Auditory Pathologies/Disorders

outer ear pathologies

these affect the pinna and the ear canal leading up to the eardrum

includes cerumen, stenosis, acute external otitis, carcinoma, trauma, periochondritis, staff infections

cerumen

also known as ear wax

caused by q-tip use (which pushes wax further in the ear canal to the eardrum), hearing aids, and oddly shaped ear canal

more common in older people, especially men

stenosis

means small ear canal

very narrow/small ear canals, easy for wax or water to get trapped

acute external otitis

known as swimmer’s ear

any water source that stagnates in the ear canal can cause inflammation

VERY painful

that wax has built up for a long time

what could dark wax mean?

earwax

consists of sweat secretions, dead skin cells, and hair

carcinoma

skin cancer that can present on the outer ear

includes basal cell, squamous cell, and melanoma

basal cell carcinoma

most common form of skin cancer, often found on head, neck, and arms

squamous cell carcinoma

second most common form of skin cancer, often appears as red bump, scaly patch, or sore that doesn’t heal

melanoma

deadliest form of skin cancer, can spread quickly to other body parts if not diagnosed early

trauma

can be from frostbite, rugby, football, wrestling, piercings

includes cauliflower ear

periochondritis

is infection and inflammation of the perichondrium of auricular cartilage/red, swelling, hot, tender outer ear that can lead to serious complications

causes include infection of haematoma auris, complication of severe otitis externa, laceration, mastoid surgery, and high ear piercing

the pinna is uniformly enlarged and thickened and its surface is red and shiny with severe pain and tenderness

outer ear malformations

includes atresia, microtia, cysts/pits/tags, and goldenhar syndrome

atresia

little or no ear formation, NO ear canal

can be unilateral or bilateral

can be syndromic (treacher-collins) or an isolated finding

microtia

very small ear; may or may not have open ear canal (ear canal is still present unlike atresia)

often low set, or rotated toward back of head

unilateral or bilateral

can by syndromic (goldenhar syndrome) or isolated

cysts/pits/tags

congenital and representative of error in ear formation

pits can weep fluid from middle ear, easily infected

goldenhar syndrome

rare congenital disorder that causes craniofacial abnormalities typically affected eyes, ears, and spine

underdeveloped bones and muscles on one side of the face

microtia typically seen with this syndrome

otalagia

means ear pain

can come from different places, not necessarily the ear

jaw pain from grinding teeth, incoming molars (TMJ)

otorrhea

foul odor emanating from the ear

could be a fungal infection, middle ear infection, or foreign object

occluding

blocked ear

(can be from cerumen or foreign objects)

non-occluding

not blocked ear

middle ear pathologies

these stop sound from moving clearly to the inner ear

includes otitis media, tympanic membrane perforation, cholesteatoma, mastoiditis, otosclerosis, malleus fixation, ossicular discontinuity, trauma, eustachian tube dysfunction, and glomus tumor

otitis media

also known as an ear infection, is the most common middle ear pathology; especially in children

serous otitis media

ear infection where effusion (build up of fluid behind ear drum) is thin, clear

mucoid otitis media

ear infection where effusion is thicker and yellowish

suppurative otitis media

ear infection where effusion is thick with yellow pus

adhesive otitis media

ear infection where thickening of eardrum causes deep retraction

acute otitis media

ear infection where there is quick onset, red, inflamed, and painful

recurrent otitis media

getting an ear infection more than 3 times in 6 months

this is when doctors decide to put tubes in eardrum

chronic otitis media

ear infection lasting more than 8 weeks

persistent otitis media

ear infection lasting more than 6 weeks with treatment

comes back even with antibiotics

tympanic membrane perforation

means hole in eardrum

can be from foreign objects, a blow to the head, barotrauma (scuba diving/flying), and otitis media

cholesteatoma

benign growth filled with skin cells/debris

ETD, retracted eardrum, allergies, URI

mastoiditis

infectious invasion of the porous mastoid bone (bone behind ear and fluid fills the holes in the bone)

from streptococcus pneumoniae or untreated middle ear infection (this is why tubes are important)

otosclerosis

fixation of the ossicular chain (connection between the malleus, incus, and stapes)

often a genetic disorder appearing in mid ‘40s, mostly in women

can get a surgery that removes bony growth and parts of the ossicles to make bones move

ankylosis

fixation of stapes only

malleus fixation

cannot move

loss of sound transmission

genetic or from chronic ear infections

eustachian tube dysfunction

this is when pressure is not being equalized

from allergies, asthma, respiratory disorders, anatomical shape and size, enlarged adenoids, and changes in air pressure

glomus tumor

a tumor that is not malignant, is very slow growing, and can disrupt the ossicular chain (needs surgical removal)

