Final Exam

Question 1

Hearing aids are designed to provide access to?

Answer 1

speech frequency range sounds

for those with not too-profound hearing loss (but still abnormal)

Question 2

Hearing aid microphone

Answer 2

• Microphone at the users ear
o Very portable as well. Microphone will amplify all of those sounds that are within a certain range that are heard by the microphone. Great if in one on one situation, as the only thing amplified will be speaker’s voice. If you are in a room full of people or chatting away and trying to hear speaker from a distance (lecture, party, etc.) the hearing aid will pick up all the speech nose that is within the range of the microphone of the ear.
• Amplifies all sounds, including background noise

Question 3

2 components of hearing aids

Answer 3

1. Transmitter- Microphone Worn by or near speaker, can be placed on lecturn or center of table, or worn around the neck of someone who walks around the room.
2. Receiver- Worn by or near person with hearing impairment

Question 4

Infrared

Answer 4

o Signal transmitted via light beam
o Indoors only (no florescent light)
• Because it is a light-based signal, Line of sight only
• IR Not commonly used now, partially because of this

Question 5

FM

Answer 5

more routinely used; FM is still most common personal ALD used today
o Indoors and outdoors
o Signal transmitted via radio waves
o Multiple broadcasts needs multiple frequencies
o Personal or soundfield

Question 6

Loop

Answer 6

•more routinely used
o Hardwired into a room or pad
o Signal transmitted via electromagnetic field, picked up by receiver in hearing aid or ALD

Question 7

Bluetooth

Answer 7

great, increasingly heard of it
o Wireless
• Truly wireless in time
o Very limited range
• Less than 30 feet; huge battery drain- the newer the devices the less drain on the battery- still very power-hungry system.
o Only works with bluetooth enabled devices

Question 8

ALD for TV

Answer 8

1. Auxiliary speaker or headset
2. TVEars- signal transmitted wired or wirelessly via FM, infrared, or electromagnetically into the receiver which is worn as headphones or earbuds, a speaker, or into the hearing aid or CI directly.
3. induction loop used in conjunction with telecoil (built in to hearing aids and CI’s)- amplifier connected to TV and wires around the room
4. Personal loop- pad on a chair that the loop is built into- principle the same but on a smaller scale. Loop system same as in a theater or church- wires laid around edge of room or area of seating and anyone with access to a telecoil in their hearing aids or via headset.

Question 9

ALD for Phone

Answer 9

amplification, caption, speaker, bluetooth, telecoil

• People with hearing impairment eligible through agencies for free or reduced priced phones

Question 10

ALD for Emergency Alerts

Answer 10

auditory, visual, vibrotactile alert devices

Question 11

ALD for School

Answer 11

children, adults in evening classes, university students, auditory processing disorders; even children with completely normal processing and hearing benefit from better quality signal to noise ratios
Personal FM – most commonly used technology in classroom ALD’s. FM= frequency modulated radio waves. FM systems can be personal (signal coming in from microphone worn around teacher’s neck straight into headset, speaker, or HA or CI). No wires, many children can all use the same technology. If there are several different students in different classes using the technology, different channels need to be selected to ensure that student is listening to the correct teacher. Teacher needs to make sure they turn microphone off in staff room or restroom.
o Teacher to student
o Into hearing aid/headphones or speaker on desk
Soundfield FM
o Teacher to whole class
o Similar to public address system
Loop – FM technology supercedes these
o Teacher to student wearing hearing aid

Question 12

ALD for Work/Meetings

Answer 12

FM most commonly used – difference in quality of signal
• Mostly used by hearing aid users but also by people with mild HI’s via a headset.
• Transmitter in center of meeting desk – pick up voices form all around or pointed specifically at meeting leader
o Receiver worn round neck of listener
o Can be directional or omni-directional
o Signal transmitted to HI user via FM- Bluetooth also possible but range is very small and additional streamer might be needed
o Rogepan???- transmitter with a very direction microphone- looks like a pen, picks up signal and transmits it via FM straight into a hearing aid, CI, or headset.
• Also amplified phones
o Via Vocational Rehab
• VR agency- meant to help people with any form of disability find or remain in work, help with many of these tecnologies

Question 13

ALD for Public Places

Answer 13

Loop commonly used
• Common system for public place- big push to loop America- get as many public places as possible to have systems like this to enable millions with HL to have same access to public events/places as those who have normal hearing. FM system is more personal- useful in noisy restaurant in exactly the same way as it can be for meetings.
• Compatible with hearing aids and headsets
• Theaters, cinemas etc often have headsets
• May be small systems in banks, nursing homes, doctors offices
• Larger systems in airports, conference centers and churches
Many theaters have loop system available at ticket desk- tell them you have hearing loss and they will give you headset and appropriately located seat.

Question 14

Type of loss that would warrant hearing aids

Answer 14

Used for patients with abnormal amount of hearing loss (mild-moderate) of any type but not too profound of a loss; loss of hearing within speech frequency ranges

Question 15

Type of loss that would warrant an ALD

Answer 15

Used for patients with a mild-moderate hearing loss of any type that need help overcoming background noise in conjunction with the help of a hearing aid for the speech-frequency range

Question 16

Type of loss that would warrant an osseo-integrated device

Answer 16

1. Single sided (unilateral) deafness (cros aid)
   o Normal hearing in one ear
   o Profound hearing loss in other ear
2. Permanent conductive hearing losses
   o Atresia, chronic outer/middle ear problems (infection, malformed ossicles), malformed ears, no ear canal openings
3. Some permanent mixed hearing losses (unilateral or bilateral)
   o Chronic conductive problems as above, with some additional sensori-neural component - bone conduction scores must be sufficient (mild-moderate at worst)
   o Available as long as sensori-neural component is not too severe

typically patients with PC or PM components are those with down’s syndrome or craniofacial abnormalities. These conditions can often be associated with some degree of mental retardation, so the progression away from the abutment and its strict hygiene standards has been helpful.

