CI Regulations, Candidacy, and Factors Affecting Outcomes

Question 1

What is the center of the FDA that is responsible for overseeing medical devices, sch as CIs and hearing aids?

Answer 1

The Center of Devices and Radiological Health (CDRH)
Regulated all companies that manufacture or import devices in the United States

Question 2

Are there several offices within the CDRH?

Answer 2

Yes, they are all responsible for different things

Question 3

What are the three classes of devices?

Answer 3

Class I - poses the least amount of risk to the patient and are subject to the least amount of regulatory controls
Class II - moderate safety risk, subject to a higher regulatory controls
Class III - poses the highest risk to the patient and subject to the highest level of regulatory control

Question 4

Is premarket approval required for class II and III medical devices?

Answer 4

Yes
The FDA requires this prior to marketing and/or commercially distributing the device

Question 5

What are the 4 steps of premarket approval?

Answer 5

Administrative and limited scientific review by FDA staff to determine completeness (acceptance and filing reviews)
In-depth scientific, regulatory, and Quality System review by appropriate FDA personnel (substantive review)
Review and recommendation by the appropriate advisory committee (panel review)
Final deliberations, documentation, and notification of the FDA decision

Question 6

What is the FDA review process for new CI devices?

Answer 6

Must submit a premarket approval application to the FDA
The ODE Ear, Nose, and Throat branch reviews the application
If further scientific review is deemed necessary, staff from the Office of Science and Engineering Laboratories (OSEL) will conduct additional assessments

Question 7

What is the FDA review process for existing CI devices (changes to manufacturing or quality control that impacts safety or efficacy)?

Answer 7

Must inform the Office of Compliance (OC) beforehand
The OC assesses the proposal and may request a scientific review by OSEL staff
May involve an FDA site visit to evaluate the new manufacturing or quality control

Question 8

Do the FDAs responsibilities extend beyond approval?

Answer 8

Yes
Manufacturers must continue collecting data on device performance post-PMA approval
To look at long-term outcomes
Done by post-approval studies, annual manufacturer reports, PMA supplement or amendment applications for significant design changes, mandatory adverse reporting (updated labeling, restrict use, and remove from market)

Question 9

What are examples of changes that require a PMA supplement (not a whole new application)?

Answer 9

New indication for use of the device
Labeling changes
The use of a different facility or establishment to manufacture, process, sterilize, or package the device
Changes in manufacturing facilities, methods, or quality control procedures
Changes in sterilization procedures
Changes in packaging
Changes in the performance or design specifications, circuits, components, ingredients, principles of operation, or physical layout of the device
Extension of the expiration date of the device based on data obtained under a new or revised stability or sterility testing protocol that has not been approved by FDA

Question 10

Are there multiple individuals part of a CI team and determining the candidacy of an individual?

Answer 10

Yes
Family
Surgeon
Audiologist
SLP
Deaf educator
Team coordinator
Social worker
Radiologists
Otologists
Neurotologist
Counselor/psychologist

Question 11

Who sets the audiologic criteria for cochlear implant candidacy?

Answer 11

The manufacturer submits a PMA application outlining indications for their device
FDA chooses to approve or reject the application - doesn’t set it themselves

Question 12

What are labeled indications?

Answer 12

Manufacturer-defined indications listed in the physician’s package insert
Some variability across the manufacturers for both adult and pediatric candidacy

Question 13

What are off-label indications?

Answer 13

Giving a CI to a patient that does not meet the approved indications set by the manufacturer
Has become common practice among CI programs in the US

Question 14

What does the FDA say about off-label usage?

Answer 14

They say that if clinicians recommend off-label use, they have the responsibility to ensure that they are well-informed about the product, base its use on firm scientific rationale and evidence, and maintain records of product’s use and effects

Question 15

What are the indications for use set by Cochlear Nucleus (company)?

Answer 15

18+ years
Bilateral pre, peri, or postlinguistic SNHL
Moderate to profound in the low frequencies and profound in the mid to high frequencies
Limited benefit from amplification (preop test scores of 50% or worse on the implanted side)

Question 16

Is there expanded criteria for the Cochlear Nucleus Hybrid (hearing aid and CI)?

Answer 16

Yes
18+ years old with normal to moderate HL in the low frequencies and severe to profound in the mid to high frequencies
Moderately severe to profound mid to high frequency hearing loss in the contralateral ear
Aided CNC word recognition score between 10% and 60% in the ear to be implanted
Contralateral CNC scores are equal or better than that of the ear to be implanted
*hearing aid stimulates the apical region - electrodes still extend down there but they are disabled

Question 17

What are the criteria for advanced bionics?

