Letter to the Editor

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950 LETFERS TO THE EDITOR

ences in opinion between various factions in deaf and hard-of-hearing habiitation.

In my opinion, collaborative efforts in the form of research and consultation on the part of those serving children with hearing

loss may be more important than getting submerged in aflurry of

action related to the implementation of newborn hearing screen-ing programs.

ANTON R. MILLER, MBCHB, FRCPC

Dept of Pediatrics, University of British Columbia and the Hearing Disorders Program

British Columbia’s Children’s Hospital

Vancouver British Columbia V6H 3V4

Canada

REFERENCES

I.Bess FH, ParadiseJL. Universal screening for infant hearing impairment: not simple, not risk-free, not necessarily beneficial, and not presently justified. Pediatrics. 1994;93:330-334

2. NIH Consensus Statement. Early Identification of Hearing Impairment in Infants and Young Children. March 1-3, 1993;11:1-24

3. Markides A. Age at fitting of hearing aids and speech intelligibility. Br

IAudio!. 198620:165-167

4. Sancho J, Hughes E, Davis A, Haggard M. Epidemiological basis for

screening hearing. In: McCormick B, ed. Pediatric Audiology 0-5 years. London: Taylor and Francis; 1988:4

5. Haggard MP. Hearing screening in children-state of the art(s). Arch Dis Child. 1990;65:1193-1195

6. Joint Committee on Infant Hearing 1990 Position Statement. AAO-HNS

Bulletin. March 1991:15-18

7. Sackett DL, Haynes RB, Guyatt GH, Tugwell P. Clinical Epidemiology: A

Basic Science for Clinical Medicine. 2nd ed. Boston: Little, Brown & Co; 1991:167

To the

Editor.-As the Surgeon General of the United States in 1989, I chal-lenged parents, physicians, state agency staff, and researchers to work together to find better ways to identify very young children

with hearing impairments. With that challenge, Iset a goal that by the year 2000, all children with significant hearing impairment would be identified before 12 months of age. Although it was an ambitious goal, I was optimistic that it could be accomplished. The last 5 years have demonstrated the feasibility of that goal, and it

has, in fact, been accomplished by people who were ready and eager to do something toward its achievement.

My challenge was necessary, because on average, children in

the United States with severe to profound hearing impairment are

not identified until approximately 2#{189}years of age-far too late. Children with milder but, nonetheless, significantly detrimental hearing losses are frequently not identified until they are 5 to 6 years of age. Over the last 40 years, dozens of governmental

commissions, task forces, and advisory groups have recom-mended immediate action, and millions of dollars have been spent

on hundreds of research and demonstration projects. Unfortu-nately, little-if any-progress was made until just recently.

It is in view of this recent progress that the article in Pediatrics by Bess and Paradise is so surprising and disturbing. The thinking reflected in that article is one reason why we have made so little

progress in achieving a goal that everyone agrees is important.

However, a wealth of recent research (virtually none of which was

referenced by the Bess and Paradise article) shows the direction in

which we need to go. Bess and Paradise are concerned about the

number of false positives and the attendant negative impact on those families. However, weighing against that are those thou-sands of infants each year who will be picked up early and have an opportunity for normal speech and language development. Admittedly, it is “not simple, [and] not risk-free” as pointed out by Bess and Paradise. But to say that it is “not necessarily bene-ficial, and not presently justified” ignores the accumulated

evi-dence of hundreds of research studies, the wisdom of thousands of practitioners, and the suffering of tens of thousands of people

with hearing loss who were not identified until they were 3 to 6 years old.

The National Institutes of Health Consensus Statement did not give the impression that a procedure is recommended that was

untested, impractical, costly, and not very effective. Such behavior

would be very unusual for a National Institutes of Health Con-sensus Panel. Programs using a two-stage newborn hearing

screening procedure have already been successfully implemented in many hospitals across the United States without the negative

consequences envisioned by Bess and Paradise. Certainly, further

improvements are possible. Progress in newborn hearing

screen-ing techniques will be made by people who are willing to use

existing knowledge to incrementally improve current practice. It is

dear that, as we continue working to refine and implement the techniques developed over the past 10 years, the goal many people

thought was impossible in 1989 can become a reality by the year 2000.

