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Hearing loss and quality of life in survivors of paediatric CNS tumours and other cancers

Authors:
  • Bayerisches Landesamt für Pflege
  • University Children's Hospital Bern Inselspital

Abstract

Purpose Hearing loss, a complication of cancer treatment, may reduce health-related quality of life (HRQoL), especially in childhood cancer survivors of central nervous system (CNS) tumours who often have multiple late effects. We examined the effect of hearing loss on HRQoL in young survivors of CNS and other childhood cancers. Methods Within the Swiss Childhood Cancer Survivor Study, we sent questionnaires about hearing loss and HRQoL (KIDSCREEN-27) to parents of survivors aged 8–15 years. We stratified the effect of hearing loss on HRQoL by cancer diagnosis, using multivariable logistic regression and adjusting for sociodemographic and clinical factors. Results Hearing loss was associated with impaired physical well-being [unadjusted estimated differences − 4.6 (CI − 9.2, − 0.1); adjusted − 4.0 (CI − 7.6, − 0.3)] and peers and social support [unadjusted − 6.7 (CI − 13.0, − 0.3); adjusted − 5.0 (CI − 10.5, 0.9)] scores in survivors of CNS tumours (n = 123), but not in children diagnosed with other cancers (all p-values > 0.20, n = 577). Conclusion Clinicians should be alert to signs of reduced physical well-being and impaired relationships with peers. Especially survivors of CNS tumours may benefit most from strict audiological monitoring and timely intervention to mitigate secondary consequences of hearing loss on HRQoL.
Vol.:(0123456789)
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Quality of Life Research
https://doi.org/10.1007/s11136-018-2021-2
BRIEF COMMUNICATION
Hearing loss andquality oflife insurvivors ofpaediatric CNS tumours
andother cancers
AnnetteWeiss1,2 · GritSommer1· ChristinaSchindera1,3· LauraWengenroth4· AxelKarow3· ManuelDiezi5·
GiselaMichel6 · ClaudiaE.Kuehni1,3 · Swiss Paediatric Oncology Group (SPOG)
Accepted: 2 October 2018
© Springer Nature Switzerland AG 2018
Abstract
Purpose Hearing loss, a complication of cancer treatment, may reduce health-related quality of life (HRQoL), especially
in childhood cancer survivors of central nervous system (CNS) tumours who often have multiple late effects. We examined
the effect of hearing loss on HRQoL in young survivors of CNS and other childhood cancers.
Methods Within the Swiss Childhood Cancer Survivor Study, we sent questionnaires about hearing loss and HRQoL (KID-
SCREEN-27) to parents of survivors aged 8–15years. We stratified the effect of hearing loss on HRQoL by cancer diagnosis,
using multivariable logistic regression and adjusting for sociodemographic and clinical factors.
Results Hearing loss was associated with impaired physical well-being [unadjusted estimated differences − 4.6 (CI − 9.2,
0.1); adjusted − 4.0 (CI − 7.6, − 0.3)] and peers and social support [unadjusted − 6.7 (CI − 13.0, − 0.3); adjusted − 5.0
(CI − 10.5, 0.9)] scores in survivors of CNS tumours (n = 123), but not in children diagnosed with other cancers (all p-val-
ues > 0.20, n = 577).
Conclusion Clinicians should be alert to signs of reduced physical well-being and impaired relationships with peers. Espe-
cially survivors of CNS tumours may benefit most from strict audiological monitoring and timely intervention to mitigate
secondary consequences of hearing loss on HRQoL.
Keywords Childhood cancer· Ototoxicity· Swiss Childhood Cancer Survivor Study· Swiss Childhood Cancer Registry·
Late effects· Cancer treatment
Introduction
Hearing loss, especially in the high frequencies, is an adverse
event of childhood cancer treatment, particularly after plati-
num chemotherapy or cranial radiation 30 Gray [1]. It is
often irreversible and can be uni- or bilateral [1]. Hearing
impaired children in the general population have lower health-
related quality of life (HRQoL) in domains of school activities
and social interactions, which are important for learning [2, 3].
The only study on hearing loss and HRQoL in young child-
hood cancer survivors is published more than 10years ago [4],
while cancer treatments constantly change. The study focused
on patients diagnosed with neuroblastoma (1989–1995)
Electronic supplementary material The online version of this
article (https ://doi.org/10.1007/s1113 6-018-2021-2) contains
supplementary material, which is available to authorized users.
* Claudia E. Kuehni
claudia.kuehni@ispm.unibe.ch
1 Swiss Childhood Cancer Registry, Institute ofSocial
andPreventive Medicine, University ofBern, Mittelstrasse
43, 3012Bern, Switzerland
2 Department forEpidemiology andPreventive
Medicine/Medicine Sociology, University ofRegensburg,
Franz-Josef-Strauss-Allee 11, 93053Regensburg, Germany
3 Division ofPediatric Hematology/Oncology, Department
ofPediatrics, Inselspital, Bern University Hospital,
University ofBern, Freiburgerstrasse 15, 3010Bern,
Switzerland
4 Institute andOutpatient Clinic forOccupational, Social
andEnvironmental Medicine, University Hospital ofMunich
(LMU), 80336Munich, Germany
5 Paediatric Haemato-Oncology Unit, Department
ofPaediatrics, University Hospital Lausanne (CHUV), Rue
du Bugnon 46, 1011Lausanne, Switzerland
6 Department ofHealth Sciences andHealth Policy, University
ofLucerne, Frohburgstrasse 3, 6002Lucerne, Switzerland
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without factoring in other comorbidities [4]. While hearing
loss can pose difficulties for any child, survivors treated for
central nervous system (CNS) tumours may suffer more from
hearing loss since they often have multiple late effects which
may reduce their social and educational skills [5]. Recently,
oncologists have become more aware of ototoxicity [68]. Sur-
vivors should be carefully monitored for audiological prob-
lems and offered timely and targeted interventions to help them
cope with hearing loss [9]. Audiological monitoring has not
yet been fully implemented for survivors in follow-up care
in Switzerland [8] and there are, currently, little data on the
effects of hearing loss on childhood cancer survivors.
We thus undertook a nationwide, population-based study
to examine the impact of hearing loss on HRQoL in children
who were recently treated for childhood cancers (1995–2010)
and focused on survivors of CNS tumours.
