Gehoorverlies kan een negatief effect hebben op de ontwikkeling van kinderen. Het risico op negatieve effecten en de mate waarin deze optreden, is o.a. afhankelijk van de mate waarin toegang tot auditieve, talige en sociaal-emotionele input wordt beïnvloed door het gehoorverlies. Periodieke monitoring van verschillende aspecten van de ontwikkeling in een multidisciplinair team (of, in overleg, door de vroegbehandeling) kan negatieve effecten van gehoorverlies op de ontwikkeling (mogelijk) voorkomen of beperken, door tijdige interventie. Deze zoekvraag leidt tot aanbevelingen m.b.t. welke auditieve ontwikkelingsaspecten dienen te worden gemonitord en wanneer en hoe deze dienen te worden onderzocht.

Auditieve ontwikkeling is het proces waarbij kinderen geleidelijk het vermogen ontwikkelen om geluiden waar te nemen, te verwerken en leren er op te reageren. De ontwikkeling gebeurt in een aantal fasen: detectie, discriminatie, identificatie en begrip. We richten ons in deze module uitsluitend op instrumenten die de ontwikkeling van deze fasen meten op basis van informatie van ouders via vragenlijsten. We beschrijven dus niet welke audiometrische detectie-, discriminatie-, identificatie- en spraakverstaansmetingen verricht kunnen worden omdat we met betrekking tot die diagnostiek geen knelpunt signaleerden.

Description of studies

Bagatto (2011) published a critical review of audiological outcome measures for infants and children. Subjective outcome evaluation tools that measure auditory-related behaviors in children from birth to 6 years of age were critically appraised. Subjective outcome evaluation tools that measure auditory related behaviors for the pediatric population were located in several domains including health-related electronic databases (CINAHL, PubMed; 2008 and 2009), visually scanning reference lists from relevant studies, hand-searching key journals and conference proceedings, searching relevant internet resources, contacting experts in the area including the Network of Pediatric Audiologists of Canada, and citation searching. The following selection criteria were used: age range = birth to 6 years, questionnaire- or interview-based, parent/caregiver respondent, audiologist administered and scored, auditory-related outcomes measured, and application to infants and children who wear hearing aids. The following aspects were evaluated: conceptual clarity, normative data, measurement model, item/scale bias, respondent burden, administrative burden, retest reliability, discriminant validity, convergent validity, ecological validity, responsiveness, alternate/accessible forms, and other languages.

McCreery (2015) performed a study to examine effects of variability in children’s auditory experience on parent-reported auditory skills questionnaires and on speech recognition in quiet and in noise, for a large group of children who were followed as part of the Outcomes of Children with Hearing Loss study. All the children and parents involved in the study were recruited as part of the OCHL investigation. They gathered data on amplification, speech recognition, and parental ratings of auditory development from 306 children with congenital hearing loss (CHH). To provide a comparison, information was also collected from 185 children with typical hearing (CNH). The better-ear Pure Tone Average (PTA) at 500, 1000, and 2000 Hz for the CHH group ranged from 20 to 92.5 dB HL during the study, with a mean of 48.5 dB and a standard deviation of 14.4 dB. Of the 306 CHH, 292 were fitted with air conduction hearing aids, while seven used bone conduction devices. Additionally, seven children with mild hearing loss did not utilize any hearing aids. Subsequently, ten children received cochlear implants after enrollment in the study. Only the data collected prior to cochlear implantation were included in the analysis for those children. This study adopted an accelerated longitudinal approach. Unlike traditional single-cohort longitudinal designs, the accelerated longitudinal method involves recruiting children at various ages, facilitating a larger participant pool and allowing for a broader examination of developmental stages. Children and their families underwent an initial baseline assessment, followed by biannual visits for those under 2 years old and annual visits for those aged 2 and above. Participation in the study was maintained for up to 4 years or until the child reached 9 years of age, whichever occurred first. The effectiveness of hearing aid (HA) in transmitting speech was evaluated through probe microphone assessments conducted at each visit. These results were then compared with Desired Sensation Level Targets. Aided SII was measured. The amount of hearing aid (HA) usage on a daily basis was gauged through parental completion of an HA utilization questionnaire (Moeller et al., 2009; Walker et al., 2013; accessible at ochlstudy.org) during every visit. This questionnaire encompassed an assessment of the average hours of HA usage per day along with the extent of HA usage across various listening scenarios. Auditory development was measured by completing either the LittlEARS questionnaire (Tsiakpini et al. 2004) or the PEACH Rating Scale (Ching and Hill, 2007) or the SSQ questionnaire (Gatehouse and Noble 2004). Parents completed the LittlEARS questionnaire (35 questions designed to evaluate a child's auditory development as well as selected expressive and receptive language skills) during the 12-month, 18-month, and 2-year study visits. If a child achieved a score of 28 or higher on the LittlEARS, they were not administered the test at the subsequent study visit. This cutoff score of 28 indicates that the child has attained the maximum level of performance on the assessment. The PEACH rating scale is a questionnaire that assesses children’s listening performance in a wide range of communicative situations, including specific questions about listening in quiet and in background noise. The PEACH rating scale has 13 questions, including one question about HA use, one question about tolerance for loud sounds, six questions about quiet listening situations, and five questions about listening situations with background noise. The Speech, Spatial, and Qualities (SSQ) of Hearing Scale (Gatehouse& Noble 2004) was administered to the parents of CHH at 4-, 6- and 8-year-old study visits. Because of this choice of ages for administering the SSQ, which falls outside of the range of ages of children considered in this guideline, we have not further looked into these results.