C. Evi-r Koop, MD

Bethesda, MD 20817-1129

REFERENCES

1. Bonfils P, UzielA, PujolR. Screening for auditory dysfunction in infants by evoked oto-acoustic emissions. Arch Otolaryngol Head Neck Surg.

1988;114:887-890

2. Kennedy CR, Kimm L, Dees DC, et al. Otacoustic emissions and

audi-tory brainstem responses in the newborn. Arch Dis Child. 1991;66: 1124-1129

3. Maxon AB, White KR, Vohr BR, Behrens TR. Using transient evoked otacoustic emissions for neonatal hearing screening. Br IAudiol. 1993;

27:149-153

4. Plinkert PK, Sestorhenn RA, Zenner HP. Evaluation of otacoustic emis-sions in high-risk infants by using an easy and rapid objective auditory screening method. Eur Arch Otorhinolaryngol. 1990247:355-360

5. Stevens JC, Webb HD, Hutchinson J,Connell J, Smith MF, Buffin JT. Click evoked otacoustic emissions compared with brainstem electric response. Arch Dis Child. 198964:1105-1111

6. White KR, Vohr BR, Maxon AB, Behrens TR, McPherson MG, Mauk GW. Screening all newborns for hearing loss using transient evoked otacoustic emissions. mt j Pediatr Otorhinolaryngol. In press

7. White KR, Vohr BR, Behrens TR. Universal newborn hearing screening

using transient evoked otacoustic emissions: results of the Rhode Island Hearing Assessment Project. Semin Hear. 1993;14:18-20

To the

Editor.-We thank Bess and Paradise, (1994)’ for bringing attention to some relevant issues related to universal newborn hearing

screen-ing. We note that their assertion that the effects of mild or

mod-erate temporary hearingloss are “entirely speculative and perhaps nonexistent” may be somewhat overstated (see, for example, Teele, Klein, Chase, Menyuk, Rosner and associates, 1990).2

The assertion that click-evoked auditory brainstem response

(ABR) may be used “. ..primarily to detect high-frequency

hear-ing loss” is not warranted and misleading. In normal auditory systems, it is true that the prime contributions to the morphology and latency characteristics of the waveforms comprising the ABR

seem to originate from the high-frequency (2 to 4 kHz) region of

the cochlea, due in part to greater synchrony of nerve-fiber firing.

In an impaired system, data suggest that high frequencies do not

define a unique generator site for the ABR, as patients with no

measurable hearing at frequencies >1500 Hz can be shown to have a click-evoked ABR. Thus, the click-evoked ABR, as used in a

screening context, may reflect best hearing and may miss sloping

losses of any kind.

The objection to universal newborn hearing screening on the basis of assumed theoretical noncompliance is not germane. The

compliance with any proposed law is not an inherent character-istic ofthat law, but if noncompliance prevails then the issue is one

of enforcement. The National Institutes of Health (NIH)

recom-mendation is just that-a recommendation, not a legal mandate, based on the presentations made to the NIH committee by the

professionals who testified.

The fact that some children will have difficulty being screened because of economic or logistic considerations should not

pre-dude the institution of a national goal. The same is true of vacci-nations, whereby the standing recommendation is that all children

should receive immunization, but it is clear that not all children

do. Moreover, there is no evidence that the economic or logistic problems will change appreciably from the child’s sixth month of life to his fifth year of life.

at Viet Nam:AAP Sponsored on September 1, 2020

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LETFERS TO THE EDITOR 951

TABLE 1. Results of First Hearing Screening on Univ ersal Basis

Hearin g Loss? Totals

Yes No Test Positive Screen Fail n Row % Column % 3 997 2.94 100.00 131 769 97.06 3.30 135 766 3.40 Test Negative Screen Pass n Row % Column % 0 0.00 0.00

3 861 234 100.00

96.70

3 861 234

96.60

Totals n

Row %

3 997 0.10

3 993 003 99.90

3 997 000.00

The pretense that children who are afflicted with congenital hearing loss of any degree do not need to hear very well during

infancy, but can be made to wait until “. ..later in childhood (eg,

6 months versus 18 months) ...“ is ill-considered in our opinion.