Materials andmethods
Study population
The Swiss Childhood Cancer Survivor Study (SCCSS)
The SCCSS is a population-based cohort of all children reg-
istered in the Swiss Childhood Cancer Registry (SCCR), who
were diagnosed since 1976, survived 5years after initial
diagnosis, and were alive at the time of the study [10]. The
SCCR includes all patients diagnosed at age < 21years in Swit-
zerland with leukaemia, lymphoma, CNS tumours, malignant
solid tumours or Langerhans cell histiocytosis [11]. Recent
estimates indicate that the SCCR includes 95% of those diag-
nosed since 1995 in Switzerland [12]. We included survivors
who were 8–15years old at survey, and diagnosed between
1995 and 2010. We traced addresses and sent their parents a
questionnaire in 2010–2016. Non-responders received a sec-
ond copy and then were reminded by phone or postal mailing.
Ethical approval was granted through the Ethics Committee
of the Canton of Bern to the SCCR and the SCCSS (KEK-BE:
166/2014).
Measurements
SCCSS Questionnaire Survey
Hearing loss Parents were asked if a doctor had told them
that their child had hearing problems (Supplemental Figure
S1). We coded missing answers (4%) as normal hearing.
HRQoL We assessed HRQoL with the KIDSCREEN-27
questionnaire for parents [13], which groups 27 items into
five dimensions of HRQoL: Physical well-being; psycholog-
ical well-being; autonomy; parents, peers and social sup-
port; and school environment. For each item, parents rated
their child’s HRQoL for the past week on a Likert Scale.
For each dimension, we calculated a Rasch score between 0
and 100 and used international norms to convert them into
T-scores (mean = 50; SD = 10). Higher scores indicate bet-
ter HRQoL. This instrument has satisfactory psychometric
properties [1416] and has been used in childhood cancer
survivors [5, 15]. We used international norms as Swiss
norm data were only available for the German-speaking
regions [17] and 30% of our participants were from French-
or Italian-speaking regions.
Sociodemographics andchronic health problems The ques-
tionnaire also assessed sociodemographic data and chronic
health problems (Supplemental TableS1). For each survi-
vor, we created a sum score of cumulative disease burden by
adding the numbers of identified health problems.
SCCR
Clinical information included age at diagnosis; age at sur-
vey; gender; cancer diagnosis; chemotherapy; radiotherapy;
brain surgery; bone marrow transplant (BMT); and relapse
during follow-up time. We classified cancer diagnosis
according to the International Classification of Childhood
Cancer—3rd Edition [18].
Statistical analysis
We stratified our analyses for tumour types (CNS and non-
CNS cancers) since we found previously that survivors of
CNS tumour scored low on physical well-being [5], and
interaction tests showed that hearing loss had greater effect
on the HRQoL dimension peers and social support in sur-
vivors of CNS tumours (pinteraction = 0.005).
We used t-tests to compare the mean scores of the five KID-
SCREEN-27 dimensions of children with hearing loss to those
of children with normal hearing and to norm values. We did
this separately for children with CNS tumours and those with
other cancers. Among survivors of CNS tumours, those with
hearing loss had worse HRQoL for physical well-being and
peers and social support. For survivors of CNS tumours we
then tested if the association might have been confounded by
other factors, including sociodemographics, clinical character-
istics, cancer treatment, or other chronic health problems (Sup-
plemental TableS2) [1921]. In multivariable linear regres-
sions, we included a priori age at survey and gender, and all
characteristics that had been significantly associated (p < 0.05)
with physical well-being and peers and social support in the
univariable models. Likelihood ratio tests determined statisti-
cal significance. We performed a sensitivity analysis includ-
ing those who completed hearing questions (n = 677) only
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and obtained similar results. We used Stata (Version 13, Stata
Corporation, Austin, Texas) to calculate Rasch- and T-Scores
and to perform all other analysis.
Results
Characteristics ofthestudy population
Of 976 parents contacted, 700 returned the questionnaire (72%
response rate, Supplemental Figure S2). Survivors of CNS
tumours (n = 123) were older at diagnosis (p < 0.001) and sur-
vey (p = 0.003), and had more often platinum chemotherapy,
cranial radiation, brain surgery, relapses, hearing loss, or more
than one health problem (all p < 0.001) than survivors of other
cancers (Tables1, 2, 3).
Hearing loss andHRQoL
In survivors of CNS tumours, hearing loss was associated with
poorer physical well-being (with hearing loss: T-score = 44 vs.
normal hearing: T-score = 48, p = 0.047) and peers and social
support (with hearing loss T-score = 40 vs. normal hearing:
T-score = 47, p = 0.040; Fig. 1). Differences remained after
adjusting for sociodemographic and clinical characteristics
Table 1 Sociodemographic characteristics of the study population
CNS central nervous system, n number, SD standard deviation
a p-values calculated from t-tests comparing survivors of CNS to survivors of non-CNScancers
b Column percentages are given
c p-values calculated from Chi-square tests comparing survivors of CNS to survivors of non-CNS cancers
d We classified participants who were not Swiss citizens at birth, not born in Switzerland, or had at least one parent who was not a Swiss citizen
as having a migration background
e We classified parental education into three categories: primary education (compulsory schooling only [≤ 9 years]), secondary education (voca-
tional training [10–13 years]), and tertiary education (higher vocational training, college, or university degree). If parents achieved different lev-
els of education, we selected the parent with the highest education
f Numbers and percentages are based upon available data
Study participants
CNS tumours n = 123 Non-CNS cancers n = 577
Mean (SD) Mean (SD) p-valuea
Age at survey, years 13 (2) 12 (2) 0.003
Sociodemographic characteristics n (%)bn (%)bp-valuec
Gender 0.100
Female 62 (50) 244 (42)
Migration backgroundd0.568
No 92 (75) 417 (72)
Yes 31 (25) 160 (28)
Language region of Switzerland 0.612
German speaking 90 (73) 400 (69)
French speaking 29 (24) 149 (26)
Italian speaking 4 (3) 28 (5)
