Test-Retest Reliability and Variability Over Time for Repeated Audiometric Assessment in Individuals With Down Syndrome.

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Learn more: PMC Disclaimer | PMC Copyright Notice Am J Audiol . 2025 Sep 11;34(4):896–908. doi: 10.1044/2025_AJA-25-00068 Test–Retest Reliability and Variability Over Time for Repeated Audiometric Assessment in Individuals With Down Syndrome Heather L Porter a, ✉ , Emily LaSpada b , Emily Buss c , Lori J Leibold a Author information Article notes Copyright and License information a Center for Hearing Research, Boys Town National Research Hospital, Omaha, NE b University of Massachusetts, Amherst, MA c Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC Disclosure: The authors have declared that no competing financial or nonfinancial interests existed at the time of publication. ✉ Correspondence to Heather L. Porter: heather.porter@boystown.org Editor-in-Chief: Erin M. Picou Editor: Kelly N. Jahn ✉ Corresponding author. Received 2025 Mar 25; Revised 2025 May 16; Accepted 2025 Jun 17; Issue date 2025 Dec. Copyright © 2025 American Speech-Language-Hearing Association PMC Copyright notice PMCID: PMC12721705  PMID: 40934388 Abstract Purpose: High rates of hearing loss are observed for individuals with Down syndrome, and regular hearing assessment is recommended for children and adults. Unknown variability of threshold estimates for repeated auditory assessments for individuals with diverse intellectual abilities, such as individuals with Down syndrome, confounds the ability to determine true threshold changes between assessments. This study evaluated variability of pure-tone thresholds measured within and across test sessions for individuals with Down syndrome. Method: Participants included 27 individuals with Down syndrome, ages 5.1–46.5 years (average = 22.7, SD = 11.8). A group of 11 neurotypical individuals, ages 15.9–48.6 years (average = 24.5, SD = 10.3) was included in intrasession variability assessment. Pure-tone thresholds in quiet were measured at 1, 4, 8, and 11.2 kHz during a single session or during repeated audiograms separated by 2.7–11.2 months (average = 5.8, SD = 2.4) and 3.0–3.9 years (average = 3.4, SD = 0.4). Results: Intrasession variability was ≤ 10 dB for 94.3%–100% of thresholds for participants with Down syndrome, with similar variability observed for neurotypical participants. Threshold differences were ≤ 10 dB for 63.2%–85% of responses from audiograms separated by an average of 5.8 months and 37.5%–62.5% of responses separated by an average of 3.4 years. Conclusions: Intrasession variability for participants with Down syndrome aligns with current clinical standards accepting variability of ≤ 10 dB between repeated threshold estimates. Differences in repeated audiometric thresholds greater than 10 dB likely reflect true changes in hearing sensitivity. Supplemental Material: https://doi.org/10.23641/asha.30052885 Down syndrome occurs in approximately one in 700 newborns annually in the United States ( de Graaf et al., 2024 ) and is associated with multiple co-occurring conditions across the lifespan (for reviews, see Bull et al., 2022 ; Capone et al., 2017 ). Triplication of chromosome 21, the genetic basis of Down syndrome, impacts neurodevelopmental, musculoskeletal, craniofacial, and immune system function ( Antonarakis et al., 2021 ). It is the most common genetic cause of intellectual disability ( Ilyas et al., 2020 ), with most individuals with Down syndrome having mild-to-moderate intellectual impairment ( Stancliffe et al., 2012 ). As such, many individuals with Down syndrome can enjoy participation in life activities with minimal-to-moderate levels of support ( Boat & Wu, 2015 ). The majority of children with Down syndrome are included part- or full-time in general classroom settings ( Krell et al., 2021 ), and independence in adulthood is important to individuals with Down syndrome and their families ( Santoro et al., 2022 ). Hearing is a key component to successful participation in many learning, socializing, and working environments. The detrimental impact of insufficiently treated hearing loss has been well documented in the general population and includes consequences for language development and communication, socioemotional development and relationships, and educational and vocational opportunities (e.g., World Health Organization, 2025 ). The impact of hearing loss might be of even greater consequence for individuals with Down syndrome, who are already at risk for difficulties related to language and cognitive function. This is significant, as hearing loss is among