audexam 2

auricular

diff shapes/size/location of ear

anotia

absence of pinna

microtia

small/malformed pinna

low-set ears

ears not positioned on head as expected

preauricuar tags and sinuses

born with them on particular spots

auricular hematoma

internal bleeding of pinna; resulting from a blunt injury (or bacteria or virus (herpes) or neoplasms (cysts/tumors); can result in damage to cartilage (full- cauliflower ear or part of ear)

atresia

absence of external auditory canal

stenosis

narrowing of external auditory canal

acquired disorders of ear canal

impacted cerumen (overaccumulation); foreign object; otitis externa; exostosis (surfer's ear-benign bony groths under the skin in ear canal); osteoma (round bony growth/benign tumor)

effusion

accumulation of fluid in middle ear space

otitis media with effusion (OME)

serous (fluid present without infection); acute (fluid present with infection); chronic (effusion remains for a while); mucoid (purulent), glue ear (effusion became highly viscus and can bind to structures; conductive HL with a small but present ABG

perforation (what is it and how does it heal)

hole in the tympanic membrane; generally heals spontaneously, but sometimes only partially heals and tympanoplasty may be needed which is putting a graft over the hole in eardrum

causes of trauma/physical puncture or rupture of the TM

foreign object, OME

barotrauma

rupture of TM due to rapid or extreme pressure changes (ex. explosions, scuba diving)

monomere/monmeric TM

when the TM heals from perforation without the fibrous layer

cholesteatoma (what is it, what is it made of, what is the effect, how is it caused, how is it treated)

benign pseudotumor in middle ear; made of dead skin and keratin; can cause breakdown of middle ear and surrounding structures; can be congenital or caused by OME or trauma; has to be surgically removed

otosclerosis

bony growth on wall of inner ear called otospongiosis (spongy one) that eventually hardens; stapes fixation into oval window is most common(growth around footplate of stapes that limits sound transfer of whole system); can end up invading cochlea; more common in females (20-40yo, exacerbated by hormonal changes(pregnancy)) ; conductive HL; treated by a powerful hearing aid or stapedectomy surgery

what kind of HL is seen with otosclerosis

conductive with Carhart's notch (small dip in BC at 2000Hz)

myringotomy

surgeon puts a hole through TM to suck fluid out from middle ear space, if it keeps coming back they will put a PE tube (typical for kids, PE tube not common for adults)

stapedectomy (tx of otosclerosis)

removal of stapes bone, which is replaced with a fat pad to cover the oval window, prosthesis attached to incus, entire ossicular chain may be replaced; 90% of hearing back unless prosthesis moves which would bring back the conductive HL

ossicular discontinuty (what is it, what is it caused by, what kind of HL, what type of tympanogram)

separation/break of one or more ossicles but typically it is of the long process of incus; caused by trauma (slap on side of head, head injury, necrosis (death of bone) from OME or cholesteatoma); results in moderate to moderately severe conductive HL with the greated ABG possible of 60dB; typa Ad (hyper complient, high admittance)

glomus tumor (what is it, how does it form, what does it cause, how is it treated)

benign, slow growing vascularized (red) tumor that forms from the paraganglia (neural cells) in the middle ear (invades middle ear space); can cause pulsatile tinnitus (can hear pulse in ears) and conductive HL; requires surgical removal

NIHL

one of the most common forms of acquired sensorineural hearing; caused by exposure to loud sounds (impulse of sustained); damage to stereocilia starting with outer hair cells, can cause metabolic changes in cochlea, can rupture inner ear membranes; usually bilateral

temporary threshold shift

what NIHL begins with; muffled hearing and tinnitus for a while

permanent threshold shift

NIHL at exposure to sounds 3k-6k Hz, causes a notch at 4kHz

national institute for occupation safety and health

more conservative; max dose of noise is 8hr 85dBA within 24hr and every inc of 3dBA cuts acceptable exposure time in half

occupational safety and health administration

less conservative; max dose of noise is 8hr 90dBA within 24hr and every inc of 5dBA cuts acceptable exposure time in half

presbycusis

age related hearing loss; half of those aged 65-75 have some degree of high f SNHL