Answer 17

18+ years
Severe to profound bilateral SNHL
Limited benefits from appropriately fitting hearing aids (50% or less on a test of open set sentence recognition (HINT))

Question 18

What is the criteria for Med-El?

Answer 18

18+ years
Bilateral moderate to profound SNHL
LF PTA of 40 dB or greater and thresholds not better than 65 dB HL at 3-8 kHz
Limited benefits from appropriately fitted binaural hearing aids (50% correct or less in the ear to be implanted and 60% or less in the non-implanted ear on CNC test (monosyllabic words))

Question 19

Is HINT often used for assessing sentence recognition?

Answer 19

Not anymore
They are very easy
Could deny someone due to well performance
Use CNC or AZBio instead

Question 20

What is the criteria for the Med-El EAS (hybrid)?

Answer 20

Thresholds at or less than 65 dB HL through 500 Hz and at or greater than 70 dB HL for 2 kHz+
CNC scrore of less than or equal to 60% at 65 dB SPL in best aided condition
No rapid progressive HL
No ABG > 15 dB
No malformation or obstruction of the cochlea or external ear
No otosclerosis or ossification
Adequate motivation and expectations

Question 21

How does the EAS work?

Answer 21

Low-pitched sounds (HA) - sounds are detected by the mics of the audio processor, they are made louder and sent through the earmold to the ear and processed by the cochlea
High-pitched sounds (CI) - sounds are detected by the mics on the audio processor, high pitched sounds are sent to the implant as electrical signals, sounds are processed by the cochlea

Question 22

Does medicare often pay for CIs?

Answer 22

Yes, if they have patients that need them
They have their own candidacy criteria too

Question 23

What is the candidacy criteria for the centers for medicare/medicaid services (CMS)?

Answer 23

Diagnosis of bilateral moderate to profound SNHL
Limited benefit from appropriate hearing (or vibrotactile) aids (60% correct scores in the best aided listening condition on open-set sentence recognition tests)
Freedom from ME infection
Cochlear lumen that is structurally suited to implantation
Freedom from lesion in the auditory nerve and acoustic areas of the CNS
No contraindications to surgery
FDA-approved labeling

Question 24

Is CI candidacy more complicated in children?

Answer 24

Yes, with many more elements too
A 3-6 month hearing aid trail may be required in some centers for children with no previous hearing aid experience

Question 25

What is the candidacy criteria for Cochlear Nucleus for children who are 9 to 24 months?

Answer 25

Profound SNHL bilaterally Limited benefit from binaural amplification

Question 26

What is the candidacy criteria for children who are 2 to 17 years?

Answer 26

Severe to profound SNHL bilaterally Limited benefit from binaural amplification Younger children: lack of progress in the development of simple auditory skills in conjunction with appropriate amplification and intensive aural habituation over a 3 to 6 month period Older children: 30% or less correct on the open set Multisyllabic Lexical Neighborhood Test A 3-6 month hearing aid trial is recommended

Question 27

What is the criteria for children for Med-El?

Answer 27

12 months of age or older Profound bilateral SNHL with thresholds of 90 dB HL or greater at 1000 Hz Younger children: lack of progress in development of simple auditory skills in conjunction with appropriate amplificatione development of simple auditory skills in conjunction with appropriate amplification and intensive aural habituation over a 3 to 6 month period Older children: less than 20% correct on the open set Multisyllabic Lexical Neighborhood Test A 3-6 month hearing aid trial is required

Question 28

What are the criteria for advanced bionics for children?

Answer 28

Profound bilateral SNHL Use of appropriately fitted hearing aids for at least 6 months in children 2 through 17 yrs of age, or at least 3 months in children 12 through 23 months of age In younger children (<4 years of age): A failure to reach developmentally-appropriate milestones measured using the infant-toddler meaningful integration scale or <20% correct on a simple open-set word recognition test (Multisyllabic Lexical Neighborhood Test) administered using MLV (70 dB SPL) In older children (≥4 years of age): Scoring <12% on a difficult open-set word recognition test (Phonetically Balanced-Kindergarten Test) or <30% on an open-set sentence test (Hearing in Noise Test for Children) administered using recorded materials in the soundfield (70 dB SPL)

Question 29

Did the FDA lower the recommended age requirement for CIs?

Answer 29

It was lowered in 2002 to 12 month and again in 2020 to 9 months While this is the FDA-recommended age, this age is not legally binding and some hospital centers in clinical trials are completing the procedure earlier based on expectations of improved outcomes for early implantation, specific circumstances may allow for earlier implantation, such as meningitis

Question 30

What are burdens that are associated with SSD?

