Quality of Life-CI: Development of an Early Childhood Parent-Proxy & Adolescent Version

Abstract

Objectives –

Severe to profound hearing loss is associated with communication, social, and behavioral difficulties that have been linked to worse health-related quality of life (HRQoL) compared to individuals with normal hearing. HRQoL has been identified as an important health outcome that measures functional ability, particularly for chronic conditions and disabilities. The current study developed the QoL-CI for early childhood and adolescents using the recommended Food and Drug Administration (FDA) and European Medicines Agency (EMA) guidelines on patient-reported outcomes.

Design –

Three phases of instrument development were conducted for both the early childhood (0–5 years old; parent proxy) and adolescent/young adult (13– 22 years old) versions of the QoL-CI. Phase 1 included development of our conceptual framework, which informed the discussion guides for stakeholder focus groups (e.g., audiologists, physicians, therapists) at CI clinics in Miami and Philadelphia (n=39). Open-ended interviews with parents (N= 18 for early childhood; N = 6 for adolescent/young adult version) and adolescents/ young adults using CIs (n=17) were then completed at both sites during Phase 2. All interviews were transcribed and coded to identify common themes, which were then used to draft items for the QoL-CI. Both versions of the QoL-CI were developed using Qualtrics to allow for quick, easy electronic administration of the instruments on a tablet device. Lastly, Phase 3 included cognitive testing in a new sample (N= 19 early childhood, N = 19 adolescent) to ensure the draft instruments were clear, comprehensive, and easy to use.

Results –

Participant responses obtained via the open-ended interviews yielded an early childhood and adolescent version of the QoL-CI that was reportedly easy to complete and comprehensive. The final QoL-CI Early Childhood instrument yielded 35 questions across 8 functional domains (Environmental Sounds, Communication, Social Functioning, Behavior, CI Device Management and Routines, School, CI Benefits, and Early Intervention). Similarly, the final QoL-CI Adolescent/Young adult version consisted of 46 items across 8 domains (Noisy Environments, Communication, CI Usage and Management, Advocacy, Social Functioning, Emotional Functioning, Acceptance, Independence).

Conclusions –

The QoL-CI is a condition-specific quality of life instrument that can be used for children ages birth through 22 years. These instruments capture the “whole” child by not only focusing on communication and auditory skills, but also academic, social and emotional functioning. Once validated, these CI-specific measures will enable providers to track long-term outcomes and evaluate the efficacy of new interventions to improve overall CI use and quality of life for pediatric and young adult users.

Introduction

Over the past two decades, tremendous progress has been made in defining and measuring health-related quality of life (HRQoL) and in recognizing its importance as a key health outcome measure (Crosby et al. 2003; Garratt et al. 2002; Higginson & Carr, 2001; Palermo et al. 2008; Quittner et al. 2009). The Centers for Disease Control has identified quality of life as a central public health goal for three Healthy People initiatives (CDC, 2020). HRQoL measures provide unique information about the impact of a disability and its treatment on an individual’s functional ability (Palermo et al. 2008; Quittner et al. 2009). Both the Food and Drug Administration (FDA) and European Medicines Agency (EMA) have both recommended that condition-specific, rather than generic, HRQoL measures be developed and implemented (FDA 2009; EMA 2005). Despite this recognition, little is known about quality of life of children using cochlear implants (CIs) and there are currently no validated instruments of quality of life that can be used throughout childhood. Thus, this study aimed to develop condition-specific quality of life instruments that can be used from early childhood through adolescence and young adulthood.

Although conventional measures of auditory and communication abilities are essential, they do not adequately capture the broader impact of childhood hearing loss on the patient’s physical, behavioral, social and emotional functioning. This is particularly true for children with severe to profound hearing loss receiving CI(s). This level of hearing loss is associated with measurable deficits in HRQoL, reflecting the broad effects of hearing loss and its concomitant effects on language learning, social and emotional functioning, and academic performance. Studies of cochlear implantation typically focus on clinical measures of efficacy related to communication (e.g., auditory skills, speech), which does not represent the crucial assessment of the impact of CI’s on everyday functioning (communication, emotional, social). HRQoL is defined as a multidimensional construct that captures four core domains: 1) disease state and physical symptoms, 2) functional status (e.g., performing daily activities), 3) emotional functioning, and 4) social functioning (Hays 2005; Rothman et al. 2007). The importance of considering broader parent and patient-reported outcomes, such as HRQoL, has been adopted as a priority by the National Institute of Health, the Food and Drug Administration, and the Center for Medicare and Medicaid services (FDA Guidance 2009; NIH 2005; CMS 2016). Assessing how children and adolescents, as well as parents of pediatric CI recipients, perceive their health and functional ability is part of the movement to establish evidence-based, family-centered care that includes patients and family members in healthcare decision-making.

HRQoL measures can be generic or condition-specific. Generic instruments typically include global or summary ratings of multiple domains and allow for comparison across different groups of patients. However, they lack precision and sensitivity in detecting changes over time (Quittner et al., 2003). In contrast, condition-specific measures of HRQOL address the challenges associated with a particular condition and have greater clinical relevance to patients and families. Rigorous standards for the development and psychometric evaluation of HRQoL measures have been established, and efforts to develop reliable and valid measures of HRQoL have been successful (Hoffman et al. 2019; McRackan et al. 2019; Quittner et al. 2012; Sawicki et al. 2011). Condition-specific HRQoL measures contain items that come directly from patient and parent interviews, and reflect the domains of functioning that are relevant and deemed important by them (Wiebe et al. 2003). This relatively new field has generated excitement and rapid development of condition-specific HRQoL measures for adult and pediatric populations with chronic conditions, such as asthma, cystic fibrosis, diabetes and cancer (Bujang et al. 2018; Cruz-Cejas & Quittner 2009; Fayers & Bottomley, 2002; Juniper et al. 1992; Quittner et al. 2005; Quittner et al. 2013).

HRQoL Measures

A review of the literature on childhood deafness and cochlear implantation illustrated the lack of condition-specific measures for this population. Although children with hearing loss all have some degree of sensory deprivation, the challenges of children using CIs are unique. Children with CIs have severe to profound hearing loss and in general experience more language delays and academic difficulties (Niparko et al. 2010; Jorgensen et al. 2018). The audiological management is also different, including frequency of appointments compared to children with mild or moderate hearing losses using hearing aids. According to the Pediatric Minimum Speech battery, children using CIs with less than one years’ experience should be followed every 2–3 months compared to hearing aid users that should be followed every 3 months (Uhler, Warner-Czyz, Gifford, & PMSTB Group 2017). Further, data supports that children with CIs also experience different social and academic challenges compared to children using other hearing devices (Harris & Terlektsi, 2011; Theunissen et al. 2012; Toblin et al. 1999). Thus, these studies support the need for a CI-specific measure that adequately captures overall functioning for children using CIs.

