CSDS 131 Final

• Components of California's NBHS program

  1. Outreach and awareness campaign

  - educational materials

  - public media campaign

  2. Screening

  -covers all newborns

  -covers compentencies of test, equipment, and basic follow up system

  3. Geographical based hearing coordination centers

  - assists hosipitals with development and implementation of their screening programs

  -certification of screening sites

  -assures follow up testing of infants who fail screening
• What tests are being utilized as part of the NBHS program?

  OAE/ AABR
• What is the time frame for screening hearing, identifying HL and intervention, audiologically and educationally in newborns?

  -OAE/AABR before discharged

  • If fail, often try second, if time allows, before discharge or will repeat screen within one month
  • *if fail rescreen-infant diagnostic hearing evaluation by 3 months

  -If HL detected on diagnostic hearing evaluation, when age appropriate- participate in behavioral thresholds

  -Once HL detected, fit with amplification by 6 months

  • A pass at any stage is not tested any further
• Minimal response levels


  Lowest level which a behavioral response to sound is exhibited , not truly a "threshold"

  -Birth-4m measures "responsiveness"
• Describe how infant's general response to auditory stimuli changes during the first 2 years


  -Birth to 4m measures "responsiveness"

  -Beyond 4-6m, behavioral response to speech similar to adult levels, FM tones may stay elevated til 1.5 yo
• BOA


  Behavioral Observation Audiometry; When baby hears sound eye widen or cessation of sucking, can be nursing technique

  - Diagnostic, may use 2 testers for judgement

  -sound presented from side or back

  - Eye blinking, change in sucking activity , startle response, orienting behavior

  -Birth-6m

  -Not thresholds: minimal response levels
• VRA


  Visual Reinforcement Audiometry; Condition child to turn to sound and reinforce with visual stimuli; Ex: sound goes off and child turns toward TV that starts playing a cartoon

  -Initially conditioned by pairing sound with light

  -6m-2 1/2 yrs

  -Ascending- descending approach

  -Once behavior is established, use intermittent reinforcement
• CPA


  conditioned play audiometry

  -2 1/2 -5 Years old

  -quick and efficient

  -Speech audiometry used to gain rapport

  :blocks, pegs, puzzle, balls, etc (used to demonstrate when sound presented)

  -sound presented at: 2k 5k 4k 1k 8k 6k 250 and 3k Hz
• How does localization develop?


  -6m-8m age

  -hearing similar in both ears

  -Eye/head movement horizontally-> vertically -> arc -> direct line

  By 8m old no localization means
• Microtia


  abnormally small pinna, seen with atresia

  -> Outer ear, congenital

  HL: Conductive

  Tx: plastic surgery
• Anotia


  absence of pinna

  -> outer ear

  -> congenital, may be part of a syndrome

  Tx: plastic surgery

  No HL (hearing loss)
• Stenosis external otitis


  -narrowing of external auditory canal

  -caused by: trauma, inflammation, genetics, tumor

  -Not usually any hearing loss, more susceptible to earwax buildup
• Atresia


  Absence of a normal body opening; occlusion; closure

  -Cartilaginous portion/bony portion, entire EAC never formed

  -congenital (treacher-collins syndrome)or acquired (trauma/burns)

  -more common in males

  -unilateral (more common) but can be bilateral

  HEARING LOSS CONDUCTIVE (OUTER AND MIDDLE EAR)
• Tympanic membrane perforations


  3 types:

  1. Central perforations: occurs in pars tena

  2. Marginal perforations: involves annulus, skin can migrate to middle ear space, more dangerous

  3. Retraction pockets (not perforations): negative middle ear pressure, TM retracts usually in pars tena

  -Causes: trauma(most common), thermal burns, foreign bodies, skull fractures, ear infections

  HL 0-40 dB HL loss

  Treatments: spontaneous healing, myringoplasty (special paper or gel form), Tympanoplasty (tissue graft)

  CONDUCTIVE HL MIDDLE EAR, TYPE B TYMPANOGRAM
• Otitis media


  AKA ear infection

  -Inflammation of middle ear

  -Middle Ear effusion= fluid as result of OM

  Types:

  Serous OM= ME inflamed with thin watery fluid, may see fluid line (meniscus)