Persson (2020) investigated hearing aid use, auditory development, and auditory functional performance in Swedish children with moderate hearing loss during the first 3 years. Participants were recruited through the Hearing and Balance Clinic at Karolinska University Hospital during 2015–2016. Children diagnosed with bilateral congenital sensorineural HL (better ear four-frequency pure-tone average 21–69 dB HL) and amplified with behind-the-ear HAs < 6 months of age were invited to participate. Children were excluded if they presented with additional disabilities and/or syndromes and one of the parents had to be native Swedish speaking. There were 38 children diagnosed with sensorineural bilateral HL through universal newborn hearing screening (UNHS) during the recruitment period of which 15 children fulfilled the criteria, and parents of 14 children gave their consent to participate in the study. During the course of the study, five children were excluded due to progression of HL, which resulted in cochlear implantations (n = 2), relocation (n = 1), no HA use (n = 1), and loss to follow-up (n = 1). The remaining nine children were fitted with HAs at a mean age of 5.1 months (range: 3.2–10 months). The initial fitting was based on auditory brainstem responses (click), and the HAs were fit to DSL Version 5.0 targets and fine-tuned. Real-ear measurements were used, which were simulated in a test box by connecting the HA to a 2-cc coupler in the real-ear measurement (Interacoustics Affinity). In cases where real-ear measurements could not be completed, average measures were used.

A reference group of CNHs (n = 30) were recruited from two well-baby clinics. Participating children entered the study between the ages of 0.5 and 4 months (M = 2). One child in the reference group relocated, resulting in 29 children (15 girls, 14 boys).

The CHL were followed according to Karolinska University Hospital’s clinical protocol with air- and bone conduction thresholds obtained at 500, 1000, 2000, and 4000 Hz (MADSEN Astera2, GN Otometrics A/S) with insert earphones (Otometrics Oto insert ER-3A from Etymotic Research Inc.) and bone oscillator (Radioear B71). In cases where a complete audiogram was not collected, the audiogram nearest at the ages of 10, 18, 24, 30, and 36 months was used. All CNH had passed the UNHS and underwent hearing screening at the ages of 10, 18, 24, and 36 months with the same equipment as the CHL. Insert earphones were used. The number of hours of HA use was collected through datalogging technology of the HAs and parental reports at the ages of 10, 18, 24, 30, and 36 months. The speech intelligibility index (SII) was calculated with the Situational Hearing Aid Response Profile software Version 7 (American National Standards Institute, 1997) for each participant. All parents completed the Swedish version of the LittlEars Auditory Questionnaire (LEAQ) (Persson et al., 2019) from individual study entry and every second month until 24 months of age. Data on functional auditory performance were collected through the Swedish version of the PEACH (Brännström et al., 2014) from all children at the ages of 18, 24, 30, and 36 months.