Question 17

Type of loss that would warrant a CrosAid

Answer 17

Used for patients with unilateral or significantly asymmetrical hearing losses

Question 18

Type of loss that would warrant a Cochlear Implant

Answer 18

Used for patients who are severely to profoundly deaf-
People with very significant sensorineural hearing losses bilaterally
•Adult: Moderate to profound hearing loss bilaterally
•Children (>1 year) Severe -profound hearing loss bilaterally
•Internal electrode replaces damaged or missing hair cells
• Limited benefit from amplification
For these patients, Even with top of the line, well programmed hearing aids patient still can only discriminate less than 50% of words without lip reading.

Question 19

Type of loss that would warrant an Auditory Brainstem Implant

Answer 19

Used for (typically NF2) patients who have damaged or missing auditory nerves

Question 20

Type of loss that would warrant an HA/CI combination

Answer 20

Used for those patients with precipitously sloping hearing losses that have good low frequency but poor high frequency hearing

Question 21

Type of loss that would warrant a BAHA

Answer 21

Permanent Unilateral or bilateral conductive hearing loss
o due to malformed or absent outer and middle ears
o Chronically draining ears

Question 22

Audiological Principles: Osseo Integrated Device

Answer 22

Bone Conduction
• Bone conduction- no interaural attenuation
• A microphone on the sound processor picks up sound on one side of the head (side with the worst hearing loss) by the microphone on the speech processor
• Processor converts sound into vibrations (via abutment or radio frequencies)
• Transfers through abutment or magnet to internal implant
• Implant vibrates skull through to cochlea(e) – picked up by best cochlea or both cochlea depending on the type of hearing loss

Question 23

Audiological Principles: Cochlear Implant

Answer 23

1. Pure tone audiometry
   o Confirm HL; Moderate to profound hearing loss
   o Or via ABR for young children
2. Aided testing
   o Sentences in soundfield with no visual clues
   • With appropriately programmed hearing aids- see if patient has access to entire speech frequency range with amplification
   o Levels not enough to enable speech and language to develop
   • Or adult achieve more than 50% word recognition
3. Prepared to adhere to rehabilitation schedule
   o Sound is very different and brain needs to learn to code it
   o Work from patient and audiologist to ensure good progress made
4. Medical evaluation to ensure that they are fit enough for surgery and that anatomy lends itself to having CI. CI’s can be inserted into dysmorphic cochleas to some extent, but if patient has ossified cochlea (bone overgrowth following meningitis), cochlea may need to be drilled out in order to insert the electrode. If there is not an auditory nerve, CI is not the answer- auditory brainstem implant is possible.

Question 24

Type of loss that would warrant a Sound-Bite Hearing System

Answer 24

variation on baha theme, serves similar population except for those with mixed hearing losses currently

Question 25

Type of loss that would warrant Middle Ear Implants

Answer 25

For those patients with Bilateral moderate to severe sensori-neural hearing loss; Those Who don’t want to or can’t wear normal hearing aids but have normal ear anatomy

Question 26

Symptoms & Hearing Loss expected from Atresia

Answer 26

Conductive loss
Absence of ear canal
o Usually associated with conductive hearing loss
• Mild – maximal (>60 dBHL)
o Can indicate abnormal middle ear structures
o Cochlear function may be normal
o Ear can be reconstructed and hearing improved
o Test using headphones and bone conductor
o Bone conduction hearing aids

Question 27

Symptoms & Hearing Loss expected from otitis media

Answer 27

Conductive loss
Middle ear fills with mucus (secretions from nasophaynx) that travels up the Eustachian Tube (ET)
•	Can get infected
o	Bacterial
•	streptococcus pneumoniae
•	Haemophilus influenzae
o	Viral (

Question 28

Symptoms & Hearing Loss expected from Meniere’s Disease

Answer 28

Sensorineural loss
Syndrome associated with 4 primary factors
o Episodic vertigo (lasting 20 min to 2 hrs, nystagmus- beating of the eye)
o Fluctuating hearing loss (associated with vertigo, low frequency at first)
• Does not stay stable, changes over time; tends to happen with low frequencies first rather than high frequencies (which most of the other pathologies we have discussed start with).
o Aural pressure/aural fullness- ear feels plugged and blocked
o Tinnitus (roaring) – rather than being high pitched ringing tends to be a lower frequency roaring sound, almost like putting a shell next to your ear at the beach

Question 29

Symptoms & Hearing Loss expected from Sudden SNHL

Answer 29

Sensorineural loss; Technically sudden hearing loss can refer to any type of peripheral hearing loss that happens in a sudden nature
• Conductive/mixed (middle and external ear)
o Injury
• Vestibular symptoms (such as vertigo) are present between 28 to 57%
• Tinnitus is noted in about 70% of patients
• Usually unilateral (only 2% bilateral)
Patterns of Hearing Loss (PICTURE PRINTED)
• Very variable, anything from profound to mid frequency to down sloping, up sloping, and flat
o Onset (sudden), progression, fluctuation, other neurological signs (facial drooping, numbness, tingling sensation, slurred speech)
o Recent Injury- acoustic, head, barotrauma (e.g. diving), exotic travel
o Pharmacological intake/medications (OTC, prescription, illegal) , change in medications
o Any cause they can contribute to loss