Parental educatione, f 0.704
Primary 8 (7) 50 (9)
Secondary 77 (63) 346 (60)
Tertiary 34 (28) 163 (28)
Child lives with/inf0.784
Both parents 103 (84) 464 (80)
One parent and partner 7 (6) 38 (7)
One parent 12 (10) 67 (12)
Institution 1 (1) 2 (1)
Siblingsf0.237
No sibling 12 (10) 79 (14)
Has sibling(s) 109 (89) 488 (85)
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Table 2 Clinical characteristics of the study population
Italics value idicates the denominator of percentages is related to those with cranial radiation
CNS central nervous system, ICCC-3 International Classification of childhood cancer, n number, SD standard deviation
a p-values calculated from t-tests comparing survivors of CNS to survivors of non-CNScancers
b Column percentages are given
c p-values calculated from Chi-square tests comparing survivors of CNS to survivors of non-CNScancers
d Other malignant epithelial neoplasms, malignant melanomas and other unspecified malignant neoplasms
e Numbers and percentages are based upon available data
Study participants
CNS tumours n = 123 Non-CNS cancers n = 577
Mean (SD) Mean (SD) p-valuea
Age at diagnosis, years 5 (3) 3 (2) < 0.001
Sociodemographic characteristics n (%)bn (%)bp-valuec
Clinical characteristics
Diagnosis (ICCC-3) n.a
I Leukaemias 273 (47)
II Lymphomas 50 (9)
III CNS tumours 123 (100) 0 (0)
IV Neuroblastoma 61 (11)
V Retinoblastoma 38 (7)
VI Renal tumours 51 (9)
VII Hepatic tumours 12 (2)
VIII Bone tumours 12 (2)
IX Soft tissue sarcomas 46 (8)
X Germ cell tumours 11 (2)
XI and XII Other rare tumoursd 2 (1)
Langerhans cell histiocytosis 21 (4)
Treatments
Chemotherapy < 0.001
Platinum 43 (35) 107 (19)
No platinum 8 (7) 415 (72)
Unknown platinum use 2 (2) 1 (1)
No chemotherapy 70 (57) 54 (9)
Cranial radiotherapy < 0.001
Yes, Graye32 (26) 46 (8)
1–29 3 (9) 30 (65)
≥ 30 27 (84) 13 (28)
No 91 (74) 531 (92)
Surgery < 0.001
Brain surgery 72 (59) 14 (2)
CSF-shunt 6 (5) 0 (0)
Both 26 (21) 0 (0)
Bone marrow transplantatione0.044
No 116 (94) 521 (90)
Yes 3 (2) 43 (7)
Relapse < 0.001
No 86 (70) 525 (91)
Yes 37 (30) 52 (9)
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and cumulative disease burden (estimated differences: physi-
cal well-being [unadjusted − 4.6; adjusted − 4.0]; peers and
social support [unadjusted − 6.7; adjusted − 5.0], Supplemen-
tal tableS2).
In contrast, survivors of other cancers had HRQoL com-
parable or higher than norm values in all dimensions, and
there was no evidence that hearing loss affected HRQoL (all
p> 0.05, Fig.1).
Discussion
Survivors of CNS tumours with hearing loss were more
likely to feel physically worse and to have poorer relation-
ships to peers than survivors with normal hearing or than
the international norm. In survivors of non-CNS cancers,
hearing loss was not associated with HRQoL.
Our study had both limitations and strengths. Although
we based our conclusion on parent-reported data [5,
2124], validity of self-reported hearing loss was good
when compared to information from medical records [25,
26]. We had no data on hearing aids, though these may
affect HRQoL. A strength was the large population-based
sample of young, recently diagnosed survivors treated
according to the latest treatment protocols, and the com-
prehensive data available on cancer type, treatment and
other chronic health problems.
The only other study that investigated effects of hearing
loss on HRQoL in young survivors included 137 US neu-
roblastoma survivors (non-CNS cancer) [4], and found that
survivors with hearing loss were less functional in school,
Table 3 Health outcomes of the study population
Italics value idicates the denominator of percentages is related to
those with hearing loss
CNS central nervous system, n number
a Column percentages are given
b p-values calculated from Chi-square tests comparing survivors of
CNS to survivors of non-CNS cancers
Study participants
CNS tumours
n = 123
Non-CNS cancers
n = 577
n (%)an (%)ap-valueb
Hearing outcomes < 0.001
Normal hearing 98 (80) 533 (92)
Hearing loss 25 (20) 44 (8)
Severity of hearing loss 0.471
Mild 13 (52) 25 (57)
Moderate 8 (32) 9 (20)
Severe (deaf) 3 (12) 4 (9)
Unknown 1 (4) 6 (13)
Laterality of hearing loss 0.490
Unilateral 6 (24) 13 (30)
Bilateral 17 (68) 24 (55)
Unknown 2 (8) 7 (16)
Chronic health problems/
cumulative disease burden
< 0.001
0 32 (26) 331 (57)
1 30 (24) 141 (24)
2 28 (23) 70 (12)
3 or more 33 (27) 35 (6)
Fig. 1 Mean scores from
KIDSCREEN-27 comparing
survivors with hearing loss
and normal hearing strati-
fied by tumour type. Higher
scores indicate better HRQoL.
International norms have a sore
of 50 ± 10 (SD). p-values cal-
culated from t-tests comparing
survivors with hearing loss and
normal hearing. CI confidence
interval, CNS central nervous
system, p p-value
43.8
48.4
46.7
49.7
52.4
52.8
39.9
46.6
48.7
52.1
Physical well-being
Psychological wel
l-being
A
utonomy & parents
Peers & social support
School environment
30 35 40 45 50 55
CNS tumours
50.5
52.1
51.8
52.3
52.3
53.4
53.1
51.1
53.5
53.0
48 50 52 54 56
Non-CNS cancers
Mean scores from KIDSCREEN-27 with 95% Cls
p=0.047
p=0.040
Hearing loss
Normal hearin
g
Quality of Life Research
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and had lower psychosocial functioning and overall HRQoL.
We found no effect of hearing loss in survivors of non-CNS
cancers. The US study did not adjust for chronic health prob-
lems common in survivors of neuroblastoma, which may
have confounded the association [6, 7]. A US study in adult
survivors (n = 406) reported a negative impact of hearing
loss on educational plans and social attainment in both sur-
vivors of CNS and non-CNS cancers [27]. It seems that the
vulnerability to hearing loss changes with increasing age as
in our study survivors of CNS tumours only were vulner-
able to hearing loss and other survivors could cope better
with hearing loss. In our study, survivors of CNS tumours
were treated more intensively and their burden of disabilities
was higher than in those of non-CNS cancers. This might
in return explain the low physical well-being in survivors of
CNS tumours, which is consistent with previous studies [5,
28]. The HRQoL dimension peers and social support was
particularly affected in survivors of CNS tumours. It might
be explained by the fact that they frequently suffer from
reduced neurocognitive functioning and sometimes have to
repeat a year in school [29].
