CSD 724 Quiz #1: Pathologies

darwin’s tubercle

a thickening of the helix or small bump on pinna

not bad or detrimental

anotia

absence of the pinna or auricle

can be completely gone or have a small cartilaginous portion remaining

also grade 4 microtia

microtia

abnormally small or deformed pinna (congenital)

grade 1 = smaller than norma1

grade 2 = part of the ear looks normal

grade 3 = just a small remnant of skin and cartilage

grade 4 = complete absence (aka anotia)

basal cell carcinoma

skin cancer on the pinna

atresia

absent external auditory meatus (ear canal)

often accompanied by abnormalities of the middle ear bones and external ear

stenosis

narrowing of the external auditory canal (EAC)

collapsing ear canal

seen more in males, particularly elderly since they have more flaccid cartilaginous portion of their ears

cerumen impaction

blockage of the ear canal by ear wax

there is great variation in color & consistency

external otitis

infection that occurs in the skin of the external auditory canal (EAC)

aka swimmers ear

fungal or bacterial

can be accompanied by otalgia (ear pain) and/or otorrhea (drainage from the ear)

osteama

benign bony tumor within the ear canal

usually one singular mass

exostoses

inward projection of bone

can be one or multiple

caused by prolonged exposure to cold water (ex: surfers)

tympanosclerosis

thickened or scarred tympanic membrane

calcium plaques or deposits on the eardrum

usually seen with ruptured ear drums

perforations of the TM

any hole in the tympanic membrane

can be caused by foreign object, trauma, or infection

can vary in size from pin-hole to a total perforation of the TM

bullous myringitis

blister on the eardrum

eustachian tube dysfunction

negative middle ear pressure

can cause retraction of the tympanic membrane

TM is being sucked in and “wraps” around the ossicles

patulous eustachian tube

eustachian tube that is chronically patent (open), which results in atophony

more observed in females, especially during/after pregnancy

otitis media

infection of the middle ear

acute otitis media

rapid onset and short duration

chronic otitis media

slow onset and of long duration

serous otitis media

thin & watery

can see resolving air bubbles sometimes

adhesive otitis media

aka glue ear

thick & mucous like

tympanostomy tube

pressure equalization (PE) tubes are a common treatment for chronic OM cases

cholesteatoma

middle ear pseudotumor

a sac of keratin mixed with squamous epithelium and fats (skin & fat growth)

more likely in chronic TM perforations

white cheesy appearance (can fill a part of middle ear cavity through a perforation)

otosclerosis

growth of spongy bone usually over the stapedial footplate

causes fixation of the stapes, which in turn cannot push the oval window very well

genetic

ossicular chain discontinuity

disruption in the connection anywhere along the ossicular chain

common in head trauma

Genetic hearing loss

Genetic factors account for 50-60% of moderate-profound SNHL present at birth/early childhood

Hereditary deafness occurs in 1-3 out of 1000 live child births

most common gene is GJB2 (autosomal recessive)

non-syndromic hearing loss/deafness

2/3 of the time

absence of other medical or physcial characteristics

syndromic hearing loss/deafness

1/3 of the time

presence of other medical or physical characteristics (ex: alport syndrome, Down syndrome, pendred syndrome…)

pendred syndrome

goiter on neck or large bulge

waardenburg’s syndrome

white forlock and a wider set of eyes

inheritance patterns

autosomal dominant and recessive

x-linked

mitochondrial (from the mother)

inner ear malformations (aplasia)

failure of the inner ear to reach full development

michel aplasia

complete absence of the inner ear and auditory nerve

mondini aplasia

incomplete development or malformation of the inner ear

schibe aplasia

membranous degeneration of the inner ear ex:(Scala vestibuli, tympani, or media)

rH factor

rH negative mom builds up antibodies which attack and damage an rH positive fetus’ red blood cells (RBC)

the baby’s damaged RBCs form bilirubin, which builds up over time in cochlea and in turn causes hearing loss

rubella

viral infection aka “german measles”

highest risk in earlier stages of pregnancy

70-90% of infants will have SNHL (if the parents had rubella)

not as common anymore due to MMR vaccines

cytomegalovirus (CMV)

many people carry this without knowing and is asymptomatic

really only risky when mother has it during pregnancy

one of the leading causes of congential non-hereditary SNHL

toxoplasmosis

parasite found in red meat and cat feces (pregnant women should stay away)

most affected adults will be symptom free

hearing loss can be progressive

anoxia

lack of oxygen during the birthing process

alters cell metabolism and results in damage to cochlea and CN

can be from premature separation of placenta or compression of the umbilical cord

premature babies are more likely to have this

prematurity

at higher risk for hearing loss, not a direct cause!

could be from oxygen deprivation or loud incubator noise

ototoxic drugs

medications that are poisonous to the ear

ex: high levels of aspirin, diuretics, mycins, chemotherapy

radiation induced hearing loss

radiation that is used for neck tumors and cancers can affect auditory structures

usually a conductive hearing loss

1/3 of patients with radiotherapy have cochlear damage for permanent SNHL

Mumps

swelling of the parotid and salivary glands

a virus

80% are unilateral

meningitis

infection of the meninges

usually bacterial, but can be viral

bilateral

noise-induced hearing loss

acoustic trauma

due to continuous noise or impact

affects high frequencies first (3000-6000 Hz)

There are temporary and permanent threshold shifts

Meniere’s disease

endolymphatic hydrops

endolymph is over-secreted or under-absorbed causing pressure on hair cells

causes fluctuating hearing loss and tinnitus

ear fullness, vertigo, nausea

affects apical (low freq end) first

idiopathic sudden SNHL

sudden loss of hearing with unknown cause

often unilateral

may be attributed to viral infection, vascular disruption, autoimmune, or membrane rupture

should be treated as a medical emergency, as medication can often help restore hearing

presbycusis

hearing loss due to age

affects high frequencies more than low

WRS score can be affected, making speech in noise hard to hear (tv, restaurant)

semicircular canal dehiscence syndrome

thinning or weakening of the bone that covers the superior SCC (semicircle canal)

causes dizziness, vertigo, disequilibrium

may have compant of patulous ET

barotrauma

trauma caused by sudden changes in pressure (ex: diving, flying)

can cause middle ear trauma-TM rupture

can rupture round or oval window leading to perilymph fistula (or leak)

can produce mild to profound hearing loss

auditory neuropathy

nerves dying, progressive over time

sounds enter ear normally, but there is a problem with transmission of signals to the brain

often can hear something but can’t say what it was

wide range of hearing loss

acoustic neuroma

benign 8th nerve cranial nerve tumor

often grows in auditory canal

also called vestibular schwannoma is vestibular portion of the nerve is affected

most frequently a unilateral high frequency SNHL

symptoms happen due to the pressure the tumor puts on surrounding areas

auditory processing disorder

all auditory structures of the ear are intact and function normally, but there is a problem somewhere in the central auditory system

not enough research is done