the most prevalent of co-occurring conditions observed in individuals with Down syndrome across the lifespan, affecting approximately 40%–80% of individuals of all ages (e.g., De Schrijver et al., 2019 ; Kreicher et al., 2018 ; Malt et al., 2013 ; Startin et al., 2020 ). In the newborn period, hearing screening failure rates are higher for newborns with Down syndrome compared to newborns without Down syndrome, with high rates of hearing loss found for infants who fail the newborn hearing screening as well as those who pass ( Centers for Disease Control and Prevention, 2012 ; Park et al., 2012 ). A high prevalence of both permanent and transient hearing loss has been described for toddlers and young children with Down syndrome ( Nightengale et al., 2017 ). This trend is also observed for older children and young adults with Down syndrome ( De Schrijver et al., 2019 ), with some evidence that progressive high-frequency hearing loss typically associated with presbycusis may begin in late childhood or adolescence (e.g., Buchanan, 1990 ; Kreicher et al., 2018 ). Considering evidence of potentially progressive high-frequency hearing loss with increasing age, it is perhaps not surprising that the prevalence of hearing loss in adults with Down syndrome may reach complete penetrance by 60 years of age (for a review, see Capone et al., 2017 ). Although much of what is known about hearing in individuals with Down syndrome is based on retrospective medical record reviews or cross-sectional studies of patients receiving clinical care (e.g., De Schrijver et al., 2019 ; Kreicher et al., 2018 ; Nightengale et al., 2017 ) and may therefore be biased toward individuals seeking medical treatment, recent prospective studies reflect similar trends (e.g., Anshu et al., 2024 ; Porter et al., 2022 ). Despite some methodological limitations in previous research, it is broadly evident that hearing loss is common for individuals with Down syndrome, and the importance of regular hearing assessment for individuals with Down syndrome across the lifespan is reflected in recent health care guidelines for children ( Bull et al., 2022 ) and health care guideline development considerations for adults ( Capone et al., 2017 ). When evaluating results from repeated hearing assessments, current standards accept variability of ≤ 10 dB (American National Standards Institute [ANSI] S3.21-2004; ANSI, 2004 ). However, this standard is based on data from neurotypical adults ( Harris, 1946 ; Steinberg & Munson, 1936 ), and applicability to individuals with diverse intellectual abilities is unclear ( Lemkens et al., 2002 ). For example, early work by Harris (1946) found test–retest differences within 5 dB for threshold estimation for 512, 1024, and 2048 Hz pure-tone stimuli in adults, comparing results obtained by two groups of testers. Later work undertaken specifically to define the expected precision in serial audiometric measurements was undertaken by Atherley and Dingwall-Fordyce (1963) , who evaluated audiometric thresholds 0.5–8 kHz in adult medical students at four intervals separated by 24 hr to 1 week. That study found differences of up to 25 dB between consecutive threshold estimates, and the authors recommended allowing variability within a single ear of 7.5–17.5 dB, depending on frequency and the probability criterion adopted (i.e., p < .05 or p < .01). More recently, Schmuziger et al. (2004) compared intrasession test–retest repeatability in adult participants recruited from a public health fair, and they reported intrasession variability ≤ 10 dB for 97%–100% of participants for 0.5–12.5 kHz pure-tone stimuli and for 94%–99% of participants for 14–16 kHz pure-tone stimuli. Only one study to our knowledge has evaluated variability in repeated auditory threshold estimates in individuals with diverse intellectual abilities; McClannahan et al. (2021) reported similar test–retest reliability for listeners with mild dementia and listeners who are neurotypical. However, listeners with mild dementia inadequately represent the wide range of phenotypic characteristics observed for individuals with Down syndrome. As such, it is unclear whether the current standard accepting intrasession variability ≤ 10 dB between repeated audiometric threshold estimates adequately describes expected intrasession variability for individuals with Down syndrome. To summarize, the importance of regular hearing assessment for individuals with Down syndrome across the lifespan is supported by evidence of high rates of transient hearing loss, permanent hearing loss, and potentially progressive hearing loss starting in childhood (e.g., Buchanan, 