Yang (2020) performed a study to assess the influence of congenital and permanent unilateral hearing loss (UHL) on early prelingual auditory development (EPLAD) of infants and toddlers, measured with the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) tool. Infants and toddlers under 3 years old, who visited the Hearing Center between September 2018 and June 2019 and met the inclusion criteria, were enrolled in the study. Based on their binaural hearing grades, children were initially categorized into three groups: 1) Bilateral Normal Hearing (BNH), 2) Unilateral Hearing Loss (UHL), and 3) Bilateral Mild Hearing Loss (BMHL). The inclusion criteria for the BNH group were as follows: 1) passing the universal newborn hearing screening bilaterally, 2) absence of any risk indicators for hearing loss, 3) no reports of hearing loss from parents or professionals, and 4) normal functioning in other physiological systems. Most children in the BNH group attended the center for routine health check-ups. Unilateral Hearing Loss (UHL) was characterized by one ear having all tested frequencies (0.5, 1, 2, and 4 kHz) equal to or less than 25 dB HL, while the other ear exhibited an average pure tone threshold (PTA) across these frequencies exceeding 25 dB HL. Bilateral Mild Hearing Loss (BMHL) was defined as a bilateral PTA ranging from 26 to 40 dB HL. In both the UHL and BMHL groups, children were included based on the following criteria: 1) having permanent hearing loss without fluctuations, and 2) completing both the auditory test battery and IT-MAIS assessment. Children with potential fluctuations in hearing loss, identified impairments in other physiological systems, or diagnosed with auditory neuropathy spectrum disorder were excluded to ensure the consistency of effects on IT-MAIS assessment. IT-MAIS is a widely used structured interview to assess EPLAD of children before 3 years old. It comprises 10 items, of which one was excluded in this study. As the participants were not wearing any rehabilitation devices, item 1 in ITMAIS, referring

to the reliance on device, was not suitable and excluded. The second item evaluates the preverbal vocalization behaviors, items 3 to 6 assess the ability to detect sound in daily lives, and the last four items assess the capacity to discriminate and identify sound. Prior to the IT-MAIS assessment, parents received instructions to observe their children's auditory behaviors for approximately one week. Instructions were sent via a social networking app, WeChat. The 9-item IT-MAIS assessment was administered by a qualified and experienced auditory technician. Concurrently, demographic data and relevant medical information of participants were collected. Following the completion of the IT-MAIS assessment, the auditory test battery was conducted for participants in the Unilateral Hearing Loss (UHL) and Bilateral Mild Hearing Loss (BMHL) groups. Since the majority of the participants were under 6 months old, hearing grades were determined based on tone burst auditory brainstem response results. Routine procedures such as otoacoustic emission and acoustic immittance tests were performed, and pediatric audiometry was conducted if children were cooperative.

Bagatto et al. (2011) conducted a review of audiological outcome measures. Twelve outcome evaluation tools with specified criteria were identified prior to assigning grades for thirteen psychometric and feasibility characteristics (Andresen, 2000). Results (Table 1) indicated that four out of the 12 tools received high grades in most of the characteristics and of these four, only two would be considered clinically feasible within an outcome evaluation guideline for infants and young children.

In light of this critical review, the LittlEARS Auditory Questionnaire and the PEACH Rating Scale scored most favorably in the majority of the review categories. To ensure the target age range from birth to 6 years is properly represented for the outcome evaluation guideline, both the LittlEARS and PEACH Rating Scale were chosen to be included. The LittlEARS targets children from birth through the first two years of hearing and the PEACH items appear to target toddlers and older children. Therefore, it is possible that a guideline could provide a two-stage process whereby the LittlEARS is used with caregiver of young infants until they reach a ceiling score and/or age on the tool. This would indicate a certain level of auditory development has occurred within the infant and the child will be developmentally ready to be evaluated by the items in the PEACH Rating Scale.

Results

Outcome of interest was auditory development. In the included studies, auditory development was measured using four instruments that seem suitable for our population of interest: LittlEARS, PEACH and IT-MAIS. The results below are reported separately for each instrument.

1. LittlEARS

McCreery (2015) reported that the overall linear regression model was significant [R2 = 0.48, F(7,89) = 12.5, p<0.001], suggesting that this combination of factors predicted 48% of the variability in LittlEARS scores. Better hearing thresholds (β=−0.32, p=0.005), higher aided audibility (β=0.28, p=0.01), more hours of HA use (β=0.19, p=0.03), higher word recognition (β=0.37, p=0.004), and higher receptive language ability (β=0.29, p=0.02) were associated with higher LittlEARS scores. Age (β=0.10, p=0.34) and maternal education (β=0.17, p=0.22) were not significant predictors of LittlEARS scores after controlling for other variables.