Associated symptoms
• Otologic: vertigo, tinnitus (perceptionof ringing sound in ears), aural fullness, aural pain
• Medical: headache, fever, recent cold/flu, malaise/fatigue, STD
• Need to be identified through historical intake

Question 30

Symptoms & Hearing Loss expected from Acoustic Neuroma

Answer 30

Sensorineural loss

Asymmetrical hearing loss in 95% of cases related to secondary compression on 8th CN and vascular supply to the cochlea
	Tend to happen in the unilateral case
	95% of these cases will be unilateral
	Tumors are arising on the vestibular portion of the nerve, as they grow they begin to compress on the acoustic/cochlear portion of the nerve. 
•	High Pitched tinnitus 
•	Vertigo 
•	Disequilibrium and ataxia 
•	Facial nerve disturbances 
•	Nystagmus (hyperventilation induced) 
	Related to the vertigo.

Question 31

Symptoms & Hearing Loss expected from NIHL

Answer 31

Sensorineural loss
Permanent threshold shift
o If hearing thresholds are raised for more than 16 hours after the noise exposure it is called permanent (OSHA)

Temporary threshold shift
o Hearing thresholds return to baseline within 16 hours post exposure
• Discovering that thresholds may return to baseline, but neural effects may not
 Seen in fine discrimination tasks

Notch at 4000 Hz, Effects can be asymmetrical

Question 32

Symptoms & Hearing Loss expected from APD

Answer 32

the condition whereby a person has normal hearing for standard audiometric tests but has very real difficulties hearing in background noise. Central/sensorineural hearing loss.
• A problem interpreting, or processing, auditory input
• A high proportion of people with hearing loss have auditory processing disorders
o Incoming signal is impaired
• Some people with normal hearing have processing difficulties
• Although diagnosis and testing can only currently be done on people with normal hearing, a very high proportion of people with hearing loss will have APD. Whether that is because the incoming signal itself is impaired or that they also have problems higher up the auditory pathway is very, very difficult to tease out. Also people with normal hearing have processing difficulties.

Symptoms
•	Can’t tell where sound is coming from
•	Can’t hear in background noise 
•	sounds are ‘jumbled up’ 
•	Can’t use context to ‘fill in the gaps’ 
•	transposing letters when verbally spelling 
o	Problems in background noise 
o	Spelling/reading difficulties 
o	Problems with complex directions 
o	Asks for repetition often 
o	Struggling academically

Question 33

Most Common ALD’s

Answer 33

School and/or work
o FM device

Personal use
o FM device
o Bluetooth device (recently)
• GPS/phone etc

Public places / static environments
o Induction loop
• Electromagnetic field within loop
• Program within hearing aid/CI to use

Question 34

Why? : Conductive Loss

Answer 34

Usually Temporary problem with outer or middle ear. loss due to inefficient passage of sound through the middle ear space which is easily managed in most instances with time, medication, surgery, or a hearing aid. No affect on the inner ear, 8th nerve, or hearing organ (cochlea) itself.

Question 35

Why?: Sensorineural loss

Answer 35

Problem with inner ear (Where cochlea is), 8th nerve, or central auditory system. Audiometry ill be affected by both routes (air and bone because both travel through the cochlea and up the nerve). o Cochlea hair cell problem (most common problem; commonly – Sensory hearing loss or problem with sense of hearing organ)
o Nerve problem (less common – neural problem)

Question 36

Why? Mixed hearing loss

Answer 36

Combination of conductive and sensorineural factors/elements.
• Sound is blocked both routes
o Individually or together
• Wax and hair cell loss (elderly)
• malformed cochlea & middle ear structures (child)
• Ear infection that started in middle ear space and has progressed to the inner ear and has started to damage the cochlea

Question 37

When& Why?: CPA (Conditioned Play Audiometry)

Answer 37

3 - 4 years to 6 -7 years
o A child over the age of about 2 1/2 years old may start to inhibit responses for such a passive test as BRA. This is where we tend to start to move towards an adapted version of pure tone audiometry. Conventionally, instead of the child pressing a button to indicate that they have heard a sound (such as an adult does), the child will throw a ball in a bucket or build a tower one Lego block at a time. This is a two-person test, one at the audiometer and one to play with the child. This is a much more labor-intensive and therefore expensive for a clinic than pure tone audiometry in its more adult form. At UF Shands, will typically do some game or another up to about 5 years old and then most kids will be able to reliably raise their hand or say yes when they hear a sound.

Question 38

When & Why?: VRA (Visual Reinforcement Audiometry)

Answer 38

6 months to 3 - 4 years
o Performed usually in conjunction with objective assessments. Performed in the sound field at the younger end of the age range, and can be performed with insert earphones or headphones for the older child. Sounds are played and the child is rewarded with some form of visual reinforcement if they turn to locate the sound. Visual reinforcement/reward might be a soft toy in a light box or a video that plays for a short while. Again, adaptations may be needed if you are testing a child with special needs, such as one that can’t move their head from side to side or a child with a visual impairment.