Since audiological monitoring is only partly implemented
for survivors in Switzerland [8], health professionals should
pay careful attention to hearing problems and their effects in
survivors of CNS tumours. Future studies should investigate
if hearing aids or other interventions, including speech ther-
apy, frequency modulation amplification systems or prefer-
ential classroom seating, help reducing problems with peers.
Conclusion
Hearing loss reduces physical well-being and impairs rela-
tionships with peers in survivors of CNS tumours, but not in
other survivors, so clinicians should be alert to these prob-
lems in this vulnerable group. They may benefit most from
strict audiological monitoring and timely intervention to
mitigate secondary consequences of hearing loss on HRQoL.
Acknowledgements We thank all childhood cancer survivors and
families for participating in our survey. We thank the study team of
the SCCSS (Rahel Kuonen, Rahel Kasteler, Jana Remlinger, Laura
Wengenroth, Corina Rueegg, Cornelia Rebholz), data managers of the
SPOG (Claudia Anderegg, Pamela Balestra, Nadine Beusch, Rosa-
Emma Garcia, Franziska Hochreutener, Friedgard Julmy, Nadia Lanz,
Rodolfo Lo Piccolo, Heike Markiewicz, Annette Reinberg, Renate
Siegenthaler and Verena Stahel) and the team of the SCCR (Verena
Pfeiffer, Katharina Flandera, Erika Brantschen-Berclaz, Shelagh Red-
mond, Meltem Altun, Parvinder Singh, Elisabeth Kiraly). We thank
Kali Tal for her editorial suggestions. Swiss Paediatric Oncology
Group (SPOG) Scientific Committee: Prof. Dr. med. R. Ammann,
Bern; Dr. med. K. Scheinemann, Aarau; Prof. Dr. med. M. Ansari,
Geneva; Prof. Dr. med. M. Beck Popovic, Lausanne; Dr. med. P. Braz-
zola, Bellinzona; Dr. med. J. Greiner, St. Gallen; Prof. Dr. med. M.
Grotzer, Zurich; Dr. med. H. Hengartner, St. Gallen; Prof. Dr. med.
T. Kuehne, Basel; Prof. Dr. med. J. Rössler, Bern; Prof. Dr. med. F.
Niggli, Zurich; PD Dr. med. F. Schilling, Lucerne; Prof. Dr. med. N.
von der Weid, Basel.
Funding This study was supported by the Swiss Cancer League (Grant
Nos: 3412-02-2014, 3886-02-2016), the Stiftung für Krebsbekämp-
fung (www.krebs bekae mpfun g.c h), Kinderkrebs Schweiz (www.kinde
rkreb s-schwe iz.ch) and received funding from the European Union’s
Seventh Framework Programme for research, technological develop-
ment and demonstration under grant agreement no 602030. The work
of the Swiss Childhood Cancer Registry is supported by the Swiss
Paediatric Oncology Group (www.spog.ch), Schweizerische Konfer-
enz der kantonalen Gesundheitsdirektorinnen und -direktoren (www.
gdk-cds.ch), Swiss Cancer Research (www.krebs forsc hung.ch), Kinder-
krebshilfe Schweiz (www.kinde rkreb shilf e.ch), the Federal Office of
Public Health (FOPH) and the Institute of Cancer Epidemiology and
Registration (www.nicer .org). The funders of the Swiss Childhood
Cancer Registry support the daily running of the registry and have not
had and will not have any role in the design, conduct, interpretation,
or publication of the Swiss Childhood Cancer Registry itself as well
as the related research projects.
Compliance with ethical standards
Conflict of interest The authors declare that they have no conflict of
interest.
Ethical approval All procedures performed in studies involving human
participants accorded with the ethical standards of the institutional and/
or national research committee and with the 1964 Helsinki declara-
tion and its later amendments or comparable ethical standards. Ethics
approval was granted by the Ethics Committee of the Canton of Bern
to the SCCR and SCCSS (KEK-BE: 166/2014) and the SCCSS is reg-
istered at ClinicalTrials.gov (Identifier: NCT03297034).
Informed consent Informed consent was obtained from all participants
(parents and survivors) for registration in the SCCR and participation
in the SCCSS survey.
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... 1,3,4 Even though hearing loss is not life-threatening, it may impair speech development, academic performance, and quality of life. [5][6][7] Research on hearing loss after platinum-based chemotherapy among CCS is extensive; however, evidence from representative nationwide samples is limited. 8 Previous studies were often singlecenter [9][10][11][12][13][14][15][16] or included selected clinics. ...
... CCS treated at a young age with high cumulative doses of cisplatin and concomitant CRT are particularly at risk. Even if it is not a life-threatening late effect, hearing loss can have negative consequences on the lives of affected individuals.[5][6][7] Our findings suggest that monitoring the hearing function among this vulnerable population of CCS should be continued beyond the end of cancer treatment.4 ...
Article
Background: Hearing loss is a potential side effect from childhood cancer treatment. We described the severity of hearing loss assessed by audiometry in a representative national cohort of childhood cancer survivors (CCS) and identified clinical risk factors. Procedure: We included all CCS from the Swiss Childhood Cancer Registry who were diagnosed ≤18 age and treated with platinum-based chemotherapy between 1990 and 2014. We extracted audiograms, treatment-related information, and demographic data from medical records. Two reviewers independently assessed the severity of hearing loss at latest follow-up using the Münster Ototoxicity Scale. We used ordered logistic regression to identify clinical risk factors for severity of hearing loss. Results: We analyzed data from 270 CCS. Median time from cancer diagnosis to last audiogram was 5 years (interquartile range 2.5-8.1 years). We found 53 (20%) CCS with mild, 78 (29%) with moderate, and 75 (28%) with severe hearing loss. Higher severity grades were associated with (a) younger age at cancer diagnosis (odds ratio [OR] 5.4, 95% confidence interval [CI]: 2.5-12.0 for <5 years); (b) treatment in earlier years (OR 4.8, 95% CI: 2.1-11.0 for 1990-1995); (c) higher cumulative cisplatin doses (OR 13.5, 95% CI: 4.7-38.8 for >450 mg/m2 ); (d) concomitant cranial radiation therapy (CRT) (OR 4.4, 95% CI: 2.5-7.8); and (e) hematopoietic stem cell transplantation (HSCT) (OR 2.7, 95% CI: 1.0-7.2). Conclusion: Three of four CCS treated with platinum-based chemotherapy experienced some degree of hearing loss. We recommend closely monitoring patient's hearing function if treated at a young age with high cumulative cisplatin doses, and concomitant CRT as part of long-term care.