Answer 30

Hard to follow group conversations Can’t tell where sounds are coming from More mental effort needed to listen Exhaustion from listening effort Anxiety in noisy places Negative impact on relationships Difficulties at work and social gatherings Anxiety of losing hearing in good ear

Question 31

What are some benefits that can result in implanting someone with SSD?

Answer 31

Significantly better speech understanding in noise Easier to follow group conversations Improved sound localization Much less listening effort Reduced social isolation Improved quality of life

Question 32

What are the indications for implantation of SSD for the Cochlear Nucleus?

Answer 32

5+ years Unilateral hearing loss Severe to profound HL in the ear to be implanted Normal or near normal hearing in the contralateral ear Limited benefit from an appropriately fit unilateral hearing device (score of less than or equal to 5% on the CNC word test)

Question 33

What is the criteria for Med-El for SSD?

Answer 33

Profound sensorineural hearing loss in one ear and normal hearing or mild sensorineural hearing loss in the other ear Limited benefit from unilateral amplification (≤5% on monosyllabic CNC words in quiet when tested in the ear to be implanted alone) Kids: 5% or less on developmentally appropriate word lists Must have at least one month experience of wearing a cros or other device to show no subjective benefit

Question 34

What is the criteria for Med-El for asymmetrical hearing loss?

Answer 34

Profound sensorineural hearing loss in one ear and mild to moderately severe sensorineural hearing loss in the other ear, with a difference of at least 15 dB in pure tone averages (PTAs) between ears Limited benefit from unilateral amplification (≤5% on monosyllabic CNC words in quiet when tested in the ear to be implanted alone) Kids: 5% or less on developmentally appropriate word lists

Question 35

Can advanced bionics be used on patients with SSD?

Answer 35

Only in Europe Received CE mark

Question 36

Can establishing the precise etiology of deafness can provide useful information in guiding the implantation process?

Answer 36

Yes, especially when awareness of etiology is likely to affect strategies of the surgical implantation (meningitis and acoustic tumors)

Question 37

How is medical candidacy determined?

Answer 37

Full otological assessment General health/fitness for surgery Imaging of the cochlea anatomy with CT or MRI to determine if it is possible to insert the electrode array, confirm the auditory nerve is present and normal, and determine which ear may be the most suitable

Question 38

What are some absolute anatomic contraindications for CI implantation?

Answer 38

Absent cochlea/cochlear nerve Neurological damage impeding auditory processing Damaged auditory cortex Other anomalies

Question 39

What are absolute medical contraindications for CI implantation?

Answer 39

Medical condition(s) preventing surgery Medical risks of surgery exceed expected benefits

Question 40

What are all of the elements that are scanned during high resolution computed tomography of the temporal bone?

Answer 40

Pattern of mastoid pneumatization Position of facial nerve ME anatomy Position of the vascular structure Cochlear malformation Cochlear ossification Enlarged vestibular aqueduct Other inner ear and skull base abnormalities

Question 41

Does surgery often result in the complete elimination of the child's residual hearing in the implanted ear?

Answer 41

Sometimes Thus, if a recommendation to implant is made, one should be certain that the child’s hearing potential is greater with an implant than it would be if he or she continued to use a hearing aid We expect some damage, but it is better than it used to be Largely dependent on the experience of the surgeon

Question 42

What are some things to consider when deciding which ear to implant?

Answer 42

Anatomy (lack of calcification, presence or absence of the auditory nerve, malformed or absent cochlea) Perhaps one ear accepts electrical stimulation better than other Leave ear with better hearing, implant worse ear, then if not successful can revert back to aiding that ear Implant better ear (opposite argument)- It has already benefited from hearing aid, will more readily acclimate to implant Hand preference Occupation Facial nerve too close to cochlea- may pick other ear If no difference may want it on right- as speech and hearing centers of brain on left

Question 43

What are some contraindications for CIs?

Answer 43

A child who has significant residual hearing levels and receives good benefit from traditional hearing aid devices Congenital or acquired absence of the VIIIth cranial nerve Congenital absence of the labyrinth (Michel’s Aplasia) Adult patient (20 years old and older) with congenital/prelingual deafness who has never acquired speech Cognitive impairment that would prevent adequate rehabilitation (e.g., acquired dementia) - brain has to be able to do the work to restore the sound Lack of adequate support or motivation so as to ensure attendance at activation and programming sessions Active external or middle ear infections (relative contraindication) Known allergy and/or intolerance of device materials (i.e., medical grade silicone, platinum, and titanium)

Question 44

Is monitoring important when an implant is not initially recommended?