Despite the evidence of the unique challenges of CI users, the majority of studies have used generic rather than condition-specific measures. For example, The Pediatric Quality of Life Inventory (Peds-QL; Varni 2001) is a common measure used to assess quality of life in children and adolescents ages 2–18 years. Although the Peds-QL offers some condition-specific modules (e.g., asthma, cancer, diabetes), a hearing loss specific module is not available. Other HRQoL measures used in research with children with CIs include the SF-36, Health Utilities Index, and KINDL (Beadle et al. 2000; Cheng et al. 2000; Krabbe et al. 2000; Zaidman-Ziat et al. 2008; Meserole et al. 2014; Ravens-Sieberer & Bullinger 1998). The KINDL has been used in previous studies to compare children with CIs to hearing peers, however, studies have demonstrated mixed results (Warner-Czyz et al. 2009; Loy et al. 2010; Huber 2005). Specifically, Warner-Czyz and colleagues (2009) found that children ages 4–7 with CIs rated their HRQoL similarly to hearing peers while Huber (2005) noted that children ages 8–12 with CIs rated their HRQoL lower than a normative sample. The aforementioned measures are insufficient and have several limitations. Specifically, items are not normed according to developmental periods (e.g., early childhood, adolescence), they lack inclusion of core domains of HRQoL, and do not capture the common challenges experienced by children with hearing loss.

Hearing loss specific measures of HRQoL are limited. To our knowledge, the Hearing Environments and Reflection on Quality of Life (HEAR-QL-26; Umansky et al. 2011) is the only hearing loss specific measure that is available. The HEAR-QL was used to assess children using hearing aids, personal frequency modulation (FM) systems, or CIs and was developed using existing literature and focus groups with children with mild to profound hearing losses and their parents. The developers did not use the process of instrument development advocated by the FDA or EMA (EMA 2005; FDA 2009) and used an extremely heterogeneous sample of children in creating the items. Furthermore, they did not include domains of functioning that represent key HRQoL dimensions (e.g., use of device--either hearing aids/CIs; Morettin et al. 2013). Additionally, this measure is only available for use for children ages 7–12 years, does not have a parent-proxy measure, and only included 8 children with a CI in their sample.

There are three measures assessing HRQoL in children with CIs via parent report (Table 1). The Parent Views and Experiences with Pediatric Cochlear Implant Questionnaire (PEVCIQ; Nunes et al. 2005) focuses on children ages 5–16 years and is administered using a semi-structured interview, which can be time intensive. Unfortunately, this measure would be difficult to widely implement, given the training and resources necessary to administer a semi-structured interview. The Children’s Quality of Life Questionnaire (Chmiel et al. 2000) covers the full developmental range and is intended for children and adolescents ages 3–20 years who are CI users. However, this questionnaire only measures two subscales (benefits and problems) and does not measure any of the core domains of functioning (social), making it an inadequate measure for assessing HRQoL in children and adolescents with CIs. There is also the Children with Cochlear Implants: Parental Perspectives measure (CCIP; Archbold et al. 2002). This questionnaire was designed for children who were implanted for at least 2 years and diagnosed with hearing loss by 3 years of age. Because it is parent report only, this measure lacks the perspective of school-age children and teens which is critical to assessing their quality of life. In addition, children need to be implanted for at least two years, thus, this measure does not capture young children who were recently implanted and any potential areas that might need intervention.

Table 1.

CI Specific QoL Measures

	Age	Respondent	Domains
Children with Cochlear Implants: Parental Perspectives measure (CCIP; Archbold et al. 2002) Validated Version: Parent Views and Experiences with Pediatric Cochlear Implantation Questionnaire (PEVCIQ; Nunes et al., 2005)	Childhood: implanted by 2 years, deafened by 3 5–16	Parent Report	Communication, General Functioning, Self-Reliance, Well-Being and Happiness, Social Relationships, Process of Implantation, Education, Effects of Implantation, Decision to Implant, and Supporting the Child
The Children’s Quality of Life Questionnaire (Chmiel et al. 2000)	3–20	Parent Report	Benefits, Problems
Quality of Life- Cochlear Implant (QoL-CI) (Hoffman et al. 2019)	6–12	Self-Report and Parent Report	Self-Report: Academic Functioning, Child Acceptance, Device Management, Emotional Functioning, Fatigue, Noisy Environments, Oral Communication, and Social Functioning Parent Report: Same domains as self-report with addition of Behavioral Problems

To ameliorate the gap in the literature, Hoffman and colleagues (2019) developed a CI-specific HRQoL measure for school-age children, the Quality of Life- CI. This measure was developed following the FDA guidance and NIH recommendations and includes a self-report and parent-proxy version for children ages 6–12 years. This measure captures children’s academic functioning, acceptance of hearing loss, listening fatigue, device management, as well as other domains (see Hoffman et al. 2019 for further details). The current study uses the same rigorous standards, methods, and investigative team as the Hoffman and colleagues (2019) study. This study expands the QoL-CI instruments to the early childhood and adolescent developmental periods.

The availability of a CI-specific HRQoL for pediatrics will complement the already available CI-specific measure for adults, the Cochlear Implant Quality of Life (CIQOL-35; McRackan et al. 2019). The CIQOL-35 consists of 35 items in six domains (communication, emotional, entertainment, environment, listening effort, and social). This measure was created following FDA and NIH recommendations and demonstrates the need for CI specific measures. The availability of CI-specific measures for both pediatrics and adults will allow for tracking and monitoring of quality of life throughout the lifespan.

Thus, the purpose of this study was to develop and expand the Quality of Life-CI instruments for early childhood (0–5 years) and adolescence/young adulthood (13–22 years). The QoL-CI instruments will cover the entire pediatric age spectrum from infancy to age 22, providing developmentally appropriate instruments to track longitudinal outcomes. Our measure includes young adults 18–22, as this important developmental window captures adolescents finishing high school and transitioning toward independence. This will enable hearing health providers to evaluate changes in HRQoL over time, test new devices and processors, and identify deficits in functioning (e.g., social skills, adjustment problems) that require assistance and further support or intervention.

Materials and Methods

We engaged in a three-phase process to develop the early childhood and adolescent/young adult versions of the Quality of Life- CI instruments. This study was reviewed and approved by the University of Miami Institutional Review Board (IRB). Phase 1 consisted of a comprehensive literature review, development of a conceptual framework, and focus groups with key stakeholders/providers (e.g., CI surgeons, audiologists, auditory-verbal therapists,). Phase 2 consisted of qualitative interviews with parents of children with CIs ages 0–5 and patients with CIs ages 13 and older. A small sample of parents of adolescent CI users also completed open-ended interviews to ensure that we were not missing any important areas of functioning. Phase 2 also included content analysis of interviews which generated saturation matrices and item generation for the preliminary Quality of Life-CI instruments. Lastly, during Phase 3 cognitive testing was completed in a new sample to ensure the draft instrument was clear, comprehensive, and easy to use. Each of these phases was completed for both an early childhood group (Ages 0–5) and adolescent/young adult group (Ages 13–22).

