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J Neurosurg Pediatrics 8:396–401,

8:000–000, 2011

Comparison of Hydrocephalus Outcome Questionnaire


scores to neuropsychological test performance in
school-aged children
Clinical article

Abhaya V. Kulkarni, M.D., Ph.D.,1 Ruth Donnelly, Ph.D., 2


and Iffat Shams, M.B.B.S., M.P.H.1

Divisions of 1Neurosurgery and 2Psychology, The Hospital for Sick Children, University of Toronto, Ontario,
Canada

Object. The Hydrocephalus Outcome Questionnaire (HOQ) is an established means of measuring quality of life,
but the cognitive component of this questionnaire has never been formally compared with gold-standard neuropsy-
chological test scores. The authors hypothesized that the HOQ Cognitive Health score would demonstrate a relatively
strong correlation with neuropsychological test scores, whereas much weaker correlations would be seen for HOQ
Physical and Social-Emotional Health scores.
Methods. A cross-sectional study of children with long-standing hydrocephalus presenting to The Hospital for
Sick Children’s Neurosurgery Clinic was performed between July 2006 and September 2008. Participating children
and families completed the HOQ and a battery of 21 standard neuropsychological tests and questionnaires. Pearson
correlation analysis was then performed.
Results. A total of 83 patients (81% participation) was accrued; the mean age was 11.5 ± 3.4 years (mean ± SD)
at the time of assessment. The mean age at hydrocephalus treatment was 1.3 ± 2.6 years. The mean overall HOQ score
was 0.69 ± 0.21. The HOQ Cognitive score had a moderate or strong correlation with 19 (90%) of 21 neuropsycho-
logical test scores, much more so than the HOQ Social-Emotional score (5 moderate or strong correlations, 24%) and
the HOQ Physical score (1 moderate correlation, 5%). For 19 neuropsychological tests (90%), the HOQ Cognitive
score had a stronger correlation than the other scores. The HOQ Cognitive score had particularly strong correlations
with the Verbal IQ, List Learning, Behavior Problems, and Metacognitive Abilities components.
Conclusions. Data from a wide-ranging representative sample of children with long-standing hydrocephalus
provide added evidence of the validity of the HOQ Cognitive score and the overall domain structure of the HOQ
itself. (DOI: 10.3171/2011.7.PEDS1179)

Key Words      •      pediatric hydrocephalus      •      health outcome      •      quality of life      •     


neuropsychology

H
ealth-related quality of life in childhood hy- as a particularly relevant component for children with
drocephalus is now recognized as an important hydrocephalus. The HOQ includes a cognitive health
outcome to consider. We have previously devel- component, for example, consisting of 12 questions. The
oped the HOQ, which has provided a simple, reliable, gold standard for objectively measuring cognitive perfor-
and valid way in which to measure HRQL in children mance has long been through the use of neuropsycho-
with hydrocephalus.17,18 Our group and others have since logical tests, including traditional IQ testing and other
used the HOQ in several research studies of HRQL.3,14– specific tests. We embarked on a study to assess detailed
16,19,20,22
Within the spectrum of HRQL, cognition is seen objective neuropsychological performance in a diverse
group of children with treated hydrocephalus, and we
compared these results to those obtained from the more
Abbreviations used in this paper: HOQ = Hydrocephalus Out-
come Questionnaire; HRQL = health-related quality of life; WAIS-
subjective parent responses to questions from the HOQ.
III = Wechsler Adult Intelligence Scale–Third Edition; WISC-IV = We hypothesized that, although there might be areas for
Wechsler Intelligence Scale for Children–Fourth Edition; WPPSI-III which the HOQ scores mirror neuropsychological test re-
= Wechsler Preschool and Primary Scale of Intelligence–Third Edi- sults, there would probably also be areas for which these
tion; WRAT-3 = Wide Range Achievement Test–Third Edition. measures would provide unique information. We hypoth-