1990 ; Kreicher et al., 2018 ; Nightengale et al., 2017 ). Uncertainty regarding expected intrasession response variability for repeated auditory assessments for individuals with Down syndrome confounds the ability to determine true threshold changes between repeated assessments over time. As such, the purpose of this study is to (a) evaluate reliability of pure-tone threshold estimates for a community-based sample of individuals with Down syndrome for repeated measurements obtained during a single test session and (b) to compare these data to previously obtained consecutive audiograms to describe variability in hearing thresholds over time. Materials and Method Procedure The institutional review board at Boys Town National Research Hospital approved this study (IRB 22-21-XP). Informed consent was obtained from all participants and their caregivers prior to participation. Participants were provided monetary compensation. This study was done as part of an ongoing study including multiple subexperiments being undertaken in our laboratory to understand hearing health care and outcomes for individuals with Down syndrome across the lifespan, Project INCLUDE. Inclusionary criteria are broad to capture a wide range of abilities; only individuals with cochlear implants are excluded. Participants are recruited through Down syndrome organizations, community events supporting individuals with Down syndrome and their families, and word of mouth. All test sessions for participants with Down syndrome and neurotypical child participants are guided by social stories and visual schedules (e.g., McTee et al., 2019 ), which were developed with assistance from our community advisory board composed of individuals in our local Down syndrome community. Available in digital and hardcopy formats, our social stories depict all study activities to help participants prepare for their visit and to reduce any potential fear or anxiety related to unknown environments. The pages of the social story walk participants through early research experiences (e.g., the building facade, entry into the research space, informed consent paperwork), research activities (e.g., otoscopy, middle ear assessment, hearing assessment), and visit completion (e.g., expression of gratitude for participation). Each page is formatted with a figure covering approximately 80% of the space and an accompanying short sentence describing the activity depicted in the figure in the remainder of the space (see Supplemental Material S1 ). Social stories are provided to families to preview before study visits and reviewed during the informed consent process. Presented to participants in hardcopy format at the start of the study visit, our visual schedules pictorially summarize the major activities described in the social stories and serve as an illustrated check list to help participants track their progress through the protocol (see Supplemental Material S2 ). Participants Participants with Down syndrome who completed all prior Project INCLUDE subexperiments were invited to participate in intrasession reliability assessment. None of the individuals with whom we had contact declined to participate in intrasession reliability assessment. Only data from participants seen for repeated audiometric assessment are included in analyses of across-session variability. Participants did not have to have both intrasession and repeated audiometric data to be included. Testing only participants who completed multiple test sessions could lead to bias in the sample, as participants who may have found previous hearing assessment difficult may not enroll in hearing-related research or continue with a research study that includes multiple hearing assessments. Participants included 27 individuals with Down syndrome, 18 male, ages 5.1–46.5 years (average = 22.7, SD = 11.8) at the time of most recent consent. A control group of 11 neurotypical individuals, four male, ages 15.9–48.6 years (average = 24.5, SD = 10.3) was recruited to assess methodological feasibility and obtain a limited subset of intrasession reliability data. Shown in Table 1 , participant demographics broadly represent the 2024 U.S. census estimates for Omaha, NE, in terms of race/ethnicity, with the exception of underrepresentation of Black/African American participants; participants also had higher reported household education levels than census estimates for our region ( U.S. Census Bureau, 2024 ). Information provided by caregivers about co-occurring health conditions for participants with Down syndrome is shown in Table 2 ; no neurotypical participants had intellectual or learning disabilities