patients complain that they can hear their heartbeat in their ear

eustachian tube

connects the middle ear to the back of the nose and upper throat, it helps equalize pressure, drains fluid, and protects the middle ear

is normally closed and opens during actions that activate the muscles of the soft palate

tips to help support its health is swallowing, yawning, or chewing gum, during flights, swallow or yawn during takeoff and landing, manage colds, allergies, and sinus congestion, and avoid smoke and environmental irritants

inner ear disorders

disorders that affect your balance and hearing

includes vestibular schwannoma, neurofibromatosis, facial nerve disorders, diabetes, meniere’s disease, noise induced hearing loss, presbycusis, trauma, and ototoxicity

vestibular schwannoma

also called an acoustic neuroma

usually unilateral slow growing tumor of VIII nerve

very poor speech discrimination compared to thresholds (ear with loss may not be able to repeat speech)

patients report full feeling in the ear and dizziness is also common

neurofibromatosis

NF1 rarely involves the ear

NF2 faster growing acoustic tumors (bilateral)

usually sudden onset of hearing loss/deafness

removal of tumors severs VIII nerve, complete deafness

cochlear neuritis

inflammation of VIII nerve

facial nerve disorders

bell’s palsy (most common)

test finding: ipsilateral acoustic reflex absent with normal hearing

can cause hearing loss and facial numbness (which is why it’s inner ear)

diabetes

in children and adults

progressive bilateral sensorineural hearing loss

meniere’s disease

usually seen as hearing loss, vertigo, and tinnitus

fullness in the ear

hearing loss can FLUCTUATE

often a “dumping ground” for unknown etiologies

fluctuating low frequency hearing loss mostly in one ear (symptoms can come some days and other days not)

noise induced hearing loss

repeated exposure leads to progressive loss

single episode > 140 dB HL can cause deafness

classic “noise notch” in hearing test

wear hearing protection to prevent loss

presbycusis

aging of the ear

can involve degradation of cochlear structures and/or central auditory cell structures

typically, very poor speech recognition compared to hearing tests results

isolation can cause hearing degradation (lack of communication and hearing speech)

noise notch

half

after increasing 3 dBs on continuous dB, permissible exposure time decreases by how much?

trauma

longitudinal fracture usually involves middle/outer ear systems (often repairable) like being in a car accident

transverse fractures typically involve inner ear systems (non repairable)

ototoxicity

drugs that are toxic for the ear

“mycin” drugs: aminoglycosides

anti-inflammatory (aspirin, blood thinners)

chemotherapy drugs

quinine (malaria)

central pathologies

include multiple sclerosis, disrupted blood supplies, and hereditary motor sensory neuropathies (HMSN)

multiple sclerosis

demyelination of nerves

plaque adhesions at the brainstem

can hit balance nerve

disrupted blood supplies

TIAs (transient ischemic attacks)

arteriosclerosis (hardening of the arteries)

aneurysm (burst blood vessel)

hereditary motor sensory neuropathies (HMSN)

charcot-marie-tooth (mutation on the X chromosome)

friedreich’s ataxia (mutation of the FXN gene that produces frataxin protein)

voluntary movement (affects balance)

auditory neuropathy spectrum disorder

wide range of audiometric results

many causes, including genetic

often misdiagnosed as central auditory processing disorder

triage audiological test: acoustic reflex testing, OAE (normal), ABR (absent)

cochlea works fine, but beyond cochlea is not, which is why patient can’t understand speech

causes of hearing loss in children

genetics, in utero infections (CMV, syphilis, rubella, toxoplasmosis), prematurity (NICU > 5 days = risk factor), postnatal infections (herpes, chicken pox), chemotherapy, trauma, and noise induced

genetics

non-syndromic: isolated hearing loss without other findings, recessive 70%, dominant 15%, x-linked, mitochondrial 15%, approximately 120 genes identified

10 most common syndromes

hearing loss in addition to another symptom

waardenburg (pigment issues), treacher-collins (craniofacial), pendred (thyroid/goiter), usher (deaf blindness), branchio-oto-renal (kidney malformation), stickler (connective tissue), alport (renal failure), NFII/NF2 (tumors on VIII nerve), jervell-lange-neilson (cardiac), CHARGE (complicated and diverse)

mondini malformation (dysplasia)

a type of malformation of the cochlea where the cochlea only has 1 ½ turns

estimated to occur at 7 weeks gestation

many malformations lumped into that term

enlarged vestibular aqueduct (EVA)

a type of malformation of the cochlea where it can be congenital or acquired in first 3 years of life, genetics not fully understood

can be associated with pendred syndrome