396 J Neurosurg: Pediatrics / Volume 8 / October 2011


Neuropsychological outcome in children with hydrocephalus

esized that the HOQ Cognitive Health score in particu- We chose tests from the clinical test battery given
lar would demonstrate the strongest correlation with the to children with hydrocephalus who are referred to the
neuropsychological tests, whereas much weaker correla- Psychology division from the Neurosurgery division at
tions would be seen for HOQ Physical and Social-Emo- The Hospital for Sick Children. These tests had to either
tional Health scores. The HOQ Cognitive score questions encompass the entire age range of this study (5–18 years),
mainly revolve around parents’ appraisal of their child’s or they had to have upward or downward extensions with
learning, memory, and elements of the Executive Func- similar formats and comparable outcome measures. A
tion component such as organization and attention. If our few words are in order here to explain the intelligence
hypotheses were correct, then that would add confidence testing scores. We used different Wechsler intelligence
to the validity of the HOQ Cognitive score and the pro- tests depending on the age of the child: the WPPSI-III;27
posed domain structure of the HOQ itself. the WISC-IV;25 and the WAIS-III.24 However, to have
comparable scores among these 3 tests, we used 3 mea-
sures from each Wechsler test: the Verbal IQ Index score,
Methods the Nonverbal IQ Index score, and the Processing Speed
IQ Index score. Each index score is summed over several
We recruited a consecutive sample of children be- subtests within each domain of verbal and nonverbal in-
tween the ages of 5 and 18 years, in whom hydrocephalus tellectual abilities and processing speed. We did not use
had been diagnosed and treated at least 6 months earlier, the Working Memory Index scores from the WAIS-III
from the neurosurgery outpatient clinic of The Hospital and the WISC-IV because there is no comparable index
for Sick Children, Toronto, between July 2006 and Sep- score on the WPPSI-III and also because the WAIS-III
tember 2008. These children had to have adequate skills Working Memory score includes a mental arithmetic sub-
in English to complete testing, as evidenced by at least 2 test, and there is no similar subtest as part of the WISC-
years of English schooling. Children who had undergone IV Working Memory score.
either a neuropsychological or psychological-educational
Most of the tests that we used have more than 1 out-
assessment within 1 year prior to participating in this
come score. We selected the specific scores a priori that
study were excluded, because this could have resulted in
we thought best reflected the child’s performance in the
artificially inflated scores. This protocol was approved by
particular area of interest. The full names of the tests and
the hospital’s research ethics board, and written consent
the particular score or scores that we used from each test
and assent were obtained from all participating families.
can be found in the Appendix.
Assessment of HRQL For the first 18 scores in this listing, higher values
indicate stronger performance by the child. The last 3
The child’s primary caregiver was asked to complete scores listed are the results from 2 different parent ques-
the HOQ. The HOQ is a 51-item questionnaire with prov- tionnaires, and higher scores indicate more problems in
en reliability and validity in measuring health outcome the areas queried. One score is from a behavioral ques-
in children with hydrocephalus.17,18 We have previously tionnaire,1 in which parents answered questions relat-
shown good correlations between the HOQ and several ing to their child’s possible difficulties in areas such as
independent measures of health,17 including the WRAT’s anxiety, depression, social problems, attention problems,
Reading subtest,29 Strengths and Difficulties Question- and aggression. The other 2 scores are from an Execu-
naires,12,13 and Functional Independence Measure for tive Function questionnaire,11 in which parents answered
Children,21 but we have not compared HOQ results to questions about their child’s everyday functioning in ar-
performance on neuropsychological tests. The HOQ pro- eas such as control of behaviors (behavioral regulation
vides scores of Overall Health, Physical Health, Cognitive score), and planning, organizing, self-monitoring, and
Health, and Social-Emotional Health, all of which range paying attention (metacognitive abilities score).
from 0 (worse outcome) to 1.0 (better outcome). Previous
work has suggested that, to be clinically meaningful, a Statistical Analysis
difference in HOQ scores needs to be approximately on The Pearson correlation was used to assess the asso-
the order of 0.10 or greater, based on how parents per- ciation between the 3 HOQ subscores (Cognitive, Physi-
ceive differences in health status.14 cal, and Social-Emotional) and each test of neuropsycho-
logical function. Similar to the suggestion by Cohen4 and
Assessment of Neuropsychological Performance others,6 we defined a strong correlation as > 0.5, moderate
Children were given a battery of neuropsychological as 0.35–0.5, and weak/negligible as < 0.35.
tests by a trained psychological assistant and supervised We hypothesized that HOQ Cognitive Health scores
by an experienced pediatric neuropsychologist. The tests would have mostly strong or moderate correlation with
involved were the core tasks from a standard neuropsy- any of the first 18 measures of neuropsychological func-
chological evaluation and were an intensive and compre- tion listed above, whereas the HOQ Physical and Social-
hensive assessment of the child’s cognitive functioning. Emotional Health scores would demonstrate mostly weak/
These are listed in Table 1 and in the Appendix, and all negligible correlation.
are widely used and well-standardized instruments. The For the last 3 measures listed, higher scores indicate
testing lasted approximately 5 hours and included tests of poorer performance in these areas. Because a higher
IQ, language, academics, memory, visual-motor and visu- score on the HOQ indicates better outcome, we expected
al-spatial skills, and visual information processing speed. a relatively strong negative correlation between the Be-