by self or caregiver report. Table 1. Participant age and participant- or participant caregiver–reported ethnicity, race, and highest household education. Variable Participants with Down syndrome Participants who are neurotypical Age in years Range 5.1–46.5 15.9–48.6 Average ( SD ) 22.7 (11.8) 24.5 (10.3) Race/ethnicity (%) White 92.3 81.8 Asian 3.8 9.1 Decline to provide or unknown race 3.8 9.1 Hispanic or Latino 14.8 9.1 Not Hispanic or Latino 85.2 54.5 Decline to provide or unknown ethnicity 0.0 36.4 Highest household education (%) Completed high school 0.0 0.0 Completed 1 or more years of university/college 3.7 0.0 Bachelor's degree 48.1 0.0 Completed technical/vocational school 3.7 0.0 Completed 1 or more years of graduate school 3.7 45.5 Master's degree 33.3 0 Doctorate degree 7.4 54.5 Decline to provide 0.0 0.0 Open in a new tab Table 2. Caregiver-reported conditions occurring for participants with Down syndrome. Condition n % Down syndrome 27 100.0 Language delay 15 55.6 Learning disability 12 44.4 Speech or language impairment 12 44.4 Allergies 10 37.0 Hearing loss 9 33.3 General developmental delay 7 25.9 Ear infections 7 25.9 Obsessive compulsive disorder 5 18.5 Ear drainage 4 14.8 Sinus infections 4 14.8 ADD/ADHD 3 11.1 Apraxia or oral motor planning problems 3 11.1 Cataracts 2 7.4 Sensory processing disorder 2 7.4 Stutter 2 7.4 Frequent colds 2 7.4 Articulation disorder 1 3.7 Autism spectrum disorder 1 3.7 Behavioral disorder 1 3.7 Asthma 1 3.7 Cerebral palsy 0 0.0 Color blindness 0 0.0 Cleft palate 0 0.0 Dyslexia 0 0.0 Dysarthria or trouble with oral motor skills 0 0.0 Epilepsy (seizures) 0 0.0 Migraines 0 0.0 Neurological disorder 0 0.0 Tonsilitis 0 0.0 Vestibular loss 0 0.0 Open in a new tab Note. ADD = attention-deficit disorder; ADHD = attention-deficit/hyperactivity disorder. Audiologic Assessments Auditory assessment was performed by an audiologist or audiology graduate student trainee using RadioEar DD450 circumaural headphones and a Madsen Astera 2 audiometer or a Maico MA42 audiometer. Assessment frequencies are specified in each section below. Conventional or conditioned play audiometry was used to assess hearing sensitivity bilaterally using a modified Hughson–Westlake two-down, one-up procedure with a 5-dB step size. The use of conventional or conditioned play audiometric methods was at the discretion of the tester based on participant ability and interest. If participants demonstrated unreliable responses during sessions, for example, due to possible fatigue, they were offered breaks and reinstruction; if this did not produce reliable responses, the session was discontinued. Test sessions could also be discontinued if needed based on session time constraints or participant preference. Middle ear assessment was performed using standard 0.226 kHz tympanometry with the Interacoustics Titan. Tympanogram classification was based on normative data in Margolis and Heller (1987) and Humes (2023) . Specifically, normal peak pressure was defined as −120 to 50 daPa, and normal compliance was defined as 0.21–2.05 ml. Normal equivalent ear canal volume was defined as 0.33–1.6 ml, unless a patent pressure equalization tube or tympanic membrane perforation was observed during otoscopy. Descriptive Assessments The Stanford–Binet Intelligence Scales–Fifth Edition (SB-5; Roid, 2003 ) was administered to participants with Down syndrome within 6 months of the initial audiometric assessment by a speech-language pathologist in designated research space at Boys Town National Research Hospital. The SB-5 nonverbal intelligence quotient standard scores are provided as a descriptive measure to broadly characterize cognitive ability in participants with Down syndrome, understanding the limitations in quantifying ability based on a standardized score of nonverbal intelligence. All participants or participant caregivers completed surveys providing demographic information including race, ethnicity, and highest household education. Intrasession Reliability Repeated audiologic assessment during a single test session occurred in a sound-treated booth in the Human Auditory Development Lab at Boys Town National Research Hospital or in the Boys Town Mini Research Vehicle (BT mini RV) during a single visit lasting approximately 1 hr. The BT mini RV is a light commercial van (i.e., 2017 Ford Transit 250 van) outfitted with individual seating, a central table, and standard electric outlets. Efforts to reduce ambient noise included use of generator power for equipment and cessation of climate control fans during audiometric assessment (ambient noise levels are described below). Assessment