... In teenagers, the high-frequency hearing loss can reduce speech recognition in noisy environment affecting learning and communicative abilities and quality of life [49], especially during school performances. Additionally, audiological surveillance is useful in children affected by central nervous system tumors since hearing loss is associated with worse physical wellbeing, poorer relationship with peers, and greater need for social support compared to normal hearing [50,51]. ...
Article
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Purpose Irreversible bilateral sensorineural hearing loss is a common side effect of platinum compounds. Because of the extended overall survival, a prolonged hearing surveillance and management of hearing impairments are emerging concerns for pediatric oncology. Methods In this retrospective observational study, we enrolled 38 children out of 116 treated at our institution by chemotherapy (cisplatin and/or carboplatin) with or without irradiation between 2007 and 2014, submitted to hearing monitoring before every cycle of chemotherapy, and who completed a 5-year long-term audiological follow-up. Chemotherapy regimens, demographic findings, cumulative doses, and cranial irradiation were compared. Results At the end of 5-year follow-up, ototoxicity was significantly increased compared to that observed at the end of chemotherapy (52.5% vs 39.5%, p < 0.001). A late onset of hearing loss was experienced in 13.1% of children, while in 26.3% progressive hearing loss was measured. Deafness at the end of chemotherapy and irradiation were significant prognostic factors for late ototoxicity outcomes (Odds Ratio 7.2—CI 1.67–31.1—p < 0.01 and 5.25—CI 1.26–21.86—p < 0.01 respectively). No significant differences were found between cisplatin and combined treatment (i.e., cisplatin shifted to carboplatin during monitoring for the onset of ototoxicity) and ototoxicity was not associated with platinum compounds cumulative dose (p > 0.05). 13.1% of children needed hearing aids at the end of follow-up. Conclusion Long-term monitoring of at least 5 years prevents the harmful effects of hearing deprivation identifying late onset/progressive hearing loss after platinum compound chemotherapy in children thanks to early hearing rehabilitation, especially in those who underwent multimodal therapy or subjected to irradiation.
... It is documented that hearing loss has an adverse effect on cognitive performance, quality of life and work, physical well-being, peers and social support, social relationships, motor skills, and psychological aspects. 17,18 Based on the aforementioned issues, using effective strategies for preventing NIHL, such as reduced noise exposure through engineering and administrative control measures, training interventions, providing hearing protection devices, and vitamin/antioxidant intake, is substantial. [19][20][21] Engineering and administrative control measures require a lot of funding for implementation and maintenance, therefore sometimes it is not cost-effective. ...
Article
Full-text available
Objectives Occupational noise‐induced hearing loss (NIHL) due to industrial, military, and other job ‐related noise exposure can cause harmful health issues to occupied workers, but may also be potentially preventable. Vitamins/antioxidant have been studied as therapeutic strategies to prevent and/or delay the risks of human diseases as well as NIHL .So, this study was conducted to systematically review the protective effects of vitamins/antioxidants on occupational NIHL. Methods Online databases including PubMed/Medline, Scopus, Web of Science, EMBASE, Science Direct, and Google Scholar were systematically searched up to 12 January 2021. Based on 6336 potentially relevant records identified through the initial search in the databases, 12 full‐text publications were retrieved, one of which can be viewed as two separate trials, because it has studied the effects of two different antioxidants (ginseng and NAC) on NIHL, separately. Results A review of the studies shows that vitamin B12, folic acid, and N‐acetylcysteine (NAC) have a considerable protective effect on NIHL. However, these protective effects are not yet specified in different frequencies. The findings regarding the protective effects of other antioxidants are inconsistent in this field. Conclusion Vitamin B12, folic acid, and NAC may have a protective effect as an antioxidant on reducing occupational hearing loss. For a conclusive evidence of vitamin/antioxidant protective therapies, future studies with precise criteria for noise exposure and similar outcome parameters are required.
... Up to 67 per cent of children treated with cisplatin develop permanent bilateral hearing loss. 2 A recent study looking at hearing loss and quality of life in survivors of paediatric central nervous system tumours found that hearing loss was also associated with impaired physical well-being. 3 Estimates of the prevalence of hearing loss in children and young people following ototoxic medication are greatly variable and are dependent on how hearing loss is graded and classified. A study that investigated the incidence of hearing loss in a group of 23 survivors of childhood cancer treated with cisplatin reported that 52 per cent of this group had bilateral high-frequency hearing loss on audiometry. ...
Article
Full-text available
Objective Platinum-based chemotherapy drugs are associated with substantial ototoxicity. The hearing of children treated with these drugs should be closely monitored. Method A questionnaire was sent out to the 19 audiology departments associated with national paediatric cancer specialist centres in the UK looking at current practice in ototoxicity monitoring. Results Responses were received from 17 of 19 centres (89 per cent). All offered some form of audiometric monitoring service. Extended high-frequency testing (9–20 kHz) was only utilised by 7 services (29 per cent). A majority of respondents were reluctant to consider self-test devices in paediatric ototoxicity monitoring ( n = 9; 53 per cent). Provision of long-term audiological follow up is sporadic with only 4 (23 per cent) respondents keeping all children with normal hearing under review once treatment is completed. Conclusion While some good practice in paediatric ototoxicity was identified, opportunities exist to improve clinical practice and protocols, promote multidisciplinary team working and to utilise technologies such as extended high frequency and self-test audiometry.
... Thus, hearing aids and the use of assistive listening devices such as frequency modulation systems are suitable. Additionally, audiological surveillance is useful in children affected by central nervous system tumours since hearing loss is associated with worse physical well-being, poorer relationship with peers and greater need for social support compared to normal hearing [45]. Furthermore, most of survival children live far from tertiary oncologic institutions, therefore many years after the end of therapies oncological check-ups become less frequent; many families continue their sporadic radiologic and clinical controls at their hometown hospital, making it difficult to obtain a long-term reliable hearing assessment. ...
... 10 Studies have highlighted the cognitive implication and effect on quality of life from severe ototoxicity-related sensorineural hearing loss in children. [34][35][36] To the authors' knowledge, despite the high prevalence in adults with chronic lung disease, no studies evaluating the effect of ototoxicity on quality of life have been performed. ...