menieres disease what is it and symptoms

endolymphatic hydrops; episodes of vertigo w vomiting; low f SNHL; aural fullness; low pitched tinnitus ; usually unilateral at onset

endolymphatic hydrops

with menieres, excessive buildup of endolymph in the scala media and vestibular labrynths

menieres tx

may include smoking cessation, eliminating coffee, reduced salt, prescription diuretics, endolymphatic shunt (surgery to drain), destruction of inner ear structures

ototoxicicity

damange to organ of fearing from noxious substances, therapeutic agents (chemo drugs, aminogycosides, loop diuretics, salicylates/aspirin, antimalaria drugs); monitoring occurs

ototoxicic aminoglysides; chemo agents

-mycin drugs, broadband strong antibiotcs (some only affect balance=vestibulotoxic); -platin drugs, generally permanent SNHL;;;HL starts in high f as a precipitous loss that progresses to lower fs

ototoxic loop diuretics

treat edema (fluid build-up) in body; w congestive heart failure, lung disease, renal disease; furosemide, bumetanide; impacts the stria vascularis; dosage dependent; generally temporary

ototoxic salicylates

anti-inflammatory drugs (mainly aspirin); sometimes high dose is required to treat rheumatoid arthritis; can cause tinnitus and typically temporary moderate SNHL

ototoxic antimalarial drugs

quinine and chlorquine; generally temporary bilateral high f SNHL

infection of inner ear, post-natal exposure

generally a consequence of another disease 1. bacterial meningitis (bacteria access the inner ear via the cochlear acqueduct or internal auditory canal) 2. untreated OME or cholesteatoma (invades via the oval or round windows

infection of inner ear, in-utero exposure

1. cytomegalovirus (CMV) (delayed onset HL, other birth defects) 2. herpes zoster (cause bilateral severe SNHL) 3. syphilis (cause bilateral severe SNHL)

sudden SNHL

rapid onset (within hours), begins as moderate, symptoms=aural fullness/tinnitus/sometimes dizziness, causes=autoimmune disorders/viral atteck/vascular embolism/menieres/acoustic neuroma/closed-head traume

temporal bone fracture

result of non-penetrating trauma aka closed head injury that can lead to HL and vestibular dysfunction

temporal bone fracture, transverse fracture

perpendicular to otic capsule, inner ear damage likely, less common, can result in CSF leakage

temporal bone fracture, longitudinal fracture

parallel along petrous portion of temporal bone, generally middle ear involvement only, can result in CSF leakage

neural disorders, retrochoclear lesion

problem behind the cochlea (can be inside auditory nerve)

neural disorders, distal neural lesion

problem beyond the cochlea before the brainstem

neural disorders, peripheral lesion

problems with hearing that do not involve the brain

acoustic neuroma (aka vesetibular schwannoma or acoustic schwannoma)

beign, slow growing tumor arising from schwann cells of vestibular portion of 8th CN; found in brainstem (cerebellar-pontine angle); fills IAC and compresses nerves; results in high f SNHL; causes tinnitus and poor word recognition; abnormal ABR (auditory brainstem response) and acoustic reflexes

auditory neuropathy spectrum disorder (ANSD aka auditory dyssynchrony)

present at birth, results from uncoordinated firing of aud nerve (so it is unable to transmit neural output from cochlea to brainstem); SNHL, poor word rec, absent ABR, present cochlear microphonic, present OAEs

central auditory processessing disorders (CAPD)

nervous system related auditory deficit that interferes w processing auditory info. no cure, therapies can try to retrain brain

auditory processing

how the CNS utilizes/interprets auditory info

3 skill sets of auditory processing that can be affected w CAPD

1. auditory discrimination, 2. temporal processing, 3. binaural processing

auditory discrimination

Analyze acoustic differences between speech sounds

temporal processing

timinng interpretation: duration of a sound, how quickly sound changes, duration of entire signal aka word/phrase

binaural processing

communication of the the hemispheres of brain

levels of binaural processing

brainstem level (ears interact to help with localization and noise management), cortical level (integrating info and adding new info as it comes which is needed for convo and notetaking), separation (focus on one target and ignore everything else