Participants

Phase 1- Stakeholder Focus Groups:

Six focus groups were conducted with stakeholders (n = 39) from two national pediatric CI centers: The University of Miami Cochlear Implant program; n = 5) and Children’s Hospital of Philadelphia Cochlear Implant Program (CHOP; n = 1). Participants consisted of an array of multidisciplinary healthcare providers (n = 39), including audiologists and speech and language pathologists. See Table 2 for demographic data.

Table 2.

Sample Characteristics for Stakeholders

Sample Characteristics	Early Childhood n = 23	Adolescent/Young Adult n = 16
Age:
N (%) 21–30	5 (22%)	4 (25%)
N (%) 31–40	8 (35%)	4 (25%)
N (%) 41–50	4 (17%)	5 (31%)
N (%) 51–60	5 (22%)	2 (13%)
N (%) 61–70	0 (0%)	0 (0%)
N (%) Above 71	0 (0%)	1 (6%)
Sex: N (%) female	20 (87%)	4 (88%)
Race
N (%) White	20 (87%)	15 94%)
N (%) Black	0 (0%)	0 (0%)
N (%) Asian	2 (9%)	1 (6%)
N (%) Native Hawaiian/ Pacific Islander	0 (0%)	0 (0%)
N (%) Biracial	1 (4%)	0 (0%)
Ethnicity
N (%) Hispanic/Latino	10 (43%)	2 (13%)
N (%) Not Hispanic/Latino	13 (57%)	14 (88%)
Education:
N (%) Highschool Diploma/GED	3 (13%)	0 (0%)
N (%) Bachelors	4 (17%)	1 (6%)
N (%) Masters	7 (30%)	4 (25%)
N (%) MD	3 (13%)	1 (6%)
N (%) Doctorate	6 (26%)	10 (63%)
Site:
N (%) University of Miami	14 (61%)	9 (56%)
N (%) Children’s Hospital of Philadelphia	0 (0%)	7 (44%)
N (%) Debbie School (Auditory/Oral Preschool)1	9 (39%)	0 (0%)
Profession:
N (%) Audiologist	8 (35%)	10 (63%)
N (%) Speech & Language Pathologist	5 (22%)	2 (13%)
N (%) Physician	3 (13%)	1 (6%)
N (%) Educator for the Deaf	3 (26%)	2 (13%)
N (%) Social Worker	0 (0%)	1 (6%)
N (%) Other	1 (4%)	0 (0%)
Years in Practice:
N (%) < 1 year	2 (9%)	0 (0%)
N (%) 1–2 years	1 (4%)	2 (13%)
N (%) 3–5 years	6 (26%)	3 (19%)
N (%) 5–10 years	8 (35%)	3 (19%)
N (%) 10+ years	6 (26%)	8 (50%)

¹The Debbie School is an early intervention and auditory-oral program that focuses on listening and spoken language development in deaf and hard of hearing children. This program provides early intervention and educational services for children who are deaf or hard of hearing ages birth to nine. Services include early and primary education.

Early Childhood Measure

Phase 2 Open Ended Interviews (n=19):

Inclusion criteria for parent-child dyads were: 1) children with unilateral or bilateral CIs, 2) chronological age between 0 years, 1 months to 5 years, 11 months, and 3) able to communicate in English and/or sign language. Exclusion criteria included: 1) children with developmental disabilities that severely limited their ability to complete open-ended or cognitive interviews (e.g., severe cerebral palsy or Autism Spectrum Disorder), and 2) children/parents who did not speak English and/or sign language. Recruitment was conducted via distribution of flyers to all families of children with CIs in our age range.

Participants had a mean age of 4.29 years (SD = 1.29), 47.4% were female, and over half were White (52.6%). Insurance data indicated that 47.4% had Medicaid, and 47.4% had private insurance. Most were bilateral CI users (84.2%), had congenital hearing loss (78.9%), and were diagnosed within a year after birth (84.2%). Table 3 provides additional descriptive data. Parents who completed Phase 2 were mostly mothers (89.5%), within the ages of 30–39 years (68.4%) and most earned a college degree (47.4%). Table 4 provides additional descriptive data.

Table 3.

Sample Characteristics for Children and Adolescents Enrolled in Open-Ended Interviews and Cognitive Testing