Article
Introduction Individuals with chronic lung disease (eg, cystic fibrosis (CF)) often receive antimicrobial therapy including aminoglycosides resulting in ototoxicity. Extended high-frequency audiometry has increased sensitivity for ototoxicity detection, but diagnostic audiometry in a sound-booth is costly, time-consuming and requires a trained audiologist. This cross-sectional study analysed tablet-based audiometry (Shoebox MD) performed by non-audiologists in an outpatient setting, alongside home web-based audiometry (3D Tune-In) to screen for hearing loss in adults with CF. Methods Hearing was analysed in 126 CF adults using validated questionnaires, a web self-hearing test (0.5 to 4 kHz), tablet (0.25 to 12 kHz) and sound-booth audiometry (0.25 to 12 kHz). A threshold of ≥25 dB hearing loss at ≥1 audiometric frequency was considered abnormal. Demographics and mitochondrial DNA sequencing were used to analyse risk factors, and accuracy and usability of hearing tests determined. Results Prevalence of hearing loss within any frequency band tested was 48%. Multivariate analysis showed age (OR 1.127; (95% CI: 1.07 to 1.18; p value<0.0001) per year older) and total intravenous antibiotic days over 10 years (OR 1.006; (95% CI: 1.002 to 1.010; p value=0.004) per further intravenous day) were significantly associated with increased risk of hearing loss. Tablet audiometry had good usability, was 93% sensitive, 88% specific with 94% negative predictive value to screen for hearing loss compared with web self-test audiometry and questionnaires which had poor sensitivity (17% and 13%, respectively). Intraclass correlation (ICC) of tablet versus sound-booth audiometry showed high correlation (ICC >0.9) at all frequencies ≥4 kHz. Conclusions Adults with CF have a high prevalence of drug-related hearing loss and tablet-based audiometry can be a practical, accurate screening tool within integrated ototoxicity monitoring programmes for early detection.
Article
Background: Some children with central nervous system (CNS) and solid tumors are at risk to develop ototoxicity during treatment. Up to now, several risk factors have been identified that may contribute to ototoxicity, such as platinum derivates, cranial irradiation, and brain surgery. Comedication, like antibiotics and diuretics, is known to enhance ototoxicity, but their independent influence has not been investigated in childhood cancer patients. Recommendations for hearing loss screening are missing or vary highly across treatment protocols. Additionally, adherence to existing screening guidelines is not always optimal. Currently, knowledge is lacking on the prevalence of ototoxicity. Objective: The aim of the Study on Prevalence and Determinants of Ototoxicity During Treatment of Childhood Cancer (SOUND) is to determine the feasibility of audiological testing and to determine the prevalence and determinants of ototoxicity during treatment for childhood cancer in a national cohort of patients with solid and CNS tumors. Methods: The SOUND study is a prospective cohort study in the national childhood cancer center in the Netherlands. The study aims to include all children aged 0 to 19 years with a newly diagnosed CNS or solid tumor. Part of these patients will get audiological examination as part of their standard of care (stratum 1). Patients in which audiological examination is not the standard of care will be invited for inclusion in stratum 2. Age-dependent audiological assessments will be pursued before the start of treatment and within 3 months after the end of treatment. Apart from hearing loss, we will investigate the feasibility to screen patients for tinnitus and vertigo prevalence after cancer treatment. This study will also determine the independent contribution of antibiotics and diuretics on ototoxicity. Results: This study was approved by the Medical Research Ethics Committee Utrecht (Identifier 20-417/M). Currently, we are in the process of recruitment for this study. Conclusions: The SOUND study will raise awareness about the presence of ototoxicity during the treatment of children with CNS or solid tumors. It will give insight into the prevalence and independent clinical and cotreatment-related determinants of ototoxicity. This is important for the identification of future high-risk patients. Thereby, the study will provide a basis for the selection of patients who will benefit from innovative otoprotective intervention trials during childhood cancer treatment that are currently being prepared. Trial registration: Netherlands Trial Register NL8881; https://www.trialregister.nl/trial/8881. International registered report identifier (irrid): DERR1-10.2196/34297.
Article
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Introduction: Children with hearing impairment (HI) often encounter difficulties in learning due to their language problems caused by HI. Therefore, children with HI also suffer from health problems, including psychological health, social relationship, and school performance. Given that the International Classification of Function, Disability, and Health proposed environment as a key element in promoting health. This study proposed to investigate the impacts of environmental support on health and learning abilities among a nationally representative sample with HI. Methods: A total of 163 children (94 boys; 88 first graders and 75 third graders) retrieved from the Special Needs Education Longitudinal Study were used for analysis. Questionnaire items on environmental support (3 items), impairment (1 item), learning ability (4 items), and health (4 items) were constructed in a structural equation model. Specifically, environmental support was linked to impairment, learning ability, and health; impairment was linked to learning ability and health. Results: Our results indicated that environmental support had positive effects on three dimensions of health (social relationship, β = 0.38; emotional functioning, β = 0.27; and school performance, β = 0.59) and learning ability (β = 0.26); negative effects on impairment (β = −0.62). Impairment had negative impacts on two dimensions of health (physical fitness and school performance, β = −0.18 and −0.22, respectively) and learning ability (β = −0.29). Conclusions: According to our findings, health-care professionals and school teachers may consider establishing good environmental support for children with HI. Thus, children with HI may have improved health and learning abilities.
Article
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Background Hearing loss is a potential late effect after childhood cancer. Questionnaires are often used to assess hearing in large cohorts of childhood cancer survivors and it is important to know if they can provide valid measures of hearing loss. We therefore assessed agreement and validity of questionnaire-reported hearing in childhood cancer survivors using medical records as reference. Procedure In this validation study, we studied 361 survivors of childhood cancer from the Swiss Childhood Cancer Survivor Study (SCCSS) who had been diagnosed after 1989 and had been exposed to ototoxic cancer treatment. Questionnaire-reported hearing was compared to the information in medical records. Hearing loss was defined as ≥ grade 1 according to the SIOP Boston Ototoxicity Scale. We assessed agreement and validity of questionnaire-reported hearing overall and stratified by questionnaire respondents (survivor or parent), sociodemographic characteristics, time between follow-up and questionnaire and severity of hearing loss. Results Questionnaire reports agreed with medical records in 85% of respondents (kappa 0.62), normal hearing was correctly assessed in 92% of those with normal hearing (n = 249), and hearing loss was correctly assessed in 69% of those with hearing loss (n = 112). Sensitivity of the questionnaires was 92%, 74%, and 39% for assessment of severe, moderate and mild bilateral hearing loss; and 50%, 33% and 10% for severe, moderate and mild unilateral hearing loss, respectively. Results did not differ by sociodemographic characteristics of the respondents, and survivor- and parent-reports were equally valid. Conclusions Questionnaires are a useful tool to assess hearing in large cohorts of childhood cancer survivors, but underestimate mild and unilateral hearing loss. Further research should investigate whether the addition of questions with higher sensitivity for mild degrees of hearing loss could improve the results.