Answer 44

Yes because a recommendation to implant may be made at a later date if appropriate progress has not been made by the child Audiometric, speech and language, and speech perception data are used If this potential looks poorer than one would expect from a cochlear implant, a recommendation to provide the child with a cochlear implant should be made

Question 45

What are the variables that can affect CI outcome/benefit?

Answer 45

Age of the patient when they receive the implant Whether the hearing loss was present before or after the patient developed language skills Motivation of the patient and their family

Question 46

Is there high inter-subject variability of CI?

Answer 46

Yes, even in adults This variability cannot be fully explained or predicted by preimplant factors

Question 47

What are some factors that affect post-op outcomes with CI?

Answer 47

Duration of hearing loss Age of implantation Pre-op hearing status Etiology

Question 48

Do many studies show that the longer the patient has a hearing loss has a negative impact on CI outcomes?

Answer 48

Yes The poorer performance observed for recipients with a longer duration of deafness is most likely attributable to changes in the auditory nervous system (e.g., cross-modal plasticity and colonization of the auditory cortices) and auditory processing

Question 49

Do some studies show a trend of poorer performance in elderly patients?

Answer 49

Yes, slightly Other studies have showed comparable performance Poorer outcomes in elderly recipients could be due to changes in auditory processing. If so, the audiologist should be prepared to provide aural rehabilitation to assist the recipient in overcoming the negative effects of auditory processing difficulties. Also, the audiologist should counsel the recipient that additional time may be required to acclimate to the cochlear implant

Question 50

Is there a relation between preop pure tone thresholds and postop speech recognition with a CI?

Answer 50

No Many recipients who do not meet the current FDA indications for CIs perform very well with CIs

Question 51

Can etiology affect final implant outcome?

Answer 51

Yes Participants with sudden idiopathic hearing loss, Meniere’s disease, and a genetic cause of performed better than the average recipient Participants with temporal bone fracture, acoustic neuroma, and auditory neuropathy spectrum disorder (ANSD) performed more poorly than the typical recipient

Question 52

Is there a significant amount of variability observed in outcomes achieved by children with CIs?

Answer 52

Yes, and that variability cannot be entirely explained by any number of factors Research has also identified a host of factors that do generally tend to influence the outcome a child achieves after cochlear implantation

Question 53

Are there typically better outcomes obtained by children who receive CIs at an earlier age?

Answer 53

Yes There is strong evidence reporting better outcomes for children who undergo implantation prior to 12 months of age

Question 54

Are CI outcomes also significantly influenced by the mode of communication that is used by the child and the family?

Answer 54

Yes Auditory-Verbal Auditory-Oral Listening and Spoken Language American Sign Language Manually Coded English Total Communication Cued Speech

Question 55

Does the etiology of a child's hearing loss impact the outcomes achieved after implantation?

Answer 55

No, but some disorders were reported to influence outcomes after implantation Genetic hearing loss Aplasia/Deficiency of the Cochlear Nerve Abnormal Cochlear Anatomy Auditory Neuropathy Spectrum Disorder Ototoxicity Cytomegalovirus

Question 56

Is maternal education level positively associated with CI outcomes?

Answer 56

Yes

Question 57

Do children who do not regularly attend audiology appointments have poorer receptive language abilities and open-set word recognition?

Answer 57

Yes, compared to those who did regularly attend

Question 58

Do children whose families have a higher SES have better outcomes?

Answer 58

Yes

Question 59

Are there better outcomes for those who have longer hours of use?

Answer 59

Yes

Question 60

Does the presence of additional disabilities/comorbidities have a negative impact on the outcomes for children?

Answer 60

Yes

Question 61

Do children with some residual hearing prior to implantation have better outcomes?

Answer 61

Yes Favorable integrity of the peripheral and central auditory nervous system, smaller effect of auditory deprivation, greater potential to benefit from amplification prior to implantation Some studies have found no differences in the postimplant outcomes of children with and without preimplant residual acoustic hearing

Question 62

Are many children who are born with severe to profound hearing loss are able to derive considerable benefit from cochlear implantation?

Answer 62

Yes To optimize outcomes, children who are born with severe to profound hearing loss should ideally undergo cochlear implantation before 12 months of age

Question 63

What are the essential features for appropriate school placement?

Answer 63

Encouragement of spoken language, opportunities to interact verbally with adults and peers, appropriate support services and school personnel who understand a cochlear implant and are willing to participate in a team approach