Sample Characteristics	Open-Ended Interviews		Cognitive Testing
	Early Childhood	Adolescent/ Young Adult	Early Childhood	Adolescent/ Young Adult
Age: M (SD)	4.29 (1.29) years	17.06 (3.17) years	3.46 (1.36) years	17.05 (3.03) years
Sex: N (%) female	9 (47.4%)	9 (52.9%)	7 (36.8%)	9 (47.4%)
Race:
N (%) White	14 (73.7%)	16 (94.1%)	12 (63.2%)	15 (78.9%)
N (%) Black	3 (15.8%)	0 (0%)	2 (10.5%)	0 (0%)
N (%) Asian	0 (0%)	1 (5.9%)	3 (15.8%)	2 (10.5%)
N (%) Native Hawaiian/ Pacific Islander	1 (5.3%)	0 (0%)	0 (0%)	0 (0%)
N (%) Biracial	1 (5.3%)	0 (0%)	2 (10.5%)	2 (10.5%)
Ethnicity
N (%) Hispanic/Latino	4 (21.1%)	5 (29.4%)	5 (26.3%)	3 (15.8%)
N (%) Not Hispanic/Latino	15 (78.9%)	12 (70.6%)	14 (73.7%)	16 (84.2%)
Insurance:
N (%) Uninsured	0 (0%)	1 (9.1%)	0 (0%)	2 (16.7%)
N (%) Medicaid	10 (52.6%)	5 (45.5%)	9 (47.4%)	3 (25.0%)
N (%) Private	9 (47.4%)	5 (45.5%)	10 (52.6%)	7 (58.3%)
Onset type:
N (%) Sudden	2 (10.5%)	3 (21.4%)	1 (7.1%)	1 (5.3%)
N (%) Progressive	2 (10.5%)	4 (28.6%)	1 (7.1%)	7 (36.8%)
N (%) Congenital	15 (78.9%)	7 (50.0%)	12 (85.7%)	11 (57.9%)
Device:
N (%) Unilateral	1 (5.3%)	6 (35.3%)	2 (12.5%)	7 (36.8%)
N (%) Bimodal	2 (10.5%)	1 (5.9%)	0 (0%)	0 (0%)
N (%) Bilateral	16 (84.2%)	10 (58.8%)	14 (87.5%)	12 (63.2%)
Age at First Implantation
N (%) < 1 Year Old	3 (15.8%)	0 (0%)	2 (11.1%)	0 (0%)
N (%) 1–2 Years Old	10 (52.6%)	3 (17.6%)	11 (61.1%)	4 (21.1%)
N (%) 2–3 Years Old	2 (10.5%)	1 (5.9%)	1 (5.6%)	5 (26.3%)
N (%) 3–4 Years Old	1 (5.3%)	3 (17.6%)	4 (22.2%)	2 (10.5%)
N (%) 4–5 Years Old	1 (5.3%)	1 (5.9%)	0 (0%)	0 (0%)
N (%) 5–7 Years Old	2 (10.5%)	4 (23.5%)	0 (0%)	1 (5.3%)
N (%) > 7 Years Old	0 (0%)	5 (29.4%)	0 (0%)	7 (36.8%)
Duration of Cochlear Implantation
N (%) 0–6 Months	2 (10.5%)	0 (0%)	5 (27.8%)	0 (0%)
N (%) 6–12 Months	2 (10.5%)	0 (0%)	2 (11.1%)	0 (0%)
N (%) 1– 2 Years	5 (26.3%)	0 (0%)	5 (27.8%)	0 (0%)
N (%) 2–3 Years	3 (15.8%)	1 (5.9%)	4 (22.2%)	0 (0%)
N (%) 3–4 Years	6 (31.6%)	0 (0%)	1 (5.6%)	3 (15.8%)
N (%) 4–5 Years	1 (5.3%)	1 (5.9%)	1 (5.6%)	0 (0%)
N (%) 5–10 Years	0 (0%)	3 (17.6%)	0 (0%)	5 (26.3%)
N (%) >10 Years	0 (0%)	12 (70.6%)	0 (0%)	11 (57.9%)
School Setting:
N (%) Mainstream1	3 (20.0%)	11 (64.7%)	5 (29.4%)	15 (78.9%)
N (%) Mainstream+pullout2	2 (13.3%)	2 (11.8%)	0 (0%)	2 (10.5%)
N (%) Auditory oral classroom3	7 (46.7%)	0 (0%)	4 (23.5%)	0 (0%)
N (%) Special education classroom4	0 (0%)	0 (0%)	1 (5.9%)	0 (0%)
N (%) Total communication classroom5	0 (0%)	1 (5.9%)	4 (23.5%)	0 (0%)
N (%) School for the Deaf6	3 (20.0%)	0 (0%)	0 (0%)	2 (10.5%)
Child grade:
N (%) Daycare	2 (11.8%)	-	2 (12.5%)	-
N (%) Preschool	2 (11.8%)	-	8 (50.0%)	-
N (%) Pre-Kindergarten	10 (58.8%)	-	5 (31.3%)	-
N (%) Kindergarten	3 (17.6%)	-	1 (6.3%)	-
N (%) Middle school	-	4 (23.5%)	-	4 (23.5%)
N (%) High school	-	11 (64.7%)	-	13 (76.5%)
N (%) College	-	2 (11.8%)	-	0 (0%)

^1.General education classroom;

^2.General education classroom where the student is pulled out to receive support or intervention;

^3.A classroom for children with hearing loss taught by a teacher of the deaf and hard of hearing focusing on listening and spoken language development;

^4.Self-contained classroom for children with various special needs;

^5.Deaf and hard of hearing classroom where teachers utilize both listening and spoken language and American Sign Language;

^6.School for students who are deaf and/or hard of hearing

Table 4.

Sample Characteristics for Parents Enrolled in Open-Ended Interviews and Cognitive Testing

Sample Characteristics	Open-Ended Interviews		Cognitive Testing
	Early Childhood	Adolescent/ Young Adult	Early Childhood	Adolescent/ Young Adult
Relationship to child
N (%) Mother	17 (89.5%)	6 (46.2%)	15 (78.9%)	6 (40%)
N (%) Father	2 (10.5%)	5 (38.5%)	4 (21.1%)	1 (6.7%)
N (%) Grandparent	0 (0%)	1 (7.7%)	0 (0%)	0 (0%)
N (%) Self	0 (0%)	1 (7.7%)	0 (0%)	8 (53.3%)
Age:
N (%) 20–29	1 (5.3%)	1 (7.7%)	8 (42.1%)	2 (22.2%)
N (%) 30–39	13 (68.4%)	1 (7.7%)	8 (42.1%)	1 (11.1%)
N (%) 40–49	4 (21.1%)	6 (46.2%)	3 (15.8%)	5 (55.6%)
N (%) 50–59	0 (0%)	3 (23.1%)	0 (0%)	0 (0%)
N (%) 60–69	1 (5.3%)	2 (15.4%)	0 (0%)	1 (11.1%)
Race:
N (%) White	14 (52.6%)	12 (38.5%)	13 (42.1%)	9 (72.7%)
N (%) Black	3 (15.8%)	0 (0%)	2 (10.5%)	0 (0%)
N (%) Asian	0 (0%)	1 (7.7%)	3 (15.8%)	1 (9.1%)
N (%) Native Hawaiian/Pacific Islander	0 (0%)	0 (0%)	0 (0%)	0 (0%)
N (%) Biracial	2 (10.5%)	0 (0%)	1 (5.3%)	1 (9.1%)
Ethnicity
N (%) Hispanic/Latino	4 (21.1%)	7 (53.9%)	5 (26.3%)	1 (9.1%)
N (%) Not Hispanic/Latino	15 (78.9%)	6 (46.2%)	14 (73.7%)	11 (90.1%)
Education:
N (%) Less than high school	0 (0%)	0 (0%)	1 (5.3%)	1 (12.5%)
N (%) Complete high school	2 (10.5%)	2 (15.4%)	3 (15.8%)	0 (0%)
N (%) Some college	1 (5.3%)	4 (30.8%)	1 (5.3%)	0 (0%)
N (%) Associates degree	4 (21.1%)	0 (0%)	4 (21.1%)	1 (12.5%)
N (%) Completed college	9 (47.4%)	6 (46.2%)	6 (31.6%)	3 (37.5%)
N (%) Graduate school or higher	3 (15.8%)	1 (7.7%)	4 (21.1%)	3 (37.5%)
Income:
N (%) Less than $15,000	0 (0%)	0 (0%)	2 (10.5%)	0 (0%)
N (%) $15,000–29,999	1 (5.3%)	2 (16.7%)	2 (10.5%)	2 (16.7%)
N (%) $30,000–49,999	5 (26.3%)	2 (16.7%)	4 (21.1%)	0 (0%)
N (%) $50,000–74,999	0 (0%)	2 (16.7%)	4 (21.1%)	0 (0%)
N (%) $75,000–100,000	3 (15.8%)	1 (8.3%)	3 (15.8%)	2 (16.7%)
N (%) More than $100,000	6 (31.6%)	4 (33.3%)	3 (15.8%)	4 (33.3%)
N (%) Don’t know/Declined to answer	4 (21.0%)	1 (8.3%)	1 (5.3%)	4 (33.3%)
Primary language spoken at home:
N (%) English	18 (94.7%)	9 (69.2%)	14 (73.7%)	10 (90.9%)
N (%) Spanish	1 (5.3%)	4 (30.8%)	2 (10.5%)	1 (9.1%)
N (%) Other	0 (0%)	0 (0%)	3 (15.8%)

Phase 3 Cognitive Testing (n = 19):

Inclusion and exclusion criteria were the same as Phase 2. Participants had a mean age of 3.46 years (SD = 1.36), 35% were female, and 36.8% were White. Insurance data indicated that 47.4% had Medicaid and 52.6% had private insurance. The majority of the sample were bilateral CI users (87.5%), had congenital hearing loss (65%), and were diagnosed within a year after birth (73.7%). Table 2 provides additional sample descriptive data. Parents who completed Phase 3 were mostly mothers (80%), within the ages of 20–29 years (45%) and earned a college degree (30%). Table 4 provides additional sample descriptive data.