frequencies included 4, 8, and 11.2 kHz; 1 kHz was also assessed for individuals who had no measurable threshold at the limit of the audiometer at 11.2 kHz as no-response thresholds do not allow a precise evaluation of test–retest reliability. Given the focus on reliability in the present study, we opted to include this lower frequency with the rationale that it is likely to provide a threshold within the range of the audiometer. The choice of assessment frequencies was motivated by evidence of potentially progressive high-frequency hearing loss beginning in late childhood for individuals with Down syndrome and descriptions of increased variability for higher frequency pure-tones compared to lower frequency pure-tones in Schmuziger et al. (2004) , although this is not always observed (e.g., Frank, 1990 ). Thresholds were assessed in quasirandom order for all frequencies bilaterally in one run, with a short break provided before repeated bilateral threshold assessment in a second run. No intrasession reliability sessions were discontinued prior to obtaining all threshold estimates described by the protocol. Three measurements of ambient noise levels were obtained over an approximately 30-min period with a Larson Davis System 824 sound-level meter within the BT mini RV in a representative test setting (i.e., parked in a quiet area, engine running, climate control fans off). Ambient noise in the BT mini RV met ANSI S3.1-1999 Maximum Permissible Ambient Noise Levels for Audiometric Test Rooms, for supra-aural headphones at 4 kHz (BT mini RV = 29.7 dB, ANSI = 32.0 dB) and 8 kHz (BT mini RV = 32.5 dB, ANSI = 32.0 dB) but are 5.1 dB higher than the ANSI standard at 1 kHz (BT mini RV = 26.1 dB, ANSI = 21.0 dB). Note that ANSI S3.1-1999 is limited to frequencies ≤ 8 kHz and does not provide values for circumarual headphones, such as those used in this study, which provide additional ambient noise attenuation compared to supra-aural headphones ( ANSI, 1999 ). Ambient attenuation provided by Radio Ear DD 450 circumaural headphones is 29 dB at 1 kHz, 46 dB at 4 kHz, and 44 dB at 8 kHz; technical specifications are not available for higher frequencies. Previously Obtained Consecutive Audiograms Retrospective data included up to three previous audiograms obtained in a sound-treated booth within the Human Auditory Development Lab during 1- to 1.5-hr sessions. Frequencies assessed include octave frequencies of 0.25–8 kHz, and 11.2 and 16 kHz bilaterally, with the order of test frequencies arranged to obtain a range of low, mid, and high frequencies in case of early test termination due to fatigue or session time constraints. In cases of early test termination, thresholds were not obtained all test frequencies. In addition, thresholds were not assessed at 11.2 kHz during the first of the three visits to our lab, only the second and third visit. Bone-conduction testing was performed at octave frequencies of 0.5–4 kHz for individuals with air-conduction thresholds of ≥ 25 dB HL, with masked bone-conduction testing performed at a given frequency if air-conduction thresholds exceeded the unmasked bone-conduction threshold by 10 dB or more. Only air-conduction thresholds at 1, 4, 8, and 11.2 kHz are included in data analysis for the present study. Thresholds were entered as the audiometer output limit plus five for instances in which no response was provided at the maximum audiometer output limit. Across the three repeated retrospective audiograms, this occurred for only seven of 279 (2.5%) of thresholds. Data Analysis Pearson correlations were used to evaluate the consistency of pairs of threshold estimates. The analyses reported below include the left and the right ear for individual participants. Repeating the analyses for the left and the right ear separately leads to functionally equivalent results, with values differing by no more than r = .065 ( p s ≥ .09). Results Intrasession Reliability Repeated audiometric testing within a single test session was completed for 19 individuals with Down syndrome, 13 male, ages 5.5–46.5 years (average = 23.9, SD = 10.5). In this cohort, standard scores for the SB-5 ranged from 42 to 62 (average = 48.8, SD = 7.7), consistent with mild-to-moderate intellectual impairment. As described in Table 3 , hearing sensitivity for participants with Down syndrome ranged from normal (i.e., ≤ 20 dB HL) to severe-to-profound hearing loss (i.e., 71–≥ 90 dB HL) at 4, 8, and 11.2 kHz; thresholds at 1 kHz ranged from moderate-to-severe hearing loss (i.e., 50–85 dB HL). Thresholds for neurotypical participants were consistent with normal hearing sensitivity at 4 kHz, with hearing