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Objective: To determine the impact of pediatric hearing loss (HL) on quality of life (QOL). Data sources: A qualified medical librarian conducted a literature search for relevant publications that evaluate QOL in school-aged children with HL. Review methods: Studies were assessed independently by 2 reviewers for inclusion in the systematic review and meta-analysis. Results: From 979 abstracts, 69 were identified as relevant; ultimately, 40 articles were included in the systematic review. This review revealed that children with HL generally report a lower QOL than their normal-hearing peers and that QOL improves after interventions. The extent of these differences is variable among studies and depends on the QOL measure. Four studies using the Pediatric Quality of Life Inventory (PedsQL) had sufficient data for inclusion in a meta-analysis. After studies were pooled, statistically and clinically significant differences in PedsQL scores were found between children with normal hearing and those with HL, specifically in the social and school domains. Statistically significant differences were also noted in total scores for children with unilateral HL and in the physical domain for children with bilateral HL as compared with those having normal hearing; however, these differences were not clinically meaningful. Conclusions: Our analysis reveals that decreased QOL in children with HL is detected in distinct domains of the PedsQL. These domains-school activities and social interactions-are especially important for development and learning. Future work should focus on these aspects of QOL when assessing HL in the pediatric population.
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QUESTIONS UNDER STUDY: Completeness is important in cancer registration. Identifying areas to improve registry procedures might help to maximise completeness. We examined characteristics of childhood cancer cases that were registered via death certificate notification (DCN) rather than during life, and estimated completeness of the Swiss Childhood Cancer Registry (SCCR). METHODS: We analysed data from all children who died from cancer in Switzerland between 1985–2009 at age <16 years (n = 978), and checked whether they had been registered in the SCCR. We used multivariable logistic regression to compare characteristics of DCN cases with deceased SCCR cases, and the DCN-to-incidence and mortality-to-incidence ratio method to estimate completeness for different diagnostic periods. RESULTS: Among 978 deceased children with cancer, 126 (12.9%) were registered via DCN. Those with tumours of digestive organs (odds ratio [OR] 5.1; 95% confidence interval [CI] 1.9–13.7), tumours of endocrine glands (OR 4.5; 95% CI 1.6–12.3), and brain tumours (OR 3.1; 95% CI 1.7–5.5) were more likely to be DCN cases than those with leukaemia. Neonates (OR 14.1, 95% CI 5.3–37.3), infants (OR 7.5; 95% CI 3.1–18.0) and 14–15 year olds (OR 2.4; 95% CI 1.2–4.9) were more likely to be DCN cases than 1–4 year olds. The DCN proportion was particularly high in infants who lived in rural regions. Estimated completeness of the SCCR increased from 85% for 1985–89 to ≥95% for 1995–2009. CONCLUSIONS: Childhood cancer registration in Switzerland was quite complete, but registration must improve for infants, particularly neonates, and children diagnosed with hepatic, endocrine and brain tumours.
Article
Background: Full audiological monitoring is the best strategy to detect hearing loss early and to provide timely intervention in the absence of a clinical method of otoprotection. Full monitoring requires audiological evaluation before, and then during and after ototoxic cancer treatment. In a worldwide context of monitoring protocols that vary substantially, we analyzed the audiological monitoring of childhood cancer patients over the last decade across treatment centers in Switzerland. Procedure: We retrospectively searched for audiological evaluations in all nine Swiss Pediatric Oncology Centers. We analyzed proportions of patients who had audiological monitoring and described type and timing of monitoring. We determined predictors of audiological monitoring using multivariable logistic regression and described time trends. Results: We included 185 patients from the Swiss Childhood Cancer Registry diagnosed 2005-2013 who had platinum chemotherapy and/or cranial radiation ≥30 Gray and who were alive at time of study. Less than half of children, 43%, had full audiological monitoring (before, during, and after treatment), while 72% were tested after cancer treatment. Non-study patients were less likely to have had monitoring in all phases of cancer treatment. Patients who received treatment with cisplatin or both platinum chemotherapy and cranial radiation were more likely to have had monitoring after treatment. Monitoring during and after treatment increased over the study period, but monitoring before treatment was insufficient in all time periods. Conclusions: Our population-based study indicates that audiological monitoring is insufficient in Switzerland, particularly for non-study patients. Clinicians’ must become more aware of the importance of full audiological monitoring.
Article
Purpose: This study aimed to estimate the burden of morbidity, in terms of health-related quality of life (HRQL), in survivors of high-risk neuroblastoma (NBL) after myeloablative chemotherapy followed by autologous hematopoietic stem cell transplant (HSCT). Patients and methods: A national population-based survey was undertaken of survivors of high-risk NBL (N = 99), diagnosed between 1991 and 2010 and treated with HSCT. Parents completed a proxy questionnaire incorporating two HRQL measures, Health Utilities Index (HUI) 2 and 3. Children >12 years of age provided self-assessments. Clinical and demographic data were collected. Independent t-test and one-way analysis of variance were used to assess differences. Comparative data were obtained from previously published work and Statistics Canada's 1998 National Population Health Survey. Results: On a scale of 0 (being dead) to 1.0 (perfect health), mean HRQL utility scores were 0.89 (SD = 0.11) in HUI2 and 0.84 (SD = 0.18) in HUI3. Parents reported morbidity in sensation (52.5%), pain (30.3%), cognition (28.0%), and emotion (24.2%) in HUI2 and in hearing (38.4%), pain (30.3%), cognition (27.3%), and speech (23.2%) in HUI3. HRQL was not significantly different compared to NBL survivors treated without HSCT, but was less than in nontransplanted survivors of acute lymphoblastic leukemia and Wilms tumor, and children in the general population, yet higher than in survivors of brain tumors. Conclusions: HRQL is compromised in high-risk NBL survivors treated with and without HSCT. A differential effect on hearing reflects additional exposure to platinum-based chemotherapy. These results should inform long-term care and the development of new therapeutic interventions.