Adolescent/Young Adult Measure

Phase 2 Open Ended Interviews (n=36):

Inclusion criteria for parent-child dyads were: 1) children with unilateral or bilateral CIs, 2) chronological age between 13 years, 1 months – 21 years, 11 months and 3) able to communicate in English and/or sign language. Exclusion criteria included: 1) children with developmental disabilities that severely limited their ability to complete open-ended or cognitive interviews (e.g., severe cerebral palsy or Autism Spectrum Disorder), and 2) children/parents who did not speak English and/or sign language. Recruitment was conducted via distribution of flyers to all families of children with CIs in our age range.

Participants had a mean age of 17.05 years (SD = 3.03), 47.4% were female, and over half were White (63.2%). Insurance data indicated that 14.3% were uninsured, 21.4% had Medicaid, and 50% had private insurance. More than half of the sample were bilateral CI users (63.2%), had congenital hearing loss (57.9%), and were diagnosed within one year after birth (58.8%). Table 3 provides additional sample descriptive data. Parents who completed Phase 3 were mostly mothers (60%), within the ages of 40–49 years (45.5%) and earned a college or graduate degree (36.5%; 36.5%). Table 4 provides additional sample descriptive data.

Phase 3 Cognitive Testing (n = 17):

Inclusion and exclusion criteria were the same as Phase 2. Participants had a mean age of 17.06 years (SD = 3.17), 52.9% were female, and over half were White (64.7%). Insurance data indicated that 9.1% were uninsured, 45.5% had Medicaid, and 45.5% had private insurance. Approximately 59% of the sample were bilateral CI users (58.8%), had congenital hearing loss (41.2%), and were diagnosed within one year after birth (47.4%). Table 2 provides additional descriptive data. Parents who completed Phase 2 were mostly mothers (46.2%), within the ages of 40–49 years (46.2%) and earned a college degree (46.2%). Table 4 provides additional sample descriptive data.

Procedures

The process of instrument development followed the steps outlined in the FDA Guidance on Patient Reported Outcomes (PRO; FDA Guidance, 2009). As part of this study, we completed the first two steps in the PRO Measurement Wheel, which requires a conceptual framework that specifies the population and key concepts. The guidance recommends a comprehensive review of everything that matters to patients and for item development to use language taken directly from patients. The first step of this process includes the comprehensive literature review, followed by focus groups to elicit as many concepts as possible to capture the entire impact of hearing loss.

Phase 1:

This phase consisted of a literature review, development of a conceptual model and discussion guides for the focus groups and open-ended interviews. Prior measures of quality of life were reviewed, in addition to other important concepts related to the effects of hearing loss on functional ability (e.g., academic performance). Next, focus groups were conducted with providers using semi-structured discussion guides. Stakeholder focus groups were conducted to elicit the perspective of hearing healthcare providers on the impact of hearing loss and CIs on quality of life. Specific items discussed with the stakeholder focus groups included both global questions (i.e. “Tell me a little bit about how having CIs has affected the teens/young adults and families you work with.”), as well as specific questions (i.e. “Do they report removing their device(s) in certain situations?; How would you describe their ability to focus and/or pay attention?”).

Phase 2:

Individual open-ended interviews were conducted using the discussion guides from Phase 1. Interviews were audiotaped, transcribed, and coded for content analysis using NVivo (Richards, 2005). Participants whose preferred language was American Sign Language, completed the interview with an interpreter present.

Phase 3:

Two preliminary draft questionnaires (QoL-CI) were developed using data obtained from the content analyses of Phases 1–2 for each age group. The draft measures were then tested using cognitive, “think aloud” procedures (Schwarz & Sudman 1996; Quittner et al. 2000) with a new sample. These “think aloud” procedures have been used in previous research (Hoffman et al. 2019; Quittner et al. 2013). Think aloud interviews include questions about what each item means and their rating of the item’s importance and relevance (e.g., “Is this question relevant to your child?,” How often do you deal with this issue?,” Are we missing an important issues?). In addition, the probing questions help to identify the reference period the respondent was using to answer the questions and adds information regarding their interpretation of the items. Paraphrasing by respondents’ sheds light on whether they understood the terms and the contexts that were used, and whether they extracted the intended meaning from the items.

All interviews were audio-recorded and transcribed. As with Phase 2, participants whose preferred language was American Sign Language, completed the interview through an interpreter. Data from this Phase was used to modify the instruments accordingly. Based on FDA recommendations, a one-week recall period was utilized to ensure that participants rated recent events that were not influenced by poor memory or recall (FDA 2009; Quittner et al. 2013).

Measures

Demographic and Medical Questionnaire:

Background questionnaires were completed by both providers and parents. The brief demographic questionnaire assessed key demographic information (e.g., age, gender, race/ethnicity, educational background, child’s etiology). Medical chart reviews were also performed to obtain medical (e.g., date of diagnosis/implantation) and audiological (e.g., aided access, speech perception scores, data logging) data for the children and adolescents.

QoL-CI:

For the early childhood version, a parent-proxy version was administered. Similarly, the adolescent/young adult version was completed by the CI recipient electronically. For both the parent-proxy and self-report versions, questionnaires were administered electronically using iPads. Questionnaires were created using Qualtrics, a HIPAA compliant online survey program. During Phase 3, parents and adolescents completed the questionnaires, followed by the cognitive testing interviews. The questionnaires were administered via iPad with automatic scoring. Items were scored on a 4-point Likert scale. For each domain, a mean score was calculated and then transformed into a scaled score using the following formula [(mean of responses)−1)/3] × 100. Consistent with previous literature, if more than half of the domain responses were missing, the domain score was not calculated (Siddiqui 2015). For the current study, there was no missing data as all participants completed each item of the QoL-CI.

Data Analysis

Content Analyses of Open-Ended Interviews:

Content derived from the provider focus groups and open-ended interviews were used for item generation. All transcripts were uploaded into NVivo, a software analysis program for qualitative data. The coding team consisted of the principal investigator, co-investigators and research assistants/coordinators. Meetings were held throughout the coding process to discuss questions and challenges related to coding. Interrater reliability was calculated using Cohen’s kappa. For further detail regarding coding procedures, please see Hoffman et al., 2019.