sensitivity at 8 and 11.2 kHz ranging from normal to profound hearing loss. Tympanometry was not collected for one participant with Down syndrome due to equipment battery failure. Of the 36 ears of participants with Down syndrome tested with tympanometry, results showed normal middle ear function for 18 ears, reduced compliance for 10 ears, large equivalent volume consistent with a pressure equalization tube or tympanic membrane perforation for four ears, and negative pressure for four ears. Tympanometry showed normal middle ear function for all neurotypical participants. Table 3. Audiometric thresholds in dB HL for the second of two threshold estimates obtained within a single test session for ears of participants with Down syndrome and neurotypical participants. Frequency Participants with Down syndrome Neurotypical participants N ears Range in dB HL (average; SD ) N ears Range in dB HL (average; SD ) 1 kHz 4 50–85 (65.0; 15.8) DNT DNT 4 kHz 36 0–105 (35.7; 26.3) 4 0–5 (5.0; 0) 8 kHz 35 0–95 (46.4; 26.6) 22 −5–95 (11.6; 21.9) 11.2 kHz 29 5–90 (47.2; 26.7) 21 −10–60 (7.9; 16.8) Open in a new tab Note. DNT = did not test. Intrasession reliability is illustrated in Figure 1 . The top panels show Run 2 thresholds as a function of Run 1 thresholds for participants with Down syndrome (blue circles) and neurotypical participants (white circles). Shaded areas indicate the 90th percentile (light gray) and 95th percentile (dark gray) of differences between repeated thresholds (i.e., Run 2 − Run 1) across all frequencies for participants with Down syndrome. Verified statistically via Pearson correlations, a strong positive association for repeated thresholds is apparent at all test frequencies for participants with Down syndrome ( r = .976–.996, p < .001) and neurotypical participants ( r = .921–.966, p < .001). Correlation coefficients for ears of individuals with Down syndrome were similar for those with normal middle ear function as indicated by tympanometry ( n = 17, r = .975–.988, p < .001) and those with abnormal tympanograms ( n = 18, r = .963–.990, p < .001). The bottom panels of Figure 1 show the distributions of threshold differences (Run 2 − Run 1) for participants with Down syndrome (blue bars) and neurotypical participants (white bars). Most Run 2 − Run 1 threshold differences in both participant groups were 0 to ±5 dB, and the data distribution is relatively symmetric. The maximum negative difference was −20 dB, occurring for one participant with Down syndrome at 8 kHz. The maximum positive difference was 20 dB, occurring for one participant with Down syndrome at 11.2 kHz and one neurotypical participant at 8 kHz. Figure 1. Open in a new tab Pure-tone thresholds in quiet for 1, 4, 8, and 11.2 kHz measured in two repeated runs within a single test session (top panels) and histograms of Run 2 − Run 1 threshold differences (bottom panels) for participants with Down syndrome (blue) and neurotypical participants (white). The 90th percentile (light gray) and 95th percentile (dark gray) ranges of threshold differences for participants with Down syndrome is shown (top panels only). DS = Down syndrome; NT = neurotypical; %ile = percentile. For participants with Down syndrome, intrasession variability across all test frequencies combined was ≤ 5 dB for 90% of thresholds and ≤ 10 dB for 95% of thresholds (see previously described gray-shaded regions, top panels of Figure 1 ). Intrasession reliability was similar for neurotypical participants, with 5% of thresholds for neurotypical participants falling outside of 95th percentile range constructed based on data for individuals with Down syndrome. Intrasession variability for each test frequency (i.e., 1, 4, 8, and 11.2 kHz) for participants with Down syndrome is shown in Table 4 . At 1 and 4 kHz, 100% of threshold differences were ≤ 10 dB; at 8 and 11.2 kHz, the proportion of threshold differences ≤ 10 dB was 94.3% and 96.6%, respectively. Similarly, threshold differences were ≤ 5 dB for 100% of responses at 1 kHz and 97.2% at 4 kHz; at 8 and 11.2 kHz, the proportion of threshold differences ≤ 5 dB was 85.7% and 89.7%, respectively. Table 4. Percentage and number of individual ears of participants with Down syndrome with threshold differences ≤ 5 and ≤ 10 dB for each test frequency for repeated audiometric thresholds obtained across two runs within a single test session (i.e., Intrasession), across sessions separated by an average of 5.8 months (i.e., Months separation), and across sessions separated by an average of 3.4 years (i.e., Years separation). Duration between sessions Threshold variability Frequency 1 kHz 4 kHz 8 kHz 11.2 kHz