, and facial paralysis |
|
|
Term
|
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
|
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
|
Definition
aftereffect of chronic inflammation of middle ear cleft & mastoid
thinkening of membrane causes ossicle fixation, ossicular discontinuation, and hearing loss. |
|
|
Term
|
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
|
Definition
radical approach, is impairment of middle ear structure is involved
|
|
|
Term
|
Definition
refers to the destruction of connecting anatomic structures located more central to the structure that was initially destroyed |
|
|
Term
|
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
|
|
Term
|
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
|
Definition
severe to profound bilateral HL
configuration and degree vary
prevalence of HL: 35% |
|
|
Term
|
Definition
moderate to severe bilateral SNHL, may br progressive
prevalence of HL: 17% |
|
|
Term
|
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
|
Definition
moderate to severe unilateral or bilateral SNHL
prevalence of HL: 10% |
|
|
Term
|
Definition
most common consequence is HL
severe to profound SNHL 10%
another 16% experiencing transient conductive HL
a postnatal infection |
|
|
Term
|
Definition
one of the common cuases of unilateral SNHL
mild high-frequency impairment to profound
a postnatal infection |
|
|
Term
|
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
|
|
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
|
Definition
|
|
Term
those who do not exhibit a disorder |
|
Definition
|
|
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
|
|
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
|
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
|
|
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 |
|
|