Saturation of Content from Open-Ended Interviews

Saturation of content was calculated by assessing the frequency with which each item was mentioned by adolescents and parents, and the point at which no new content was identified. We followed the recommendations by Guest and colleagues (2006) which define saturation as the point in which data collection is not contributing to any changes to the codebook. NVivo saturation matrices were used to guide the item generation phase for the QoL-CI instruments.

Development of Draft Measures:

Items were generated to reflect the most frequent themes identified in the saturation matrices (occurring across at least 25% of transcripts) and aggregated into scales based on the conceptual framework in Phase I. Items were written using language from the open-ended interviews by parents and adolescents to improve the clarity, comprehension, and content validity of the measures (Creswell & Miller 2000). Frequent themes were aggregated into scales based on the original framework.

Cognitive Testing:

Cognitive interviewing techniques are now widely used to gain information from respondents about how they understand and formulate their answers to questionnaires (Quittner et al., 2000). They have been used to identify a variety of problems with questionnaires, such as awkward wording, redundancy of items, problematic terms, and difficult response options. These techniques have recently been applied successfully to the development of HRQoL instruments (Quittner, Cejas, & Blackwell, 2013). During the cognitive interview process, we captured patient and parent feedback on the items and used an iterative process to modify the questions as needed to improve their clarity and comprehensiveness of the QoL-CI instruments.

Data from the cognitive testing phases were used to examine preliminary floor and ceiling effects and item distributions (Hays & Hayashi 1990; Hays et al. 1993). Responses to each question were grouped together to identify potential problems with the items (e.g., no variability across respondents) or response scales (e.g., floor, ceiling effects). Revisions were made to develop the final draft instruments for both the QoL-CI early childhood and adolescent/ young adult versions.

Results

Early Childhood Measure:

Phase 1:

Stakeholder Interviews:

Twenty-three providers (n=23) participated in the early childhood focus groups. Providers included audiologists (n=8), speech language pathologists (n=5), physicians (n=3) and educators (n=6). Their experience with CIs ranged from less than 1 year to over 10 years. Most of the providers were female (n=20). See Table 2 for complete demographic information. Communication and child behavior were among the most common themes reported by providers. Providers stated that parents often report that narrating and talking frequently is not natural for them. In addition, providers discussed the child’s frustration with difficulties communicating.

Phase 2:

Open Ended Interviews:

All transcripts (n=18) were coded using codebooks designed by the research team. The final version of the codebook had 20 major themes and 62 subthemes. The themes were divided into larger categories (Environmental Sounds, Communication, Social Functioning, Behavior, CI Device Management and Routines, School, CI Benefits, and Early Intervention). These themes were used to develop the items that were evaluated in the cognitive testing phase.

Major themes that occurred at 100% saturation, meaning all parents mentioned at least one of the subthemes, were “child autonomy,” “CI benefit,” “CI candidacy,” “communication,” “device management,” “family,” and “peer interactions”. Other major themes that also had high saturation were “behavior” (94.4%), “physical/recreational activity” (94.4%), “school” (88.9%), and “therapies” (94.4%).

Fifty themes occurred at a frequency greater than 25%, and saturation was achieved by the 9th interview for all subthemes. indicating that no new information emerged after the 9th interview. A review of the major themes indicated that saturation occurred after the fourth interview. See supplemental material for the saturation matrix with the 20 major themes.

Item Generation:

By looking at the subthemes and major themes, 8 broad categories were developed to encompass all items for the questionnaire. The categories were Environmental Sounds, Communication, Social Functioning, Behavior, CI Device Management and Routines, School, CI Benefits, and Early Intervention.

Using the transcripts and saturation matrix, items were created using the language that parents used. For example, when parents were asked about their child’s behavior, many mentioned the word “tantrum.” A question was created to reflect this; “How often does your child have a tantrum when not getting his/her way?” The initial version of the instrument had 8 domains and 39 items.

Phase 3:

Cognitive Testing:

For the early childhood version, nineteen parents who did not participate in any of the prior phases (Table 4) completed the QoL-CI Early Childhood questionnaire and provided feedback about the instrument and items. The initial version of the instrument contained 39 items across 8 domains. After examining cognitive interviews with 4 participants, some questions were altered in response to parent feedback. We added an option to skip the school section of the questionnaire if the child doesn’t attend nursery/preschool or Kindergarten. We also added an additional question in the communication section to query whether the child is using words or vocalizing/babbling.

After cognitive testing was completed, we reviewed all items and parent feedback and made necessary modifications. Overall, parents agreed that the questionnaire covered all domains of their child’s life. They enjoyed answering the questionnaire and felt the questions were easy to understand. Although the feedback was mostly positive, modifications were made to clarify some questions, remove questions that did not work, and add “not applicable” as an option for specific questions (How easy is it for your child to follow along with TV shows or movies?), and a “skip” option if the child is less than 3 years old. A few questions were slightly reworded (i.e., changing the words “academic progress” to “learn in school”).

Descriptive statistics were reviewed for each item (see Table 5). The means for scales ranged from 51.05 – 83.33 and standard deviations from 8.78 – 20.22. Item level means ranged from 1.84 – 3.74, and standard deviations ranged from .51 – 1.4. Good response variability was seen across items. Following this iterative process, the final instrument resulted in 8 domains and 35 questions (Table 6).

Table 5.

Descriptive Statistics for QoL-CI domains

	Mean	SD	Range
Early Childhood
Environmental Sounds	65.35	15.28	33.33 – 100
Communication	51.05	17.72	13.22 – 80
Social Functioning	54.21	14.11	26.67 – 75
Behavior	66.37	8.78	38.89 – 77.78
Device Management	64.04	19.28	33.33 – 88.89
School	77.63	18.01	25.00 – 100
CI Benefit	83.33	20.22	33.33 – 100
Early Intervention	64.47	17.32	25.00 – 91.67
Adolescent/Young Adult
Noisy Environments	60.96	14.97	41.67 – 91.67
Communication	61.40	14.04	33.33 – 83.33
Device Management	71.05	15.99	37.50– 95.83
Advocacy	62.11	15.40	33.33 – 100
Social Functioning	66.23	17.51	29.17 – 91.67
Emotional Functioning	69.82	18.37	40 – 100
Acceptance	68.42	22.20	26.67 – 100
Independence	58.25	14.03	33.33 – 93.33

Table 6.