Intrasession ≤ 5 dB % 100 97.2 85.7 89.7 N ears 4/4 35/36 30/35 26/29 ≤ 10 dB % 100 100 94.3 96.6 N ears 4/4 36/36 33/35 28/29 Months separation ≤ 5 dB % 85.0 47.4 44.4 58.8 N ears 17/20 9/19 16/36 10/17 ≤ 10 dB % 85.0 63.2 70.6 68.8 N ears 17/20 12/19 26/36 12/29 Years separation ≤ 5 dB % 50 31.3 31.3 DNT N ears 8/16 5/16 5/16 DNT ≤ 10 dB % 62.5 37.5 37.5 DNT N ears 10/16 6/16 6/16 DNT Open in a new tab Note. DNT = did not test. Previously Obtained Consecutive Audiograms Project INCLUDE at Boys Town National Research Hospital first launched in 2019 and has included multiple subexperiments since that time. The general study design has participants return to the lab for data collection on different subexperiments as they are available, with audiometric data obtained at study entry and recollected whenever the previous audiogram is more than 6 months old. However, data collection paused during the COVID-19 pandemic, resulting in prolonged durations between audiograms. To date, individual participants have completed up to three audiometric assessments, which can be classified into two time frames: audiograms separated by months or by years. Described further below, the three repeated audiometric assessments are labeled Audio A, Audio B, and Audio C. Separated by months, Audio A and Audio B are the most recent audiograms obtained in our lab. Obtained prior to the COVID-19 pandemic, Audio C is the oldest audiogram obtained in our lab, separated from Audio B by years. Repeated Audiograms Separated by Months Two repeated audiograms were obtained in sessions separated in duration by 2.7–11.2 months (average = 5.8, SD = 2.4) for 19 participants with Down syndrome, 15 male. Participant ages ranged from 7.7 to 45.7 years (average = 23.0, SD = 11.4) at the time of the most recent audiogram. In this cohort, standard scores for the SB-5 were 42–66 (average = 49.3, SD = 8.2), consistent with mild-to-moderate intellectual impairment. Figure 2 plots audiometric threshold (dB HL) for participants with Down syndrome (filled gray circles) for Audio A, the most recent audiogram, with box plots representing the 25th–75th percentile and mean data. Audiometer output limits are shown as a filled gray region at the bottom of the panel. As shown in Table 5 , ears compared across Audio A and Audio B had extensive heterogeneity in threshold estimates across participants. Across frequency, threshold estimates for both Audio A and Audio B ranged from normal hearing sensitivity to profound hearing loss. Figure 2. Open in a new tab Audiometric threshold (dB HL) for participants with Down syndrome (filled gray circles) for Audio A, the most recent audiogram, with box plots representing the 25th–75th percentile and mean data. Audiometer output limits are shown as a filled gray region at the bottom of the panel. Table 5. Audiometric thresholds in dB HL for threshold estimates obtained for ears of participants with Down syndrome compared across two sessions, Audio A and Audio B, separated by 2.7–11.2 months (average = 5.8; SD = 2.4). Frequency N ears Range in dB HL (average; SD ) Audio A 1 kHz 20 15–75 (36.8; 18.7) 4 kHz 19 0–85 (36.3; 26.0) 8 kHz 34 0–95 (35.1; 24.5) 11.2 kHz 17 −5 to 95 (50.0; 32.1) Audio B 1 kHz 20 10–80 (39.5; 21.4) 4 kHz 19 5–80 (44.2; 21.7) 8 kHz 34 10–90 (41.9; 23.1) 11.2 kHz 17 10–95 (57.1; 29.8) Open in a new tab Tympanometry for the most recent assessment showed healthy middle ear function for 24 ears, reduced compliance for three ears, large equivalent volume consistent with a pressure equalization tube or tympanic membrane perforation for seven ears, and negative pressure for four ears. Middle ear status as described by tympanometry was consistent between visits for 76.3% of ears ( n = 29). As shown in the top panels of Figure 3 , there is a strong positive association between repeated thresholds for 1, 4, 8, and 11.2 kHz stimuli ( r = .836–.935, p < .001); however, qualitatively fewer threshold differences were within the 95th percentile (i.e., ≤ 5 dB) and 90th percentile (i.e., ≤ 10 dB) than observed for participants with Down syndrome during intrasession reliability assessment (see Table 4 ). At 1 kHz, 85% of between-sessions threshold differences were ≤ 5 dB, and 85% were ≤ 10 dB. The proportion of differences within these ranges was less at higher frequencies. Specifically, at 4 kHz, 47.4% of differences were ≤ 5 dB and 63.2% were ≤ 10 dB; at 8 kHz, 44.4% were ≤ 5 dB and 70.6% were ≤ 10 dB; at 11.2 kHz, 58.8% were ≤ 5 dB and 68.8% were ≤ 10 dB. As shown in the histograms of between-session threshold differences in the bottom panels of Figure 3 , threshold differences ranged from −20 