Question 39

When & Why?: PTA (Pure Tone Audiometry)

Answer 39

6-7 Years (or whenever they are able to cooperate) - Elderly
o Basis of all hearing tests and audiology
o Use this and detailed history to determine communication difficulties a patient is experiencing and how to treat them
o Will not give you all info but detailed audiogram and detailed history as possible will go a long way to treating effectively
o SLP’s- understanding PTA is important to identify people with hearing or processing difficulty that could be aided by surgery or hearing instrumentation- describe basic process to patients and family members if hearing test is recommended
o Pure tones used to get a measurement at exact frequencies, which then relate to the ability to hear different speech sounds
• Pure tone audiometry :
o The basic hearing test
o Involves finding the lowest sound pressure level a person can reliably detect within a specific frequency range (250 – 8000Hz)

Question 40

When & Why?: High frequency audiometry

Answer 40

0-6 months.
HF tympanometry
o 1000Hz probe tone
• Infants (0-6 mo)
o HF tympanometry for middle ear status
• IS there fluid within the middle ears causing a hearing problem or is the cause of the failed hearing screen a more permanent problem with the cochlea itself?
o Pediatrics use 1000 Hz, because mass effects are not abnormal
• Their external and middle ears have not fully developed/stiffened, and so their systems have less stiffness than as an adult and are mass dominated. When we use this 1000 Hz probe tone in a mass dominated system, we get our single peak. However, if we use a 226 Hz probe tone in a pediatric what we would see is notching (opposite of what we would see with adult patients)

Question 41

When& Why?: Tympanometry

Answer 41

Newborn - elderly
To test the function of the middle ear
o Mainly TM / ossicular chain relationship
• It measures the energy transmission through the middle ear space
o Helps with differential diagnoses if used in conjunction with pure tone audiometry
o Most middle ear pathologies result in an increase in stiffness of the middle ear system
• Increase in stiffness of system causes more low frequency sounds to be reflected back into the ear canal from the tympanic membrane
• It is not the stiffness of just the tympanic membrane that is measured, but the whole middle ear system
Differential Diagnosis
• High Frequency Tympanometry
o Instances where we do use high frequency tympanometry quite often is with pediatrics- newborns under the age of 6 months use 1000 Hz probe tone rather than 226.
o Pediatrics use 1000 Hz, because mass effects are not abnormal
• Their external and middle ears have not fully developed/stiffened, and so their systems have less stiffness than as an adult and are mass dominated. When we use this 1000 Hz probe tone in a mass dominated system, we get our single peak. However, if we use a 226 Hz probe tone in a pediatric what we would see is notching (opposite of what we would see with adult patients)
o 678 Hz in adult patients, we can measure conductance and susceptance simultaneously but separately
• 4 normal patterns 1B1G, 3B1G, 3B3G, and 5B5G
• Abnormal if too many peaks or too wide, usually associated with ossicular discontinuity
• Will create notching in the response, different patterns of this notching that can be associated with being normal and different pathologies. A lot of variation in responses and not often used in the clinic for diagnostic purposes.

Question 42

When & Why?: AABR (Automated Auditory Brainstem Response)

Answer 42

• Any newborn baby that does not pass OAEs and is going home that day
o Otherwise repeat OAEs the next day
• Any baby on the NICU for more than 1 week
o Due to risk of neural problems
• Tests whole auditory pathway
o Including nerve function
• Less sensitive to noise, movement and fluids
• Takes longer (up to 3 mins of testing, ~5 minutes to prepare baby)
• More obtrusive (sticky pads etc)

Question 43

When & Why?: OAE’s (otoacoustic emissions) PART 1

Answer 43

Newborn - elderly
Initial test: screening DPOAE’s
• All well babies
o Can be done in the crib, while being held or breastfed
o Takes between 10 – 30 seconds each side
o Non-invasive
o Probe plays quiet (35dBnHL) sound, sensitive microphone picks up ‘echo’ caused by vibration of outer hair cells
o Must pass 3 out of 4 frequency bands between 2 – 5kHz
o Only tests function up to outer hair cells level
• Sensitive to external noise and fluids in ear canal
• Newborn hearing screening
o 0 – 1 month
o As a generalization, newborn hearing screening is conducted up to one month. Often completed while baby is still in the hospital after their birth. For those babies who are born at home, their pediatrician will refer them to a facility like ours to test their hearing by four weeks of age. This is in accordance with the Joint Committee on Infant Hearing guidelines. Crux of guidelines are that the earlier the hearing loss is detected, the earlier appropriate intervention is initiated and the less delayed a child’s communication ability will be.

Question 44

When & Why?: BOA (Behavioral Observation Audiometry)

Answer 44

0 - 6 months
o Objective tests are conducted in addition to behavioral observation. This behavioral observation seems as simple as playing sounds and seeing what happens- may be looking for startle, eye blink, or a change in sucking behavior. You can imagine this is not a particularly scientific test, but can be surprisingly accurate when performed by skilled, experienced audiologists.
o Supplementary to objective tests and not enough on its own.

Question 45

When & Why?: OAE’s Part 2

Answer 45

Newborn- elderly
If middle ear function is normal
• Know function of the middle ear system- the reason you do tympanometry
• Ideally you are going to try and get OAE’s-this will give you a good objective measure of the child’s hearing before you have to try and elicit behavioral info. It will also give you a certain level of confidence going into the soundproof booth if you have already got clarification that the peripheral hearing system is normal.
• Only worth doing if middle ear function is normal- if they have fluid in that middle ear space, then the likelihood is that OAE’s are going to be absent- will not give you additional info- can try but do not bill.