Persson (2020) reported that CHL outcomes were within the normative range according to the instrument scale, except the scores of one participant. There were no significant differences in scores between the CHL and CNH groups at the ages of 10, 18, or 24 months; 10 months t(34) = 0.215 (p=0.83), 18 months t(31) = 1.207 (p=0.26), and 24 months t(36) = 0.783 (p=0.43). The correlations between hours of HA use (datalogging) and auditory development (LittlEARS) at 10 months were positive and weak (r = 0.32), at 18 months negative and weak (r = −0.21), and at 24 months positive and weak (r = 0.19). However, HA use at 10 months demonstrated positive moderate correlations to LittlEARS scores at 18 months (r = 0.48) and at 24 months (r = 0.58). Also, HA use at 18 months demonstrated a positive and moderate correlation to auditory development (LittlEARS) at 24 months, which was significant (r = 0.67, p = 0.05). Correlations between aided audibility (SII) and auditory development (LittlEARS) were positive and weak at 10 months (r = 0.36) and 24 months (r = 0.41), and positive and moderate at 18 months (r = 0.51). When analyzing SII to the LittlEARS at various ages to examine the cumulative effect, correlations were all positive but weak.

2. PEACH

McCreery (2015) reported that the average age at which the PEACH was administered was 21.8 months (SD=4.7 months). The correlation between PEACH and LittlEARS scores for children who had both measures at the 2-year visit was positive and significant (r=0.68, p<0.001), indicating that children with higher LittlEARS scores also had higher PEACH scores. The overall linear regression model was significant [R²=0.43, p<0.001], suggesting that this combination of factors predicted approximately 43% of the variability in Total PEACH score. Lower better-ear PTA (β=−0.48, p=0.02), higher aided audibility (β=0.58, p<0.001), and receptive language ability (β=0.49, p<0.001) were associated with higher Total PEACH scores. The relationship between Total PEACH score and average HA use (p=0.35), O&C word identification (p=0.20), and maternal education (p=0.85) was not significant after controlling for the other predictors.

Persson (2020) reported a significant difference in scores between the CHL and CNH groups on the Noise subscale at 30 and 36 months of age (see table 2).

Table 2. PEACH mean scores, ranges and standard deviations on all scales at all ages over time in children with normal hearing (CNH; n = 29 at 18 months and 24 months, n = 27 at 30 months, n = 28 at 36 months) and children with hearing loss (CHL; n = 9 at 18 months and 36 months, n = 8 at 24 and 30 months). Copied from Persson (2020).

Note. The differences between groups were significant on the noise scale at 30 and 36 months. PEACH = Parents Evaluation of Aural/Oral Performance in Children.

*CHL (M = 72.5, SD = 8.4) and CNH (M = 87.2, SD = 11.9); t(33) = −3.23, p = .03.

**CHL (M = 77.2, SD = 16.02) and CNH (M = 88.5, SD = 12.6); t(35) = −2.19, p = .35.

The correlations between HA use and functional auditory performance (PEACH) were negative and weak at 18 months (r = −.36), 24 months (r = −.20), 30 months (r = −.12), and 36 months (r = −.18). Correlations between HA use and PEACH at different ages were all negative and weak. Correlations between aided audibility (SII) and functional auditory performance (PEACH total scale) were positive and weak at 18 months (r = .41) and 24 months (r = .27), and negative and weak at 30 months (r = −.14) and 36 months (r = −.43) Correlations between SII at the various ages of 18, 24, and 30 months to PEACH total scale at 24, 30, and 36 months decreased in strength with age and were negative and weak. When comparing SII and the quiet and noise scale on the PEACH, the correlations were weak across all ages but were higher in the noise scale compared to the quiet and total scales. The highest correlation between SII and the PEACH noise scale was at 18 months (r = .69).