QoL-CI: Number of items and domain descriptions

Domain	# of items	Description
Early Childhood
Environmental Sounds	4	Difficulty hearing in different environments
Communication	8	Ability to vocalize
Social Functioning	4	Ability to socialize with others
Behavior	5	Frequency of difficult behavior
Device Management	4	Difficulties wearing CI
School	4	Frequency of problems at school
CI Benefit	3	Improvements in functioning
Early Intervention	3	Engagement in therapies
Adolescent/Young Adult
Noisy Environments	4	Difficulty hearing in loud environments
Communication	6	Ability to vocalize
Device Management	8	Difficulties wearing CI
Advocacy	5	Comfort speaking up for yourself
Social Functioning	8	Ability to socialize with others
Emotional Functioning	5	Frequency of negative emotions
Acceptance	5	Difficulty fitting in
Independence	5	Frequency of relying on others for help

Adolescent/Young Adult Measure:

Phase 1:

Stakeholder Interviews:

Additional stakeholder focus groups were conducted to assess provider experiences with managing adolescent and young adult CI users. Sixteen providers, consisting of audiologists (n=10), speech language pathologists (n = 2), physician (n = 1), educators for the Deaf (n = 2) and a social worker (n = 1) participated. Half of the providers had 10 or more years of experience working with children with CIs (n = 8). See Table 2 for the demographics of the providers. Communication was a key area identified by providers for this age group. For example, one provider said, “I think the environment is a huge impact on how they’re hearing, but I think it’s also their language. They may not have the language, you know, the global understanding or language to fall back on to fill in the gaps and so they are working really hard in those situations.” Acceptance and social functioning were also discussed. Providers mentioned that some adolescents/young adults have no problem telling people about their hearing loss, while others are more apprehensive. They also highlighted significant variability in friendships for this age group. Providers described some CI users in this age group as “loners” or only having one friend, while others had large groups of friends.

Phase 2:

Open Ended Interviews:

Adolescents (n=17) and their parents (n=6) reported similar themes during the open-ended interviews, thus, interviews were coded using the same codebook created by the research team. The final codebook contained 20 major themes and 109 subthemes. Eight larger categories were developed using these themes (Noisy Environments, Communication, CI Usage and Management, Advocacy, Social Functioning, Emotional Functioning, Acceptance, Independence).

Parents of the adolescents/young adults had 76 subthemes that occurred at a frequency greater than 25% and saturation was not achieved until the final (7^th) interview. The adolescents/young adults discussed 71 subthemes that occurred at a frequency greater than 25%, with saturation achieved by the 14^th interview. All 20 major themes occurred at a frequency greater than 25% in both the parent and adolescent/young adult group. Saturation of the major themes was achieved by the 4^th interview for parents and the 3^rd interview for adolescents/young adults.

Given the importance of peer relationships in adolescence, the major theme of friendship was subdivided to obtain a better understanding of what adolescents’ report under this category. Topics included were “activities with friends (100% saturation)”, “bullying (88.24% saturation),” “dating (94.12% saturation),” “hearing versus Deaf world (11.76% saturation),” “making friends (82.35%),” and “support from others”(47.06%). Other major themes discussed by adolescents included “advocating for self,” “mood,” “work and volunteering,” “future,” “communication,” and “family relationships.”

While some major themes were the same in both the birth to five and adolescent/young adult measure, others were specific to the developmental period. For example, device management, communication, noisy environments and school were consistent themes reported by parents and CI users in both the early childhood and adolescent age groups. In contrast, behavior, CI candidacy and developmental milestones were only discussed by parents of children in the early childhood group. Similarly, advocating for self, mood and work were specific to the adolescent/young adult group.

Item Generation

Using the subthemes and major themes, 8 broad categories were developed to encompass all items of the questionnaire. The categories were Noisy Environments, Communication, CI Usage and Management, Advocacy, Social Functioning, Emotional Functioning, Acceptance, Independence

By reviewing the transcripts and saturation matrices, items were created for each category. Wording of the questions mirrored the language used by the adolescents/young adults. For example, adolescents/young adults used the word “mad,” while the parents typically used the word “angry.” A question on the adolescent/young adult measure reads “How often do you feel mad?”

The draft of the QoL-CI adolescent/young adult version consisted of 47 items assessing 8 domains (e.g., communication, independence, emotional functioning).

Phase 3:

Cognitive Testing

Similar to the early childhood version, we completed the cognitive testing for the QoL-CI adolescents/young adults. Nineteen adolescents (n = 19) completed the questionnaire and provided feedback (See Table 3 for sample demographics). Following the first 8 participants, revisions were made to the response choices on two scales. Specifically, wording was changed from confidence to comfortable. In addition, we removed the item; “How often are you happy about having your CI(s)” since participants uniformly responded, “very true” and felt that it was not a necessary item. Wording was also changed on a few questions to make them clearer. Specifically, the item “How comfortable are you traveling with your CI(s)” was modified to “How comfortable are you traveling away from home with your CI(s) (e.g., sleepovers, vacation, camp)?”

Overall, participants described the QoL-CI questionnaire as appropriate, clear, and easy to complete. When asked how they felt about the questionnaire, participants responded by saying “All of them (the questions) were great,” “it was good,” “it’s useful because … people don’t get it ,” “…to answer these questions it is very helpful…” Adolescents reported that the measure captured their experiences with hearing loss and CIs fully. Many reported enjoying completing the questionnaire and described the items as very relevant to their life. The only modification after the initial changes were adding a “Not Applicable” response choice to the question “How comfortable are you asking for accommodations (e.g. extra time, preferred seating, notetakers)?”. This option was added because some respondents did not need any accommodations at this point.

Descriptive statistics were reviewed for all items (see Table 5). Means for the scales ranged from 58.25 – 71.05 and standard deviations from 14.03 – 22.20. Item level means ranged from 2.26 – 3.63, and standard deviations ranged from .459 – 1.17. The final QoL-CI Adolescent/Young adult version consisted of 8 domains and 46 items (see Table 6).

Discussion

More than 50 years ago, the World Health Organization proposed the first definition of HRQoL as “a state of complete physical, mental, and social well-being, and not merely the absence of disease” (World Health Organization [WHO] 1947, p. 29). Recently, health-related quality of life and patient reported outcomes have been recognized as critical to the evaluation of the benefits of new interventions and device health outcomes (Palermo et al. 2008; Quittner et al. 2009). This is particularly true for conditions, such as hearing loss, which have documented effects on communication, behavior, and emotional functioning. The purpose of this study was to develop CI-specific health-related quality of life instruments for pediatric CI users using the FDA Guidance on Patient Reported Outcomes.

Clinicians and researchers have historically utilized generic measures of quality of life (Quittner, Cejas, Barnard, & Niparko 2016; Warner-Czyz et al. 2009). However, these measures do not adequately capture functional ability for patients with particular conditions or disabilities and are not sensitive to detecting changes based on interventions. Current literature, as well as, FDA and EMA guidelines strongly recommend use of condition or disease specific measures. Hearing loss is diverse, as it includes individuals with mild hearing loss who may not use any hearing device due to residual hearing, individuals who only use hearing devices in certain situations (i.e., school) or individuals with severe to profound hearing loss who undergo cochlear implantation. Thus, a generic hearing loss measure would not address the unique challenges of children with CIs and ignores the already identified differences between users of hearing aids and CIs, such as language delays, academic performance, and emotional functioning (Niparko et al. 2010; Jorgensen et al. 2018).