Question 46

What is Tympanometry?

Answer 46

Tympanometry is a quick and non-invasive procedure for the detection of middle ear dysfunction. It provides objective info regarding the functional status of the tympanic membrane and the middle ear system. It is the measure of the changes in stiffness or compliance of the middle ear system in response to changes in pressure.

Question 47

Tympanogram Type A

Answer 47

3 variants:
type A - normal type/shape of tympanogram
o type AS - more shallow typanogram that still has a single peak; Hypo-compliant. Fairly stiff system
o type AD- has a larger peak consistent with higher immittance; Hyper-compliant, Ossicular discontinuity, ‘floppy’ TM

positive pressure (A)
o Eardrum bulges out as pressure in the outer ear decreases. Pressure in the middle ear remains the same
o System is under tension (stiff) due to differences in pressure on each side of TM, more sound is reflected back, sound is louder at microphone of tympanometer

Question 48

Tympanogram type B

Answer 48

flat in shape
Equal pressure (B)
o Eardrum is under least amount of tension (least stiff) so sound travels through more effectively
o Less sound is reflected back, sound at microphone is quieter and louder to patient

Increased stiffness (type B)
o	Middle ear pathologies
•	Otitis media with effusion
•	Ossicular fixation
o	Scarred tympanic membrane
•	Several ear infections / perforations
•	Several middle ear surgeries
Flat response / no peak
o	Sound bounces off TM regardless of applied external pressure due to stiffness of middle ear system
o	** flat responses with large ear canal volumes are indicative of a TM perforation

Usually gives rise to a conductive hearing loss (see an air-bone gap)

Question 49

Tympanogram Type C

Answer 49

shifted towards negative pressure

negative pressure (C)
o	Eardrum bulges into middle ear space due to an increase in the outer ear pressure.  Pressure in the middle ear remains the same
o	System is under tension (stiff) due to differences in pressure on each side of TM, more sound is reflected back, sound is louder at microphone of tympanometer

Normal compliance at negative pressure
• System most compliant when negative pressure in external ear = negative pressure in middle ear
• Resolving/evolving effusion

Question 50

Ototoxicity: Introductions

Answer 50

A variety of drugs, such as certain powerful antibiotics and some anti-neoplastic drugs generally used against (poten-tially) life-threatening diseases can cause auditory and/or vestibular dysfunction. In general the site of lesion is almost exclusively cochlear, and balance dysfunction may derive from comparable degeneration.

Question 51

Purpose of ototoxicity monitoring research

Answer 51

1) evaluating the overall efficacy of current clinical methods for ototoxicity monitoring; (2) justifying inclusion of this area in the audiolo-
gist’s scope of practice; and (3) developing useful ototoxicity monitoring programs for clinical and research applications.
The dual purpose of this document is thus to provide a position statement on the clinical audiologist’s role in ototoxicity monitoring and guidelines for the implementation of an ototoxicity monitoring program

Question 52

Purpose of ototoxicity monitoring

Answer 52

(1) early detection of changes to hearing status presumably attributed to a drug/treatment regime so that changes in the drug regimen may be considered, and
(2) audiologic intervention when handicapping hearing impairment has occurred.
The term “ototoxicity monitoring” is generally taken to express the principle of early identification, yet the concept also embraces the principle of early intervention.

Question 53

***Audiologist’s role in ototoxicity monitoring

Answer 53

(1) In-service education
(2) the interpretation of results.
(3) management of a hearing loss that is not treatable medically. Such assistance to the patient/ patient’s family may include counseling, communication strategies, and prescribing amplification and/or assistive listening device(s).
(4) Only the audiologist is endowed by their professional training with the ability to achieve both objectives of ototoxic-ity monitoring. The audiologist thus should take the lead in developing ototoxicity-monitoring programs, driven by the dual goals, again, of preventing or minimizing hearing loss and helping the patient to maintain the most effective hearing communication possible. These clearly are important ‘quality of life’ issues and quality of life is now recognized as a global imperative, whatever the medical management.

Question 54

New ototoxicity monitoring research

Answer 54

(1) comparisons among monitoring and analysis techniques for a variety of patient populations.
(2) new drugs are being developed that appear to have excellent therapeutic efficacy without ototoxic side effects (Campbell et al. 2003a). Still, other drugs and dietary supplements are being evaluated specifically to prevent ototoxicity when delivered either before or in combination with ototoxic drugs
(3) research on otopro-tectants might lead to the assumption that the need for ototoxicity monitoring will disappear with effective ototoxicity prevention. It seems more likely, however, that monitoring will be essential to ensure effectiveness of such countermeasures.