J Neurosurg: Pediatrics / Volume 8 / October 2011 397


A. V. Kulkarni, R. Donnelly, and I. Shams
TABLE 1: Neuropsychological testing results

Pearson Correlation w/
Patient Test Population HOQ Cognitive HOQ Physical HOQ Social-Emotional
Test* Scores† Scores† Score Score Score
Verbal IQ index 93 ± 17 100 ± 15 0.54‡ 0.38 0.33
Nonverbal IQ index 87 ± 18 100 ± 15 0.43 0.32 0.25
Processing Speed IQ index 84 ± 17 100 ± 15 0.44 0.45 0.31
PPVT 96 ± 16 100 ± 15 0.45 0.30 0.25
EOWPVT 95 ± 16 100 ± 15 0.49 0.27 0.31
Ambiguous Sentences 8±4 10 ± 3 0.41 0.18 0.26
Oral Comprehension 99 ± 13 100 ± 15 0.44 0.23 0.24
Letter-Word ID 91 ± 21 100 ± 15 0.48 0.17 0.18
Calculation 83 ± 27 100 ± 15 0.50 0.27 0.27
Passage Comprehension 85 ± 19 100 ± 15 0.48 0.24 0.27
Stories Delayed 9±4 10 ± 3 0.38 0.26 0.39
Faces Delayed 9±4 10 ± 3 0.28 0.17 0.04
Design Memory 7±3 10 ± 3 0.43 0.25 0.20
Sentence Memory 10 ± 3 10 ± 3 0.44 0.17 0.24
List Learning 44 ± 15 50 ± 10 0.58‡ 0.30 0.36
List Memory Delayed −1 ± 1 0±1 0.47 0.17 0.32
Beery VMI 83 ± 17 100 ± 15 0.36 0.33 0.27
Spatial Relations 92 ± 14 100 ± 15 0.29 0.25 0.03
Behavior Problems 58 ± 12 50 ± 10 −0.53‡ −0.43 −0.53‡
Behavioral Regulation 57 ± 14 50 ± 10 −0.39 −0.29 −0.47
Metacognitive Abilities 62 ± 13 50 ± 10 −0.57‡ −0.31 −0.4

* See Appendix for test details. Abbreviations: EOWPVT = Expressive One-Word Picture Vocabulary Test; PPVT = Peabody
Picture Vocabulary Test; VMI = Visual-Motor Integration.
†  Values in the 2nd and 3rd columns represent the mean ± SD for the patient and test populations, respectively.
‡  Strong correlations (> 0.5).

havior Problems and the Social-Emotional HOQ scores. a tumor, but none had received radiotherapy or chemo-
We hypothesized that the last 2 measures listed (Be- therapy. The mean HOQ scores were as follows: Overall
havioral Regulation and Metacognitive Abilities) would Health (0.69 ± 0.21), Cognitive Health (0.57 ± 0.29), Phys-
have at least moderate negative correlations with both ical Health (0.79 ± 0.19), and Social-Emotional Health
the HOQ Social-Emotional and Cognitive scores. These (0.74 ± 0.19).
measures of executive function encompass such a broad The results of the 21 neuropsychological measures
range of loosely defined abilities (such as initiation, plan- and their correlations with the HOQ scores are shown in
ning, organizing, attention, and self-monitoring) that it Table 1. An in-depth analysis and discussion of the pat-
seems likely that both emotional and cognitive aspects tern of the neuropsychological test results is beyond the
of a child’s functioning will be affected by strengths or scope of this paper and will not be attempted here, but
weaknesses in these areas. instead will be presented in a separate work. A summary
All analyses were performed using the SPSS Ad- of the strengths of correlations between the neuropsycho-
vanced Statistics software package (version 17.0; SPSS, logical scores and HOQ scores is shown in Table 3. In
Inc.). general, the HOQ Cognitive score had a greater number
of strong correlations than either the HOQ Physical or So-
cial-Emotional scores. Also, for every test except Behav-
Results ioral Regulation, the HOQ Cognitive score had a stronger
A total of 103 patients and their families were ap- correlation than the other scores.
proached to participate in this study, of whom 83 (81%)
agreed to enroll. The most common reason for refusal to Discussion
participate was lack of available time to complete the in-
tensive neuropsychological testing. The characteristics of In a wide-ranging representative sample of children
the participants are shown in Table 2. The school grade with long-standing, treated hydrocephalus, our study
level of the children ranged from senior kindergarten to showed that the HOQ Cognitive score had a strong cor-
Grade 12. Five children had hydrocephalus secondary to relation with several tests of neuropsychological function.