Without early intervention, these children indeed do not hear very

well, and the basic auditory foundations for the precursors of

speech and language that are typically acquired in infancy and that impact expressive (exempli gratia, reciprocal vocalization), receptive (eg, alerting to voice, recognition and lateral orientation to sound) skills and behaviors, are at best distorted.

Bess and Paradise’ have raised two issues of interest; one of which is simply the consequence of a basic mathematical calcula-lion and trivial only in the sense that it reiterates the obvious, the other being based mostly on philosophy is somewhat more diffi-cult to resolve. They note quite correctly the relationship between positive predictive value and the predictible (since the sum of the positive predictive value and false-alarm value is the number of patients with positive test findings-one variable is totally do-fined by the other two) false positive rate. Such a narrow focus can be used to generate a somewhat misleading interpretation of the value of any test or test battery. We note that although the NIH consensus statement does recognize the potential application of evoked otoacoustic emissions, it equally acknowledges the use of

ABR as the prime screening tool in those systems which have that availability. Indeed automated ABR has been shown to have a 100% sensitivity with a 96.7% specificity (Hall, Kileny, Ruth, and

Peters-Kripal, 1987). Table I illustrates the results of an auto-mated ABR screening that may be expected from a birth rate of 3997 000 per year.4

For the sake of minimizing the number of confounding

van-ables, these computations are based on the assumption (apparent-ly held by Bess and Paradise) that a prevalence of 0.1% accurately describes hearing losses of interest. By standard procedure, the 135 766 children who fail the first screening would be rescreened. Table 2 illustrates the results of the second screening.

Eight thousand three hundred forty-five children will have failed both screenings, 3997 of whom are afflicted with moder-ate (>35 dB HL) to severe hearing loss. For the sake of

argu-ment, let us claim that there is no other kind of hearing loss

worth detecting, and that by extension 4348 normal-hearing

children are misidentified as having a hearing problem.’ The

selective use of this statistic alone can make the universal screening look rather bad with a positive predictive value of 47.9%. Thus, for any given child who fails the screening pro-cess, and ignoring any other information, there is a slightly higher probability that the child is normal-hearing rather than hearing-impaired. Without the screening process, the probabil-ity of correct identification is 0.1 %. In that sense, the screening process yields a 480-fold improvement over the absence of such

a process. The emphasis on positive predictive value is partic-ularly relevant when one wishes to establish the presence of the disease. We can also choose the statistic that will improve the appearance of the universal screening by noting that the nega-tive predictive value is 100%, the sensitivity also is 100%, the false-alarm rate is 3.3%, the specificity is 96.7%, and the

uni-versal screening efficiency under these conditions is 96.8%,

,Inclusion of mild losses and even unilateral hearing loss will only serve to increase the prevalence and therefore improve the efficiency of the

screen-ing, and reduce the cost per capita, while possibly decreasing the

false-alarm rate as well as the sensitivity.

which is somewhat better than most audiologic test batteries used for differential site-of-lesion determination. The emphasis on negative predictive value is of particular relevance when one wishes to rule out the disease. A narrow focus on any single statistic, in combination with selected use of certain data, can be used to support diametrically opposed points of view, each with similar surface validity.

Bess and Paradise’ are not suggesting doing away with screening, but merely postponing it from neonatal to about 6 months. The cost savings incurred by that postponement are undear to us atthis time.

At a unit cost of $55.81 per screening, the cost of the first

screening test amounts to $223 072 570. The cost of the second screening test amounts to $7 577 100. The 8345 children who failed

both screenings will require follow-up threshold tests, which we estimate at $300, bringing the follow-up test costs to $2 503 500.

The total annual cost of the universal hearing screening is about $233 320 000. This brings the cost per identified hearing-impaired child to about $58 330. Another way to look at the cost of this universal hearing screening process is that it costs 90 cents per United States resident, based on a population of 258 104 000.

One consideration that remains incorporates a value judgment as to whether the benefits of early identification warrant the cost.