cause of CAPD

lesions along auditory pathway inclcuding the central structures (retrocochlear, brainstem, right and left hems); could also be from central deafness but that is rare and is the complete inability to categroize sound stimuli with the peripheral system intact

symptoms of CAPD

diff w understand speech in noisy enviros, localizing sound source, hearing on phone, understanding prosody changes in humor/sarcasm

what makes CAPD diagnosis tricky

there are many conditions that can cause diff with listening, comprehension, and attention so you can't just diagnose this from symptoms

types of CAPD tests

detailed case history, behavioral test batteries, electrophysiologic tests

what age do u have to be to be properly tested for CAPD

7, because you have to have baseline of higher level processing skills such as learning, attention, language to be able to be properly diagnosed using a multidisciplinary team; the tests are hard and take forever

CAPD test redundancy

two tests should indicate problems, just one or all showing problems means its prob not CAPD

three major CAPD profiles

decoding deficits, integration deficits, prosodic deficits

decoding deficits

understanding speech based problem= system can't extract pitch/loudness/timing cues, considered truest of all CAPDs bc its all auiditory-based deficit, discrimination based problem, located in left hem and/or high brainstem

integration deficits

deficit in comm btn right and left hems (cortical), unable to combine infor and synthesizr meaning, hard to process info quickly, system becomes easily overwhelmed when lots of info is given, so someone would have a hard time taking notes

prosodic deficits

Unable to process the rise and fall in pitch and loudness (syllabic emphasis/intonation), Right hemisphere problem (aka Right Hemisphere APD), Difficulty with analysis and synthesis of prosodic features related to communication

CAPD tx

no cure all but there are 2 types: 1. bottom-up (stimulus driven, trying to help retrain system)=auditory training, skills remediation, environmental modifications such as AAC (often multiple types in various scenarios; 2. top-down (strategy driven)=language,cognitive,metacognitive strategies, educational interventions, workplace,recreational,home accommodations

direct CAPD tx strategies

attempt ot remediate the processing deficits directly such as one-on-one therapy options, computer based listening tasks, home-based therapies but theres not rlly evidence

auditory training

inner ear development in utero by month

2- vestibule vs cochlea, 3- adult form, 4-5- hair cell differentiation and rapid growth, 6- near adult size, 7- first auditory experiences

middle ear development in utero by month

1- eustachian tube begins, 2-ossicles begin, 3-adult form, 4-5- ossicles form and begin to ossify (harden), 7- ossicles fully ossified, 8-tympanic cavity opens

outer ear development in utero by month

2-canal begins, 6-pinna adult form, 7- canal opens

syndromic hearing loss (SHL)

HL accompanied by additional clinical features, about 30-40% of all HL; common co-morbidities: abnormalities with eye, kidney, musculoskeletal, nervous system, metabolic disorders

connexin 26

half of SNHL are due to mutation in GJB2 which is responsible to this inner ear protein; responsible for creation of ion channels and is located in scala media (with organ of corti) and is involved with regulating potassium levels that allow hair cells to create action potentials

aminoglyconside induced hearing loss

mitochondrial inheritance of a predisposition to ototoxicity from these antibiotics even permanant profound HL from just one dose

usher syndrome

one of most common SHL, affects retinas, autosomal recessive inheritance

usher syndrome: USH1

congenital severe to profound SNHL and vestibular dyfunction/early night blindness w progressive narrowing of visual field to severe blindness

usher syndrome: USH2

congenital moderate to severe SNHL with no vestibular dysfunction/ retinitis pigmentosa diagnoses btn 10-40yrs

usher syndrome: USH3

most rare type/late onset progressive HL until profound in 30s with varied vestibular problems/variable progression of retinitis pigmentosa

waardenburg syndrome

disorder of pigmentation of eyes,hair,skin,srtia vascularis in cochlea/autosomal dominant inheritance (1-3% of all congenital HL)

classifications of waardenburg

pigmentation variety (often blue eyes and white forelock): W1=60% have SNHL and dystopia canthorum (wide-set eyes)/W2=90% have SNHL/W3=SNHL, dystopia canthorum and musculoskeletal abnormalities of the upper limbs/W4= addition of Hirschsprung disease

why does asha think it was unethical for audiologists to sell hearing aids in the past