398 J Neurosurg: Pediatrics / Volume 8 / October 2011


Neuropsychological outcome in children with hydrocephalus
TABLE 2: Characteristics of 83 patients with long-standing TABLE 3: Summary of strengths of correlations between HOQ
hydrocephalus scores and neuropsychological tests

Characteristic Value* Correlation (no. of tests, %)


mean age at assessment 11.5 ± 3.4 yrs Test Component Strong Moderate Weak/Negligible
mean age at 1st op for hydrocephalus 1.3 ± 2.6 yrs HOQ Cognitive 4 (19) 15 (71) 2 (10)
no. w/ ETV as primary procedure 9 (10.8) HOQ Physical 0 3 (14) 18 (86)
no. w/ cause of hydrocephalus HOQ Social-Emotional 1 (5) 4 (19) 16 (76)
  posterior fossa cyst 14 (16.9)
  IVH due to prematurity 12 (14.5) score would be very highly correlated with all or even
  aqueductal stenosis 11 (13.3) most neuropsychological tests. After all, we know that
 myelomeningocele 10 (12.0) when we report results from objective tests, we are com-
  trauma/spontaneous hemorrhage 6 (7.2) paring an individual child against comprehensive norms
collected from large numbers of same-age or same-grade
  congenital communicating hydrocephalus 6 (7.2)
children. Therefore, when we say that, based on these ob-
 tumor 5 (6.0) jective results, a child is “within the average range” on
 postinfectious 4 (4.8) a test in which the scores are normally distributed, we
  other cyst 4 (4.8) mean that the child falls within the middle 50% of a rep-
  other cause 11 (13.3) resentative sample of children. However, when a parent
mean total days in hospital for initial treatment of hy- 16.2 ± 19.8 completes the HOQ, they are asked to say how true each
 drocephalus statement is for their child, with no comparison group
specified. We do not know to what extent parent evalua-
mean total days in hospital for hydrocephalus compli- 18.4 ± 28.5
tions could be affected by extrinsic factors such as their
 cations
general expectations for children who have hydrocepha-
*  The mean values are presented ± SD; the remaining values are ex- lus, or whether they think their child is really trying, even
pressed as the number of patients, with the percentage in parentheses. though they might have difficulty in some specific areas.
Abbreviations: ETV = endoscopic third ventriculostomy; IVH = intraven- We conceptualize the HOQ to be measuring aspects of
tricular hemorrhage. quality of life, so we do not see it as a substitute for neu-
ropsychological tests, which generally are tests of func-
tion. Because the HOQ measures the effect of function on
Particularly strong correlations were shown with the Ver- quality of life, although the concepts being measured are
bal IQ, List Learning, Behavior Problems, and Metacog- related, they are complementary rather than redundant.
nitive Abilities components. We had previously shown a Our study adds further confirmation of the validity
strong correlation (0.59, Pearson test) between the HOQ of the HOQ structure and the HOQ Cognitive score in
Cognitive score and the WRAT-329 in a sample of 90 chil- particular. Aside from showing the strong correlations of
dren with hydrocephalus as part of the initial validation HOQ Cognitive score with neuropsychological tests, it
of the HOQ.17 Only the Reading subtest of the WRAT-3 is equally important that we have shown a much weaker
was used in that study, because it requires the least physi- correlation between almost all of these tests and the HOQ
cal ability from the children (the other subtests, Arithme- Physical and Social-Emotional scores. This suggests that
tic and Spelling, have a written component), and it had these other scores are in fact measuring something unique
the highest test–retest reliability (0.93) of all the subtests, and are not providing redundant, highly correlated infor-
along with excellent internal consistency (a = 0.91).29 It mation. This adds validity to the hypothesized domain
was acknowledged, however, that the Reading subtest of structure of the HOQ.
the WRAT-3 was a limited test of cognition. This sub- We envision the primary use of the HOQ as a re-
test from the WRAT-3 just requires the child to decode search tool for the reliable measurement of quality of life
words, with no test of whether the words are understood, outcome in children with hydrocephalus. Although we
and is almost identical in form to the Letter-Word ID test are now expanding its use to routine clinical monitoring
we used in the current study. Therefore, we had yet to of patients, its role in clinical management remains to be
show that the HOQ Cognitive score was a valid measure determined. As we gain more experience in the clinical
of wider, more comprehensive aspects of cognition. That setting, we hope the HOQ might be able to help identify
was the primary purpose of this study: to assess the valid- patients who would benefit from more detailed neuropsy-
ity of the HOQ Cognitive score by using a wide range of chological testing. Again, we do not see the HOQ as a
standardized neuropsychological tests, and to determine substitute for neuropsychological tests.
whether the HOQ Cognitive score provided information We acknowledge several limitations in our work. First,
unique from the other HOQ subscores (Physical and So- despite this being one of the largest neuropsychological
cial-Emotional scores). Our results suggest that the HOQ studies of children with hydrocephalus, our sample size
Cognitive Health score is uniquely correlated with several remains relatively small. It is possible that, with more par-
well-established measures of cognition and is thus unique ticipants, we might have discovered stronger correlations
from HOQ Physical or Social-Emotional Health scores. in other areas. A second limitation is that ours is only a
It was not expected that the HOQ Cognitive Health single-center experience. Further tests of validation in oth-