Question 55

***Pharmalogical agents of interest

Answer 55

The most frequently used ototoxic drugs are the platinum coordination complexes, aminoglycosides, loop diuretics, and nonsteroidal anti-inflammatory agents (Humes, 1999; Garcia et al. 2001;)
In practice, the audiologist is most likely to encounter ototoxic hearing loss in cases that have been treated with anti-neoplastic drugs or aminoglycosides

Question 56

Exacerbation of ototoxic effects

Answer 56

(1) Ototoxic drugs may not be administered, indeed, often are not administered, in isolation, or in isolation from other effects toxic to the ear, such as other drugs, environmental toxic chemical exposures, and/or excessive noise exposure
(2)Furthermore, if a localized disease process was involved in the first place as it is in most drug-treatment cases, then, it may have its own implications for audiovestibular dysfunction, and also may interact with a given drug
(3) disease may exacerbate the effects of
ototoxic medications, which emphasizes another reason to obtain pre-treatment audiometry/audiologic data whenever
possible

Question 57

***Most important tool for clinician in diagnosing/evaluating ototoxicity

Answer 57

a thorough history

Question 58

Non-medical ototoxic exposure

Answer 58

(1) environmental chemicals such as organic solvents, asphyxiant gases, pesticides, and heavy metals
(2) workers may be monitored for noise induced hearing loss, yet overlooked for screening of chemical ototoxin

Question 59

FDA requirements

Answer 59

(1) ototoxicity monitoring is often not included as a part of clinical trials for new drugs,
(2)at this time, the FDA does not have Good Clinical Practice (GCP) guidelines for monitoring ototoxicity in clinical trials of new drugs. Yet, there are poten-
tial models in the literature
(3) Test procedures approved for clinical trial include: air-conduction testing in the conventional and high-frequency audiometry (HFA) frequency ranges; bone-conduction testing as indicated; tympanometry and word recognition at baseline, dizziness handicap inventory (DHI)

Question 60

***Three main approaches to audiologic ototoxicity monitoring

Answer 60

(1) The basic audiologic assessment
(2) high frequency audiometry (HFA)
(3) otoacoustic emission (OAE) measurement

Question 61

Why is HFA used for ototoxicity monitoring?

Answer 61

The earliest effects of ototoxic drugs tend to be manifested by the outer hair cells (OHCs) of the basal cochlear turn
the effects of these drugs tend to first occur tonotopically basalward to the effective place limit of the conventional audiogram

Question 62

Hesitance about using HFA for ototoxicity monitoring

Answer 62

Hesitance to adopt HFA early on derived from concern for excessive intersubject variability of threshold measures, in part, due to the problem of standing
waves in the ear canal above its resonant frequency. This anxiety has greatly diminished since the 1980’s as improved instrumentation emerged. HFA thresholds were shown to be measurable with acceptable variances, and HFA was shown also to be effective for ototoxicity detection
Surprising, in light of the vulnerability of high-frequency
hearing to the upward spread of masking and the prevalence of low frequencies in environmental noise, HFA has been shown to be efficacious in a quiet hospital room for bedside testing

Question 63

Limitations of HFA use for ototoxicity monitoring

Answer 63

(1)Many patients with hearing loss in the conventional frequency range may not have measurable hearing at high frequencies (Osterhammel 1980; Kujansuu et al. 1989), the elderly being the most vulnerable to such exclusion
(2)Any prior history of hearing loss also
potentially limits the usefulness of HFA, as the most common losses are sloping high-frequency types.
(3)Test efficiency is also an issue.
(4)for all the drugs in question, effects tend to be systemic (i.e., progressing from high to low frequencies)
and bilaterally symmetrical. Yet, this audiometric pattern is not always the case, so both ears routinely tested

Question 64

Presentation of ototoxic hearing changes and unique monitoring

Answer 64

Ototoxic hearing changes tend to present first within a limited range of frequencies near the highest frequencies
detected by each individual patient (Fausti et al. 1999). Consequently, most changes are observed to occur within one octave of the highest audible frequency in each patient. This range has been found to be unique for each individual and specific to the individual’s hearing configuration. A shortened, serial monitoring protocol, individualized to each patient’s hearing configuration thus has been proposed

Question 65

Potential utility of OAE testing for ototoxicity

Answer 65

HFA usually detected ototoxic changes prior to DPOAEs, although both HFA and DPOAES changed prior to thresholds in the conventional frequency range (Knight et al. 2007). However, OAEs may still be useful as a part of an ototoxicity monitoring program, because they do not require a behavioral response and are time
efficient. TEOAE (Beck et al. 1992; Zorowka et al. 1993; Stavroulaki et al. 1999) and DPOAE (Muhleran and Degg 1997; Ress et al. 1999; Lonsbury-Martin & Martin 2001) responses tend to change before hearing thresholds in the
conventional frequency range, but not before changes in HFA thresholds
DPOAEs may detect ototoxic change earlier than TEOAEs (Lonsbury-Martin & Martin 2001) perhaps because, practi-cally, DPOAEs can be measured at higher frequencies than TEOAEs

Question 66

Limitations of auditory tests for ototoxicity

Answer 66

(1)One important limitation of OAE testing as mentioned above is that the results are significantly affected by middle ear pathology such as otitis media (Allen et al. 1998). That is, OAEs are difficult to record reliably, if detected at all, in the presence of otitis media (Owens et al. 1992). And, as previously noted, the patient populations receiving ototoxic medica-tions have increased susceptibility to otitis media, which interfere with OAE ototoxicity monitoring.
(2) One of the primary advantages of HFA over OAE testing is that the significant change criteria for the former test are well established with excellent specificity and sensitivity
both HFA and OAE testing are problematic in patients with hearing loss, particularly in the elderly
(3)OAE measurement in children is a particularly attractive approach for ototoxicity monitoring, namely, as an effi-
cient objective test. Otitis media is common in children and in immunosuppressed chemotherapy patients, in general, as well as in patients receiving head and neck radiation, and in patients with infections undergoing treatment with aminoglycoside antibiotics. In any event, using a test battery approach increases the chances of obtaining reliable ototoxicity monitoring data over time.