  Mucoid OM= thicker effusion

  Suppurative OM= ME inflamed and contains infected liquid with pus

  Adhesive OM= thickening of fibrous TM tissue, severe retraction, negative pressure

  -2nd most common childhood disease besides common cold

  -9/10 by 5th birthday birthday

  LONGTERM SNHL, treatment: antibiotics only when accompanied by fever and ACUTE OM

  CAN RESULT IN MYRINGOTOMY (placement of tubes that equalize pressure) in ears
• Cholesteatoma


  Tumor like mass filled with epithelial cells and cholesterol (grows into the ME space)

  -secondary to TM perforations and/or OM

  Symptoms include: Otorrhea, HL, Vertigo, Tinnitus pain, head aches, facial nerve pain paralysis, ET dysfunction

  
  

  Treatment: surgical removal
• Otosclerosis


  Lesions of osseous/bony portion of Inner Ear stapedial footplate

  -Layer of new bone laid down at same time older bone resorbed, produces spongy type of bone

  -Footplate becomes wedged in OW (anklyosis) and fixated

  -70% hereditary, 2X more common in women

  TYPE As and TYPE B TYMPANOGRAM

  -Carhart's Notch

  Bilater, unilateral in only 10-15% of cases

  -may see bluish cast in whites of patient's eyes
• How does Otitis Media develop?

  -Often caused by faulty Eustachian tube function

  -pressure equalization prevented

  -may be negatively influenced by large adenoids (negative pressure drops)

  -Fluid from membranous linings of ME is pulled into the ME cavity or infection may travel from opening of ET

  -Fluid accumulates and may become infectious
• What are common causes of congenital SNHL?

  -German measles (Rubella)

  -Mumps

  -Syphilis

  -Cytomegalovirus (CMV): HL in children

  -Zika Virus

  =impact during 1st trimester of pregnancy
• What are the inner ear disorders?

  Noise induced HL, Ototoxicity, Meniere's Disease, Prebycusis, Sudden idiopathic SNHL, Auditory tumor, and Semicircular Canal Dehiscence
• What are the outer and middle ear disorders?

  Microtia, Anotia, Stenosis external otitis, Atresia, Tympanic membrane perforations, Otitis media, Cholesteatoma, and Otosclerosis
• noise-induced hearing loss (NIHL)

  • gradually progressive SNHL resulting from repeated exposure to dangerous sound levels.

  • Loss of outer hair cells

  - Noise Notch- greatest at 3k-4k with recovering at 8k

  -more common in men than women
• Ototoxicity


  -Degenerative changes to Inner Ear as a result of certain drugs

  -Impact cochlea and/or vestibular system

  -Congenital by maternal ingestion

  -Acquired at any age

  DRUGS: Antibiotics in aminoglyosick family (ex: gentamycin, streptomycin, kanamycin, etc), Quinine, Salcylates (asprin), some diuretics (ex: Lasix) and Chemotherapy agents (ex: cisplatin)

  HL impacted in high Hz

  2 tests: Ultra high Hz and OAE testing

  SNHL, BILATERAL, USUALLY STARTS IN HF

  TYPE A TYMPANOGRAM
• Meniere's disease


  Abnormal condition within the labyrinth of the inner ear that can lead to a progressive loss of hearing. The symptoms are dizziness or vertigo, hearing loss, and tinnitus (ringing in the ears).

  -Times of remissions and exacerbations

  -HL is usually Unilateral, fluctuates, and progressive (only 5-10% bilateral)

  -Results from: head trauma, infection, degeneration of IE, allergy, or occasion a tumor (but usually from unknown cause)

  -Alcohol, smoking, drugs, and caffeine increase symptoms

  Treatment: diet restrictions, drugs, surgery with a shunt placed to drain excessive endolymph, and audiotory nerve severed.
• Prebycusis


  SNHL, progressive hearing loss resulting from aging

  -cochlea affected

  -Bilateral, symmetrical

  -HF initially 2k- 8k Hz

  3 types:

  1. Cochlear prebycusis: cochlear hair cell loss, HL often in very low and high frequency region

  2. Neural Prebycusis: degeneration of spinal ganglion. Made up of cell bodies of auditory nerve fibers

  3. Metabolic: stria vascularis within the cochlea suffered reduced blood supply resulting in reduced responsiveness of hair cells
• Sudden Idiopathic SNHL