Article
Ototoxicity is a well-established toxicity associated with a subgroup of antineoplastic therapies that includes platinum chemotherapy, radiation or surgery involving the ear and auditory nerve, and supportive care agents such as aminoglycoside antibiotics and loop diuretics. The reported prevalence of ototoxicity in patients who have received potentially ototoxic therapy ranges from 4% to 90% depending on factors such as age of the patient population, agent(s) used, cumulative dose, and administration techniques. The impact of ototoxicity on subsequent health-related and psychosocial outcomes in these patients can be substantial, and the burden of morbidity related to ototoxic agents is particularly high in very young children. Considerable interindividual variability in the prevalence and severity of ototoxicity has been observed among patients receiving similar treatment, suggesting genetic susceptibility as a risk factor. The development and testing of otoprotective agents is ongoing; however, to the author's knowledge, no US Food and Drug Administration-approved otoprotectants are currently available. Prospective monitoring for ototoxicity allows for comparison of auditory outcomes across clinical trials, as well as for early detection, potential alterations in therapy, and auditory intervention and rehabilitation to ameliorate the adverse consequences of hearing loss. Cancer 2016. © 2016 American Cancer Society.
Article
Objectives: To examine the discrepancy between survivor-parent and sibling-parent reports of health-related quality of life (HRQL) and the level of agreement (i.e., correlation) between child reports (i.e., survivor and sibling) and parent-proxy reports of HRQL. Methods: Fifty-one families participated. Pediatric cancer survivors (49% male; 6-18 years of age) and one sibling (47% male; 9-18 years of age) completed a measure of their HRQL. As well, one parent (14% male; 27-65 years of age) from each family completed a proxy report of their children's (i.e., survivor and sibling) HRQL. Consensus was determined through discrepancy and agreement scores, between parent-proxy and children's (i.e., survivors and siblings) self-reports of total HRQL, and physical, emotional, social, and school functioning subscales. Results: Repeated-measures analysis of variance (ANOVA) revealed significant group differences for total HRQL (F = 6.79, P ≤ 0.01). Repeated-measure ANOVAs of subscale discrepancy scores revealed significant group differences for physical functioning scores (F = 6.39, P < 0.01). A significant interaction was also found for social functioning when age at diagnosis was considered as a covariate (F = 10.30, P < 0.01). Zero-order and intraclass correlation coefficients revealed different levels of agreement between parent and child reports. Specifically, there was poorer agreement between parent-proxy and sibling's self-reports, particularly on social and emotional subscales. Conclusions: Discrepancy and agreement are both important indices to consider when examining consensus between parent-proxy and child self-reports. The findings from this study have important implications for future research and suggest that the impact of cancer on siblings should be further investigated.
Article
BACKGROUND Survivors of childhood cancer who are treated with platinum-based chemotherapy and/or cranial radiation are at risk of treatment-induced hearing loss. However, the effects of such hearing loss on adult social attainment have not been well elucidated.METHODS Adult survivors of pediatric central nervous system (CNS) solid tumors (180 survivors) and non-CNS solid tumors (226 survivors) who were treated with potentially ototoxic cancer therapy completed audiologic evaluations and questionnaires assessing their perception of social functioning and social attainment (ie, independent living, marriage, and employment). Audiograms were graded with the Chang ototoxicity grading scale. Analyses were stratified by tumor type (ie, CNS vs non-CNS). Multivariable logistic regression models were conducted with adjustment for age; sex; chronic health conditions; and, for the CNS group, IQ. Adjusted odds ratios (ORs) and 95% confidence intervals (95% CIs) were reported.RESULTSSerious hearing loss (that requiring a hearing aid or deafness) was detected in 36% of survivors of CNS tumors and 39% of survivors of non-CNS tumors. Serious hearing loss was associated with an increased risk of perceived negative impact in ≥1 areas of social functioning (survivors of non-CNS tumors: OR, 1.83 [95% CI, 1.00-3.34]). Among survivors of non-CNS tumors, serious hearing loss was associated with 2-fold increased risk of nonindependent living (OR, 2.19; 95% CI, 1.19-4.04) and unemployment or not graduating from high school (OR, 1.85; 95% CI, 1.00-3.34).CONCLUSIONSA substantial proportion of adult survivors of childhood cancer treated with potentially ototoxic therapy have serious hearing loss. Treatment-induced hearing loss was found to be associated with reduced social attainment, both perceived and actual, in this study sample. Cancer 2015. © 2015 American Cancer Society.
Article
Childhood cancer and its treatment may affect health-related quality of life (HRQoL) in childhood cancer survivors, but population-based studies in young survivors are scarce. We aimed to: (1) compare HRQoL between young survivors and population norms and (2) find factors that influence parent-reported HRQoL in survivors. As part of the Swiss Childhood Cancer Survivor Study, a questionnaire was mailed to parents of survivors aged 8-16 years, registered in the Swiss Childhood Cancer Registry, ≥5 years after diagnosis. We used the KIDSCREEN-27 instrument to compare self- and parent-reported HRQoL between survivors (N = 425) and standardized norms in the five dimensions of physical well-being, psychological well-being, autonomy, peers and school environment (mean = 50, SD = 10). We then used multivariable linear regressions to test the influence of socio-demographic and cancer-related factors on HRQoL. Self-reported physical well-being was comparable to norms. Other HRQoL dimensions were higher than norms, with the highest mean = 52.2 (p < 0.001) for school environment. Parent-reported HRQoL in survivors was comparable to population norms; only physical well-being was lower (mean = 47.1, p < 0.001), and school environment was higher (mean = 51.1, p = 0.035). Parent-reported HRQoL was lower for survivors of CNS tumors (physical well-being: β = -5.27, p = 0.007; psychological well-being: β = -4.39, p = 0.044; peers β = -5.17, p = 0.028), survivors of neuroblastoma (psychological well-being β = -5.20, p = 0.047), and survivors who had had a relapse (physical well-being β = -5.41, p = 0.005). Assessing HRQoL during follow-up care, with a focus on physical well-being, specific diagnoses (e.g., CNS tumor) and late complications (e.g., relapse) might help to early identify problems and offer support to survivors with reduced HRQoL.