audiologists might suggest a higher price than is actual for their benefit

what kind of hearing aid do kids get

behind the ear because their ear grows too fast and it would be too expensive. also they prob need FM systems in schools and that is only way to get it

hearing aid fitting sequence

assessment, treatment planning, selection, development, verification, orientation, validation

pendred syndrome

most common cause of autosomal recessive syndromic HL/ associated with broad range of HL/ often includes enlarged vestibular aqueduct (EVA)/ progressive HL that is sometimes sudden, 65% of time there is progressive vestibular dyfunctionmay include mondini malformation (not enough turns in cochlea)/ hallmark of syndrome=goiter (thyroid dysfunction, enlarged)

branchio-oto-retinal (BOR) syndrome

autosomal dominant inheritance/ disruption of tissue development in neck, malformation of ears and kidneys/ brachio=second branchial arch which may result in cleft cysts or pits near collar bone/ oto=HL that can be any of the three types and may have pre-auricular pits or tags/ renal=kidney structure abnormalities which can result in end-stage renal disease later in life

charge syndrome

autosomal dominant inheritance/ presentation varies widely/ acronym: Coloboma (gap/hole in iris/eyelid); Heart defects; choanal Atresia (narrowing of nasal passage); Restriction (in growth/development); Genital abnormalities; Ear abnormalities (middle and inner ear problems with hearing and balance issues)

alport syndrome

usually x-linked/ causes progressive kidney failure which leads to end-stage renal disease where u may need dialysis and kidney transplant/ progressive SNHL which can progress to profound/ usually identified in late childhood school age and may require cochlear implantation

jervell and lange-nielsen

autosomal recessive inheritance/ congenital SNHL/ arrhythmia/ prologation of the QTc interval resulting in fainting or sudden death

stickler syndrome

can be dom or rec/ includes diffs in eyes (severe myopia), musculoskeletal (joint laxity), orofacial structures, and ears and hearing/ 5 diff subsets depending on genes encoding diff collagen types

stickler syndrome variants

marshall syndrome (shorter stature, similar other features)/ pierre robin sequence (cleft palate, glossoptosis which is when tongue is further back in oropharynx, micorgnathia which is small jaw)

treacher-collins syndrome

autosomal dom inheritance/ affects development of he bones and tissues of face (midface), affects jaw which can impact airway, feeding, and speech/ half of people with this have hearing loss=usually conductive, and is due to abnormalities of outer and middle ear aka microtia,atresia,or anotia and ossicular abnormalities

hearing aid candidacy

assessment of HL, resolution/consideration of medical issues related to ear/consideration of patient interest/audiometric candidacy

audiometric candidacy for hearing aids

minimum HL: mild HL in 500-4kHz region/ severity: if HL is too great may need CI/ WRS: poor word rec may impact benefit/ speech in noise testing: may need advanced technologies to help in noise/ loudness discomfort level (LDL) and uncomfortable level (UCL): pt may not be able to tolerate loud sounds

two types of basic hearing aid technologies

analog: og tech, no computer processing, sounds collected and amplified, generic fitting strategies/ digital: standard of hearing care, sounds collected and processed, highly programmable to pt's needs, better features to manage noise

digital features of hearing aids: multiple channels

able to adjust aid output in discrete f regions to make speech clearer

digital features of hearing aids: multiple programs

can put automatic settings like a restaurant setting

digital features of hearing aids: compression (automatic gain control AGC)

limits how loud sounds get and minimized distortion, there is a cutoff so loud sounds couldn't hurt hearing, wide dynamic range compression (WDRC) preserves the experience of loudness so they can still tell if a noise is loud of quiet but still sounds natural

digital features of hearing aids: directional microphones

one forward facing and one rear facing microphones, these allow the aid to determine location of speech vs noise, processing allows for Digital Noise Reduction which reduces background noise based on its freq range and location aka cuts off back microphone when they want to hear someone they are looking at

digital features of hearing aids: feedback reduction

phase cancellation of feedback sounds

digital features of hearing aids: linked hearing aids

sounds from one aid can be transmitted wirelessly to opposite ear

digital features of hearing aids: data logging

says how long aids are worn, user adjustments to volume and programs, and assesses pt's enviro throughout day