  -Usually unilateral: develops over few days or seemingly instantaneously

  -Decrease of at least 30 dB over at least 3 days at 3 octaves

  -Adults

  -Suggested etiologies: autoimmune diseases, viral or other infections, rupture of basilar membrane, vascular disorders, tumors, neurological disorders

  -Treat as an emergency- if not then hearing loss may not be recovered
• Auditory tumor


  "Acoustic neuroma"- most are benign

  -symptoms: HL, facial nerve involvement, vestibular disorders (balance and equilibrium), tinnitus, and poor word recognition scores

  -Gradual HL-can be sudden

  -95% are unilateral HL

  -Diagnostic audiologic tests: WRT, AR, ABR, and ENG

  -Confirmed with CT scan and/or MRI

  -treatment: surgical removal
• Semicircular canal disease


  "Superior canal dehiscence syndrome"

  -superior semicircular canal most often affected than posterior

  -symptoms: dizziness, vertigo, disequilibrium

  -"echo" sensation when talking (autophony)

  -Cause by: thinning/weakening of the bone that covers the superior semicircular canal (essentially resulting in 3rd window in labyrinthine system)

  -symptoms induced by intense sound
• What is audioneuropathy and audiometric findings on pure tones: ABR and OAE tests?

  Audioneuropathy: auditory characteristics that support normal outer hair cells function and abnormal responses from the CN VIII and brainstem

  -can't be detected with MRI

  -Site of dysfunction may be with synapse of inner hair cells and auditory nerve (but no one really knows)

  New info: most common genetic cause of auditory neuropathy is insufficient production of a protein called otoferlin

  -15% NICU graduates

  -Bilateral usually but can by unilaters

  -difficulty processing rapidly changing acoustic signals- auditory temporal processing

  
  

  Pure tone test= normal to profound HL (most common low frequency HL)

  Speech recognition= variable

  OAE= present/normal

  ABR= absent/abnormal

  -acoustic reflexes= absent ipsilateral and absent contralateral
• APD + characteristics


  Also known as central auditory processing disorder

  -brain taking in information and processing it

  -affects auditory behavior (what?), academic, language, cognitive/mode of learning, and medical (see chart)

  Treatment= strategies, environmental modifications, use of hearing assistive technology

  
• What are the candidacy requirements for testing for APD?

  1. normal hearing sensitivity

  2. minimum of 7 years of age

  3. near-normal to normal IQ

  4. no greater than a moderate language impairment

  5. sufficient attention abilities to perform the assessment tasks
• 4 Areas that are evaluated as part of auditory processing battery of tests

  1. Temporal processing: timing aspect

  2. Dichotic listening- sentence 1 ear vs other ear: client is instructed to say the sentence in instructed ear

  3. Low redundancy monaural speech- certain frequency filtered out of speech

  4. Binaural interaction- back and forth between ears to put a word together. Example: left ear [da] right ear [g] together = dog
• Aural Rehabilitation


  treatment to improve communication ability of those with hearing loss acquired after the development of spoken language

  -provided when HL is acquired prior to development of spoken language
• What are the 6 areas that comprise aural rehabilitation?

  1. Hearing assistive technology devices

  2. Speechreading

  3. Auditory training

  4. Speech language

  5. Communication strategies

  6. Counseling/educating
• What are the main components of a hearing aid?

  Microphone: picks up auditory signal and converts it into electrical signal

  Amplifier: increases the intensity

  Receiver: converts it back to acoustic signal and sends it to listener

  Supply source: provides power

  
• What are the 4 factors that need to be considered when selecting a hearing aid?


  1. Style

  2. Features

  3. Warranties

  4. Bilateral vs unilateral
• Gain


  How much is amplified
• Frequency response


  Frequencies amplified
• Output limiting as it pertains to hearing aids

  Restricts ultimate loud
• Describe bone conduction hearing aid and basic function

  -uses small abutment to attach sound processor directly to implant

  -sound processor picks up sound vibrations from the environment, transferred through abutment to a small titanium implant inserted in the bone behind the ear

  -sound vibrations sent directly through the bone to the IE (Cochlea) where they are converted into electrical impulses by hair cells