J Neurosurg: Pediatrics / Volume 8 / October 2011 399


A. V. Kulkarni, R. Donnelly, and I. Shams

er centers will be important to prove the reproducibility of Spatial Relations. Subtest from the Woodcock-Johnson III
our work. Tests of Cognitive Abilities.31 Ability to manipulate shapes mentally
but with no visual-motor component.
Behavior Problems. Child Behavior Checklist for Ages 6–18.1
Conclusions Behavioral questionnaire filled out by the parent; Total Problems
score.
In a wide-ranging representative sample of children Behavioral Regulation. Behavior Rating Inventory of Execu­
with long-standing hydrocephalus, the HOQ Cognitive tive Function11 (BRIEF); behavioral questionnaire filled out by the
Health score demonstrated mostly strong and moderate parent. Overall rating of child’s ability to inhibit or shift behavior and
correlations with a number of tests of neuropsychologi- to control emotions.
cal function. The HOQ Physical and Social-Emotional Metacognitive Abilities. BRIEF; behavioral questionnaire
filled out by the parent. Overall rating of the child’s ability to initiate,
Health scores demonstrated much weaker correlations. to plan and organize, to self-monitor, and to pay attention.
These data provide added evidence of the validity of the
HOQ Cognitive score and the overall domain structure of
Disclosure
the HOQ itself.
The authors report no conflict of interest concerning the mate-
Appendix rials or methods used in this study or the findings specified in this
Details of tests and scores used in this study. paper. Funding for this study was provided by the SickKids Founda-
Verbal IQ index. For ages 5 years–5 years 11 months, WPPSI- tion/Institute for Human Development, Canadian Institutes of Health
III Verbal IQ score; for ages 6 years–15 years 11 months, WISC-IV Research, and the SickKids Research Institute. The funding organi-
Verbal Comprehension Index score; for ages ≥ 16 years, WAIS-III zations were completely independent of the design and conduct of
Verbal Comprehension Index score. the study; collection, management, analysis, and interpretation of the
Nonverbal IQ index. For ages 5 years–5 years 11 months, data; and preparation, review, and approval of the manuscript.
WPPSI-III Performance IQ score; for ages 6 years–15 years 11 Author contributions to the study and manuscript prepara-
months, WISC-IV Perceptual Reasoning Index score; for ages ≥ 16 tion include the following. Conception and design: all authors.
years, WAIS-III Perceptual Organization Index score. Acquisition of data: all authors. Analysis and interpretation of
Processing Speed IQ index. For ages 5 years–5 years 11 data: Kulkarni, Donnelly. Drafting the article: Kulkarni, Donnelly.
months, WPPSI-III Processing Speed score; for ages 6 years–15 Critically revising the article: all authors. Reviewed submitted ver-
years 11 months, WISC-IV Processing Speed Index score; for ages sion of manuscript: all authors. Approved the final version of the
≥ 16 years, WAIS-III Processing Speed Index score. manuscript on behalf of all authors: Kulkarni. Statistical analysis:
PPVT. Peabody Picture Vocabulary Test–Third Edition;9 Kulkarni. Study supervision: Kulkarni.
receptive language vocabulary test.
EOWPVT. Expressive One-Word Picture Vocabulary Test– Acknowledgments
Revised (2000 edition);10 expressive language vocabulary test.
Ambiguous Sentences. Subtest from the Test of Language The authors thank Margaret Wilkinson and Patricia Arseneau
Competence–Expanded Edition;28 higher-order language test. for their invaluable assistance in administering and scoring the neu-
Oral Comprehension. Subtest from the Woodcock-Johnson ropsychological tests.
III Tests of Achievement30 (WJ-III); understanding orally presented
sentences. Ethical Approval
Letter-Word ID. Subtest from the WJ-III; reading individual
words. Approval was obtained from the Institutional Review Board of
Calculation. Subtest from the WJ-III; test of math calculations. The Hospital for Sick Children, Toronto.
Passage Comprehension. Subtest from the WJ-III; under-
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