3. IT-MAIS

Yang et al. (2020) reported that there are no significant differences between groups in the age of assessment (Kruskal-Wallis test=2.078, p=0.354), ethnic proportion (Fisher’s exact test =4.221, p=0.293), and sex distribution (X2=0.903, p=0.637). Between the groups, no significant differences are observed in the 9-item total scores (median test=3.352, p=0.187; Kruskal-Wallis test=3.013, p=0.222) and scores of item 3–6 (which assess the ability to detect sound in daily lives) (median test=1.239, p=0.538; Kruskal-Wallis test=1.632, p=0.442). The scores of item 7–10 (which assess the capacity to discriminate and identify sounds), however, are significantly different among the three groups (median test=6.751, p=0.034). Pairwise comparison demonstrates the scores of item 7–10 in the UHL group (18.8% [6.3%, 43.8%]) are significantly lower than those in the BNH group (31.3% [6.3%, 70.3%], median test=6.617, p=0.03). The scores of item 2 (which evaluates the preverbal vocalization behaviors) are significantly different between UHL(0[0,1]) and BNH(1[0,3]) group (Kruskal-Wallis test=4.61, p<0.001), also between BMHL(0[0,1]) and BNH(1[0,3]) group (Kruskal-Wallis test=3.201, p=0.004).

Yang et al. adopted fitting curves, estimated by the 9-item IT-MAIS, to observe early auditory development trajectories in the BNH, UHL, and BMHL groups. Early auditory development develops in the same way in the three groups, which starts early after birth and increases in a logarithmic way. However, the developmental speed varies in three groups. Compared with UHL and BMHL groups, early auditory development with BNH grows more rapidly in the first 12 months after birth, and at the age of 5 months, the score reaches to 50%. After 20 months, early auditory development measured by IT-MAIS approaches to an asymptotic value, and at 23 months, it reaches to the ceiling. Early auditory development in the UHL and BMHL groups grow slower than that in the BNH group. It is estimated that scores reach to 50% at 6.5 and 8 months and reach a ceiling value at 35 and 40 months in the UHL and BMHL groups, respectively. The trajectory of UHL is between that of BNH and BMHL and grows nearer to BMHL.

Level of evidence of the literature

Grading of evidence was not possible due to the limited number of studies included and heterogeneity in study design and population. Furthermore, pooling of results was also not possible.

A systematic review of the literature was performed to answer the research question: what auditory development skills are at risk in children aged 0-4 years with established permanent hearing disorders?

PECO:

Indirectness

Since it was anticipated that some studies would (also) include children aged slightly older than 4 years that could add further information (more nuance) to the research question, while it was deemed unlikely by the working group that results would not be applicable for 0–4-year-old patients with hearing loss (this relates to indirectness, see Guyatt 2011), it was decided that studies where the population had a median age of around 5 years could also be added to the systematic review. Patients with cochlear implants were not included in the study population (unless otherwise noted).

Relevant outcome measures

The guideline development group considered auditory development as a critical outcome measure for decision making.

The working group did not define a minimal clinically important difference for outcome measures. Therefore, default thresholds were used: 0.5 SD for continuous outcomes and a 25% difference in relative risk (RR< 0.8 or RR>1.25) for dichotomous outcomes.

Since it was anticipated that included studies used different rating instruments to measure the same outcome, the mean differences from all included studies cannot be pooled. However, these mean differences can be divided by their respective standard deviations (SDs) to yield a statistic known as the standardized mean difference (SMD) which can be pooled. The interpretation of the magnitude (needed to define the minimal clinically important difference) of the SMD is given as small, SMD 0.2-0.5; medium, SMD 0.5-0.8; or large, SMD > 0.8. These SMDs are also provided as an indicator to define the minimal clinically important difference for continuous outcome measures.

Search and select (Methods)

The databases Medline (via OVID) and Embase (via Embase.com) were searched with relevant search terms until 31-07-2023. The detailed search strategy is depicted under the tab Methods. The systematic literature search resulted in 216 hits. Studies were selected based on the following criteria:

• Systematic review (detailed search strategy, risk of bias assessment, and results of individual comparative studies available), randomized control trial or observational comparative study;
• Research question includes all elements of the PECO;
• Full text available;
• Full text written in English or Dutch

52 studies were initially selected based on title and abstract screening. After reading the full text, 48 studies were excluded (see the table with reasons for exclusion under the tab Methods), and 4 studies were included.

Results

Four studies were included in the analysis of the literature. Important study characteristics and results are summarized in the evidence tables. The assessment of the risk of bias is summarized in the risk of bias tables.