To date, the only self-report CI-specific quality of life measure for children is the QoL-CI (Hoffman et al. 2019). The QoL-CI is only available for school-age children ages 6–12, with a parent proxy. The current study utilized the FDA PRO guidance and a methodology consistent with the Hoffman and colleagues study (FDA 2009) to extend the QoL-CI to early childhood and adolescence. Our comprehensive data obtained from the focus groups, interviews, and cognitive testing provided insights into the functional ability of children with CIs across all ages. These data highlighted the importance of assessing condition-specific content that might otherwise be missed when using generic measures of quality of life. An in-depth review of the content posed by professionals, children with CIs, and their parents, identified key domains that continue to be important across the pediatric age continuum. Topics such as communication, device management, and social functioning were key areas of functioning raised in both the early childhood and adolescent versions. Furthermore, noisy environments, acceptance of hearing loss, emotional functioning, and behavior were among key areas reported during the current study which also appear on the school-age version of the QoL-CI study (Hoffman et al. 2019). Other areas, such as independence and early intervention, were topics only discussed for specific developmental periods, adolescent/young adult and early childhood, respectively.

These measures can also be used to assess the “whole child,” rather than focusing solely on auditory and linguistic performance (Cejas & Quittner 2018). All versions of the QoL-CI were developed by the same research group, using the same rigorous methods. Thus, this will allow for easy comparison of scores as the child develops. In addition, our open-ended interviews included experienced CI users, as well as new CI users, to ensure that all appropriate areas were captured regardless of where the child was in their hearing journey. Once validated, these measures will provide unique and valuable information about the effects of cochlear implantation on a child’s functional ability and expand on traditional measures of auditory and communicative abilities by assessing keys areas that are often overlooked (e.g., social, emotional, behavioral, and academic functioning).

Social functioning was a primary topic of discussion across age groups. This is consistent with national and international studies reporting social conversations, social interactions and friendships as primary areas of concern for children with hearing loss (Haukedal et al. 2018; Hoffman, Cejas, & Quittner 2016; Punch & Hyde 2011). A study in the Netherlands identified that children with CIs ages 1.5 to 5 years old had delayed social competence and emotion regulation difficulties compared to children with normal hearing (Wiefferink et al. 2012). Other studies conducted with adolescents continued to identify social functioning, school, and identity as areas of difficulty (Rich et al. 2013). These studies along with our current data highlight the need for better evaluation and monitoring of children’s social functioning. The QoL-CI may serve as a measure to quickly monitor social and emotional functioning for pediatric CI users.

Additionally, the measure is administered via an iPad or tablet which facilitates quicker completion, approximately 20 minutes, and does not require additional staff effort for data entry or analysis. This method of administration also allows parents and patients to complete the QoL measure while waiting for appointments or during audiology visits. In addition, it increases the feasibility of incorporating yearly evaluations into CI programs and allows for comparisons of quality of life throughout different developmental periods. Further, the electronic method of administration allows for the visual and audio supports that are needed for school-age children, as well as individuals with language delays. For pediatric users who may have reading difficulties, the audio option reads the question and answer choices (see Hoffman et al., 2019 for further details). Although the QoL-CI may also be administered via the traditional “paper and pencil” method, it would not allow for automatic scoring of the QoL-CI and domain scales.

Limitations

The primary limitations of this study are related to the heterogeneity of the CI population. Although efforts were made to recruit children that use different forms of communication, most of the sample used spoken communication. Eleven percent of children who participated in the adolescent open-ended or cognitive interviews utilized sign language. Importantly, children who used sign language did not respond differently to any of the items than children who used spoken language and reported that all items were appropriate and captured their functional ability. Given the location of our CI clinic, there was a high percentage of Hispanic participants and a small number of Black participants; thus, not representing the general US population. The validation of the QoL-CI should include representation of all race and ethnicity groups. Another potential limitation is that the data were obtained from two highly specialized pediatric CI centers with access to a multidisciplinary team. Other families may not have this same access, and thus, their perspectives may be different. It will be important to include a more representative sample in the psychometric validation to ensure that these measures can be used across the United States.

Clinical Implications & Future Directions

In accordance with the FDA guidance on patient reported outcomes (2009), the next step is to conduct a multisite psychometric validation with a large sample of pediatric CI users. Once these measures have been validated, they can be translated into other languages (e.g., Spanish, French). This will allow for comparison of outcomes across centers, treatment philosophies, and countries. Once validated, these measures can also be utilized in future research to compare quality of life across etiologies (i.e., genetic loss vs. sudden onset), degree of hearing loss, one vs two implants, or other audiological or medical variables. This is critical as CI candidacy continues to change, with new, expanded criteria. The QoL-CI may be used to help evaluate any changes in intervention or programming strategies. Using patient reported outcomes, in addition to objective measures of speech understanding and audiological performance, will be critical for informing future decisions on cochlear implantation.

Moreover, given that the QOL-CI evaluates the entire pediatric age range, this measure can potentially be utilized to identify predictors of success and adaptation while examining HRQoL over time. Once the psychometric validation of the QoL-CI is conducted, CI centers can administer these tools annually to identify those at-risk, target interventions to those in need, and track patient outcomes. Early identification of areas of concern is crucial in preventing long-term disorders. For example, even adolescents who are performing well audiologically, may struggle with accepting their hearing loss, and may benefit from supportive counseling to prevent depression or anxiety in the future. Quality of life assessment is an easy, cost-effective, and non-intrusive way to obtain a global picture of how an individual is feeling and functioning in daily life.

Upon completion of the psychometric validation, the QoL-CI will be a valuable clinical and research measure that may be used to further evaluate the effects of language abilities and hearing and audiology history on quality of life. Our study included children with varying language abilities to ensure that questions on the QoL-CI were appropriate for CI users that primarily used spoken communication, as well as American Sign Language. However, a multisite psychometric validation study including a large sample of CI users with varying medical and audiological backgrounds and communication modes is warranted prior to wide adoption of these QoL-CI instruments. The psychometric study will provide further information related to the internal consistency and reliability of the scales developed during this study, as well as validation of the instruments against currently available quality of life instruments and objective audiological measures (e.g., speech perception). This study may also yield normative data for CI users with varying communication skills.

In conclusion, the QoL-CI is a condition-specific quality of life instrument that can be used for children ages birth through 22. The QoL-CI self-report and parent proxy versions can be administered electronically for easy administration in clinical settings, with immediate scoring that facilitates review of results with patients and their parents. Utilization of QOL instruments as part of medical treatment supports current recommendations for providing patient-centered, collaborative care.