Question 67

IS OAE testing efficient alone in testing for ototoxicity?

Answer 67

NO. For this reason (limitations with otitis media) alone, tympanometry, and ideally multi-frequency tympanometry, should routinely be evaluated when OAE testing is included as part of the test battery. Hence, OAE measurement probably should not be the sole method of ototoxicity monitoring, because interruptions in monitoring may occur whenever otitis media is present.

Question 68

Test battery for diagnosing/monitoring ototoxicity

Answer 68

High frequency audiometry can be conducted in the presence of otitis media, but it cannot then be assumed that any changes in the HFA range are secondary uniquely to ototoxicity. At this juncture, whenever changes
in any responses are noted, a complete follow-up audiologic assessment is indicated to rule out a conductive component (i.e., via conventional methods) to assure that any observed shifts are potentially attributable to ototoxicity (i.e., sensorineural loss). Clearly, using a test battery approach increases the chances of obtaining reliable ototoxicity monitoring data over time.

Question 69

Tinnitus and ototoxicity

Answer 69

(1) Tinnitus is a common side effect of many ototoxic drugs (Seligman et al. 1996), particularly cisplatin (Kopelman et al. 1988), but currently no formal tinnitus monitoring procedures have been developed.
(2)Tinnitus assessment methods are
rarely reported. Sometimes tinnitus is merely analyzed via patient self-reports. Furthermore, patients with life-threatening illnesses may not self-report tinnitus, because they are overwhelmed with other issues.
(3)When monitoring patients for ototoxicity, questioning them systematically about any tinnitus symptoms at each appointment is strongly encouraged.

Question 70

Auditory evoked potential testing for ototoxicity

Answer 70

(1) Technical issues of stimulating and record-ing valid and reliable high-frequency ABRs. However, as noted previously, their methods have yet to be incorporated into manufactured evoked-potential test systems, let alone evaluated for clinical utility.
(2) high-frequency ABR testing would be most needed in patient populations, who cannot provide reliable behavioral responses for HFA, such as young children on chemo-therapy. However the use of sedation, particularly repeated use of sedation, to obtain reliable ABR recordings and track changes over time seems unadvisable and likely contraindicated because of their other health and medication issues.

Question 71

Potential bias of noise exposure and ototoxicity

Answer 71

Eluded to earlier was the importance of querying the patient’s history of noise exposure, and the issue of concomitant noise exposure is a matter that certainly warrants concern. Concern here is not limited to prior noise exposure. In fact, patients should always be counseled to avoid noise exposure during and for several months following (potentially) ototoxic drug administration. noise exposure can exacerbate the ototoxicity of both aminoglycosides and cisplatin
The effects of overexposure to noise also tend to be expressed predominantly in the dysfunction/destruction of OHCs located more basalward along the hearing organ. Therefore, unless the ototoxic exposure dose effectively exceeds that of the prior noise exposure, further loss appears not to occur.

Question 72

Vestibuotoxicity monitoring: Introduction

Answer 72

Although the vestibulotoxicity of some drugs, particularly certain aminoglycosides, is well established (e.g., see Selig-
man et al. 1996; Day et al. 2007), no widely accepted guidelines for vestibulotoxicity monitoring exist. Furthermore, some clinical vestibular tests would be impractical, if not ill-advised, for routine monitoring of this patient population. Patients of interest typically suffer malaise and have low tolerance for any discomfort that might be caused by such testing. However, there are several relatively benign instruments to help screen for balance disorders, although less-than-fully
assessed for sensitivity and specificity for ototoxicity monitoring.

Question 73

Ototoxicity: techniques to assess vestibular system function

Answer 73

(1) Quantitative
caloric testing [i.e., either bithermal, monothermal warm, or ice water caloric testing, usually referred to as electronystagmography testing (ENG)], rotational testing [i.e., either whole body, or head-only vestibular autorotation testing (VAT), vestibular evoked myogenic potentials (VEMPs), and computerized dynamic posturography (CDP)].
(2) Informal/bedside
Head-thrust and dynamic visual acuity tests. However, these informal tests are sensitive to impairments of high-frequency function. Thus, they are not helpful for the identification of the earliest signs of bilateral peripheral vestibular system impairment that begin in the lower frequencies.
(3)there are self-report measures of dizziness disability/handicap that include the DHI (dizziness handicap inventory) noted above

Question 74

Adult/Geriatriatric vs. Pediatric Ototoxicity testing

Answer 74

(1)both HFA and OAE testing can be problematic in patients with hearing loss developed in
advance of their ototoxic exposure, and this (a priori) is the complication most common to ototoxicity monitoring in the
elderly
(2)Hearing preservation is particularly critical in this patient population, because some are still acquiring speech and language. Untreated hearing impairment compromises literacy development (Yoshinaga-Itano 1999, 2003) and scholastic achievement in the years ahead (Moeller et al. 2007), as well as presenting quality of life issues that pervade all populations. These patients, especially young children, may well be too ill and/or too immature to participate well in behavioral audiometry (conventional or high frequency). Objective methods thus become essential. Otoacoustic emission testing, again, is particularly attractive, while being mindful of the complications of otitis media (Allen et al., 1998) or simply crying. sedating these ill children, who are frequently on multiple medications, may be contraindicated, particularly for repeated testing.
(3)Effective disease treatment is paramount and must be considered in program development. In adults and children alike, the approach to ototoxicity monitoring must be weighed according to clear objectives and realistic assessment of potential outcomes. Ototoxicity monitoring protocols, therefore, are somewhat disease and/or treatment specific