The 90 cents for every man, woman, and child brings temporary worry to the parents of 4348 children, and immediate assurance of having ruled out hearing loss for the parents of 3861 234 children.

Comfort is brought with a 30-day delay to the parents of an

additional 131 769 children who had to wait for the second screen-ing. We don’t know of any data that could demonstrate that the cost justifies or fails to justify this benefit.b

If there is no evidence to suggest direct benefits to society from early intervention secondary to identification of hearing loss, then by extension for those who believe that no action is justifiable until

unquestionable proof ofbenefits isestablished (rather than a prepon-derance of quasidrcumstantial evidence) the ethical course of action is precisely to do nothing. Under those circumstances, one would expect honest adherents to that belief not to provide services to children on the basis of their early identification, and not to charge for any consultation made on the basis of early identification. We know of no professional who will be honest enough with the parents of a child to notify them that a hearing problem has been discovered for which at least partial compensatory intervention exists (amplifica-tion) but that nothing will be done about it because published re-search is not condusive enough. As has been suggested before (Raf-fin and Matz, 1994), we agree with Bess and Paradise’s suggestion

that the best scientific study for this type of question would be one

that entails a double-blind design involving at least three matched

groups: nonintervention, placebo, and intervention. However, we

suggest that, pragmatically, this kind of study might be difficult to

implement because:

1. It would probably not be approved by any human-subject

review board, due in part to the possible adverse effects of

bIncomparison, tohighlight this society’s priorities, each enemy killed during the Viet Nam war (Duggins G. Report on Minority Affairs. The WA Veteran. 1994;14:9) cost $1 048 151. The budget deficit for 1993 alone ($290403 000000)

amounts to $1125.14 for every man, woman, and child4. The cummulative budget deficit amounted to $17603.24 for every man, woman, and child, and the annual interest alone on that deficit amounted to over $1000 for every man, woman, and child as of dose of work 28February 1994 (Jesse Helms, Testi-mony before the Senate of the United States, 1March 1994).

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TABLE 2. Results of Second Hearing Screening on Universal Basis

952 LE1TERS TO THE EDITOR

Hearin g Loss? Totals

Yes No Test Positive Screen Fail n Row % Column % 3997 47.89 100.00 4 348 52.11 3.30 8345 6.15 Test Negative Screen Pass n Row % Column % 0 0.00 0.00 127 421 100.00 96.70 127 421 93.85 Totals n Row % 3997 2.94 131 769 97.06 135 766.00

withholding known effective therapeutic intervention for

hearing loss;

2. It would bedifficult toimplement on thebasis of “informed consent” (since parents of such children could not possibly be informed as to

the nature of the impact ofwithholding intervention);

3. It might be difficult to support on an ethical basis (witness some

of the negative reactions to the placebo study of Bretlau and

colleagues, 1981);6

4. It may place the investigators and their affiliated

“deep-pock-ets” in a medically-legal difficult position. For example, one could envision a scenario whereby early intervention is shown

to provide some benefit. Then, the parents of those children for whom either a placebo or no intervention was given might interpret the absence of intervention to constitute a significant

infringement on their child’s abifity to pursue happiness (a right guaranteed by the Constitution) and could take the

inves-tigators to task-asserting any academic, behavioral, social, or

emotional difficulties thenceforth encountered are the result of

the absence of early intervention, given no absolute proof to the contrary-for a rather not-so-scientific compensation).

Under those conditions, the pure scientist may be viewed not so much as a person who enhances the knowledge that benefits

society, but may be viewed as something less than a hero for using children as guinea pigs in scientific experiments.

Bess and Paradise have raised some thought-provoking

per-spectives on universal neonatal hearing screening. We do not

believe that considerate caution should preclude the establish-ment of a national goal. In addition, we believe that the absence of

incontrovertible evidence detailing the benefits of any clinical treatment approach should not prevent the clinician from using the best available evidence to date (with all its imperfections

recognized) to formulate the most appropriate course of action for the care of patients in conjunction with his best clinical judgment.

MICHAEL J. M. Rinri, Pi-iD

Department of Audiology

Loyola University Medical Center

Maywood, IL

GREGORY J.MATZ, MD Department of Otolaryngology

Loyola University Medical Center

Maywood, IL

REFERENCES

1. Bess FH, ParadiseJL. Universal screening for infant hearing impairment: not simple, not risk-free, not necessarily beneficial, and not presently justified. Pediatrics. 199493:330-334

2. Teele DW, Klein JO, Chase C, Menyuk P, Rosner BA, and the Greater

Boston Otitis Media Study Group. Otitis media in infancy and intellec-teal ability, school achievement, speech, and language and age 7 years.

Iinfect Dis. 1990;162:685-694

3. HallJW, Kileny PR, Ruth RA, Peters-KripalJ. Newborn auditory

screen-ing with Algo-1 vs. conventional auditory brainstem. Paper presented at the American Speech-Language-Hearing Association Convention 1987, New Orleans, LA

4. Statistical Abstracts of the United States 1993-The National Data Book. United States Department of Commerce 1993, ISBN: 0-16-0420474

5. Raffin MJM, Matz GJ. Research design for early intervention. Audiology

Today 1994. In press

6. ThomsenJ, Bretlau P.Toss M,Johnsen NJ. Placebo effects in surgery for M#{233}ni#{232}re’sDisease. Arch Otolaryngol. 1981;107:271-277

To the

Editor.-The importance of early identification of hearing loss is sum-marized in the report issued by the United States (US) Department of Health and Human Services,’ entitled “Healthy People 2000.” The goal is to reduce the average age at which children with significant hearing impairment are identified to no more than 12

months. The report states:

The future of a child born with significant hearing impairment

depends to avery large degree on early identification (ie, audio-logical diagnosis before 12 months of age) followed by

immedi-ate and appropriate intervention. If hearing-impaired children

are not identified early, it is difficult, ifnot impossible, for many

of them to acquire the fundamental language, social, and cogni-tive skills that provide the foundation for later schooling and

success in society. When early identification and intervention occur, hearing-impaired children make dramatic progress, are

more successful in school, and become more productive

mem-bers of society. The earlier intervention and habilitation begins, the more dramatic the benefits (p. 460).

On the average, children in the US with severe to profound

hearing impairment are not identified until 24 to 30 months of age,

while children with milder, yet nonetheless, significantly

detri-mental hearing losses frequently are not identified until they are 5 to 6 years of age.’ It is also alarming that only about 3% of all

children born in the US participate in any type of newborn

hear-ing-screening program.3 Thejoint Committee on Infant Hearing is

in the process of revising their position on infant screening. Their

1990 Position Statement recommended hearing screening only for

infants who exhibit one or more of ten risk factors for hearing

loss.4 However, recent research has revealed that as many of half

of all children with bilateral severe-to-profound hearing losses have not exhibited any of the high-risk factors.54

The September 28, 1993 draft of the Joint Committee on

Infant Hearing Position Statement recognizes these findings

and states:7

Because normal hearing is critical for speech and oral

lan-guage development as early as the first six months of life,

hearing loss ininfants must be identified before three months of age. To acquire access to most infants, the Joint Committee on Infant Hearing recommends screening infants prior to hospital discharge from the newborn nursery.

... a method of hearing screening must be able to identify infants with hearing losses of 30 dB HL and greater in one or

both ears, in the frequency region important for speech

recog-mtion (approximately 500 through 4000 Hz). Of the various approaches to newborn hearing screening currently available, two physiologic measures [auditory brainstem response (ABR) and otoacoustic emissions (OAE)l most nearly achieve this goaL

In addition to the National Institutes of Health Consensus

Statement in support of universal hearing screening,8 it is clear

that many professionals from different disciplines now believe the

time has come to support universal hearing screening. Much of the

recent support has come as a result of studies showing that OAEs

are capable of identifying infants with hearing loss of

approxi-mately 30 dB HL and greater.93

I have personal admiration for Doctors Bess and Paradise and

their long-standing contributions to our understanding of effects of hearing disorders on children. However, I must express concern

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1994;94;950

Pediatrics

Michael J. M. Raffin and Gregory J. Matz