Question 75

Rehabilitation for ototoxicity

Answer 75

Audiologist can and must play primary role. Here, it very likely will be inadequate to assume that, after Detecting significant changes in hearing and/or (especially) finding a significantly handicapping degree of impairment, management will fall into place automatically, i.e., via routine referrals to the audiologist or audiology clinics. In fact, in the backdrop of serious health conditions of the patient and just how ill the patient may be, it seems more likely that audiologic management will be
only an after-thought and generally overlooked. The impact of frequent changes in hearing status as the patient progresses through the drug-treatment protocol also needs to be considered. Ototoxicity monitoring protocols need to be more dynamic than typical clinical programs. Additionally, there are issues concerning the makeup of the treatment plan and how the costs will be covered.

Question 76

Ototoxicity treatment modalities

Answer 76

Treatment modalities are familiar—appropriate selection of assistive listening devices and/or hearing aids (presuming
no medically treatable conditions, especially otitis media)—but the specifics of the case and, again, the more dynamic situation may dictate a different balance of these modalities. For example, selection of an appropriate assistive listening
device might take precedence over hearing aid selection (i.e., typically the opposite of the routine management flow).

Question 77

Considerations for implementing an ototoxicity monitoring program

Answer 77

The implementation of an effective ototoxicity monitoring program ultimately depends upon the coordination of a varietyof program components, as summarized in the forgoing, and the effectiveness of audiology methods and the individual patient’s circumstances. All such programs start at the level of actual audiologic testing, with critical decisions on what is to be treated as a significant change in hearing status, regardless of the particular test methods. Here, in turn, are a variety of issues.

Question 78

NCI CTCAE ototoxicity grades for children (with adult guidelines in parentheses)

Answer 78

Grade 1: Threshold shift or loss of 15-25 dB relative to baseline, averaged at two or more contiguous frequencies in at least one ear (same for adults);
Grade 2: Threshold shift or loss of >25-90 dB, averaged at two contiguous test frequencies in at least one ear
(same for adults);
Grade 3: Hearing loss sufficient to indicate therapeutic intervention, including hearing aids (e.g., >20 dB bilateral HL in the speech frequencies; >30 dB unilateral HL; and requiring additional speech language related services) (Adults: >25-90 dB, averaged at three contiguous test frequencies in at least one ear);
Grade 4: Indication for cochlear implant and requiring additional speech language related services (Adults: pro-found bilateral hearing loss >90 dB HL).

Question 79

Brock’s Hearing Loss Grades

Answer 79

originally designed for children receiving platinum based chemothera-peutics, are:
Grade 0: Hearing thresholds

Question 80

ASHA criteria for determination of ototoxic threshold shift to date

Answer 80

Significant ototoxic change must meet one of the following three criteria: (a) ≥20 dB decrease at any one test frequency, (b) ≥10 dB decrease at any two adjacent frequencies, or (c) loss of response at three consecutive frequencies where responses were previously obtained.

Question 81

Patient identification and testing management

Answer 81

In contrast to advances in newborn hearing screening, a broad mandate for the implementation of ototoxicity has yet to develop. Indeed, it seems unlikely that such a mandate will be forthcoming in the near future, considering the complex issues of how targeted patients should be identified and where/how are testing and monitoring costs are to be absorbed. These issues are likely, as well, to be highly different among medical centers’ practices and the nuances of health-care practices and financing from state to state. At this juncture, monitoring programs remain strongly dependent upon individual initiatives, although a number of references are available clearly demonstrating the overall efficacy of ototoxicity monitoring and to guide the audiologist. In general, the audiologist needs to develop excellent working relationships with oncologists for referrals for patients on platinum-based chemotherapeutics, and infectious-disease specialists for patients on aminoglycoside antibiotics. Although other types of drugs may be ototoxic, regularly scheduled clinical ototoxicity monitoring is usually limited to those two drug classes, as noted earlier. With modern medical database systems, it seems practical, in principle, to be able to identify efficiently those patients receiving a treatment with a potentially ototoxic drug and to target patients via automated referral generation. However, at this writing, no such system has been described in the literature. In ototoxicity monitoring, it is essential to be proactive. To the extent possible, it is absolutely critical to obtain a baseline evaluation prior to the patient receiving the monitored drug therapy. For treatment of infectious-disease patients, aminoglycoside administration may occur on an emergency basis, so prior audiologic assessment may not be feasible. Follow-up evaluations then should occur just prior to each course of platinum-based chemotherapy, after any temporary threshold shift has had time to recover, and before the patient is connected to intravenous lines or monitoring equipment. This schedule will also allow patients to be tested when they are feeling at their best, thus, providing more reliable behavioral responses.

Question 82

Ototoxicity monitoring protocols

Answer 82

For aminoglycoside antibiotics, weekly or biweekly monitoring is recommended, ideally. Because aminoglycosides can also cause delayed hearing loss, follow-up testing should also be scheduled a few months after drug discontinuation. Lastly, ototoxicity may occur secondary to a wide variety of agents, and monitoring protocols may need to be designed accordingly.