dB, for one participant at 1 kHz, to 40 dB for two participants, one at 1 kHz and one at 8 kHz. The average absolute value of between-sessions threshold differences across the frequencies ranged from 0 to 30 dB (average = 9.4, SD = 6.6) for individuals with consistent middle ear status between visits ( n = 29) and 1.7–30 dB (average = 12.9, SD = 12.1) for individuals with differing middle ear status between visits ( n = 9). Figure 3. Open in a new tab Thresholds in quiet for 1, 4, 8, and 11.2 kHz pure-tones obtained in two visits separated by an average of 5.8 months with symbol shading representing months between sessions, as described in the legend (top panels) and histograms of Audio A − Audio B threshold differences (bottom panels). The 90th percentile (light gray) and 95th percentile (dark gray) ranges of threshold differences obtained from repeated audiograms in a single session for participants with Down syndrome is shown for comparison (top panels only). DS = Down syndrome; %ile = percentile. Repeated Audiograms Separated by Years Two repeated audiograms were obtained in sessions separated in duration by 3.0–3.9 years (average = 3.4, SD = 0.4) for nine participants with Down syndrome, five male. Participant ages ranged from 8.7 to 20.4 years (average = 15.2, SD = 4.4) at the time of the most recent audiogram. In this cohort, standard scores for the SB-5 ranged from 43 to 60 (average = 48.4, SD = 5.9), consistent with mild-to-moderate intellectual impairment. As shown in Table 6 , threshold estimates for ears compared across Audio B and Audio C had extensive heterogeneity. Across frequency, threshold estimates for Audio B and Audio C ranged from normal hearing sensitivity to profound hearing loss. Table 6. Audiometric thresholds in dB HL for threshold estimates obtained for ears of participants with Down syndrome across two sessions, Audio B and Audio C sessions; Audio C was obtained prior to the COVID-19 pandemic, 3.0–3.9 years (average = 3.4; SD = 0.4) before Audio B. Frequency N ears Range in dB HL (average; SD ) Audio B 1 kHz 16 10–80 (23.1; 18.5) 4 kHz 16 0–65 (24.7; 17.9) 8 kHz 16 15–85 (34.7; 18.0) Audio C 1 kHz 16 5–65 (24.4; 18.7) 4 kHz 16 0–75 (22.2; 22.0) 8 kHz 16 0–105 (34.4; 32.8) Open in a new tab Tympanometry for the most recent assessment showed healthy middle ear function for 14 ears, reduced compliance for two ears, and large equivalent volume consistent with a pressure equalization tube or tympanic membrane perforation for two ears. No ears showed evidence of negative pressure in the middle ear space. Repeated tympanograms for two participants were unavailable for comparison due to equipment failure during one test session for one participant and test refusal during one test session for one participant. Of the remaining 14 ears with repeated tympanometry results, middle ear status as described by tympanometry was consistent between visits for 78.6% of ears ( n = 11). Shown in the top panels of Figure 4 , there is a positive association for thresholds obtained in two visits separated by an average of 3.4 years for 4-kHz pure-tones ( r = .581, p ≤ .001), but no association between thresholds for 1- or 8-kHz pure-tones ( r = .107–.329, p > .066). As shown in Table 4 , at 4 and 8 kHz, less than half of the between-sessions threshold differences were within the 95th percentile (i.e., ≤ 5 dB) and 90th percentile (i.e., ≤ 10 dB) ranges observed for participants with Down syndrome during intrasession reliability assessment. At 4 kHz, 31.3% of differences were ≤ 5 dB and 37.5% were ≤ 10 dB. Similarly, at 8 kHz, 31.3% of differences were ≤ 5 dB and 37.5% were ≤ 10 dB. At 1 kHz, 50.0% and 62.5% of differences were ≤ 5 and ≤ 10 dB, respectively. As shown in the histograms of between-sessions threshold differences in the bottom panels of Figure 4 , differences between thresholds obtained in different test sessions ranged from −50 dB for one participant at 1 kHz to 60 dB for one participant at 8 kHz. For individuals with repeated tympanometry results, the average absolute value of between-sessions threshold differences across frequency ranged from 3.3 to 41.7 dB (average = 20.0, SD = 13.8) for individuals with consistent middle ear status between visits ( n = 11) and 5.0–15.0 dB (average = 8.9, SD = 5.4) for individuals with differing middle ear status between visits ( n = 3). Figure 4. Open in a new tab Thresholds in quiet for 1, 4, and 8 kHz pure-tones obtained in two visits separated by an average of 3.4 years with symbol shading representing years between sessions, as described in the legend (top panels), and histograms of Audio B − Audio C threshold differences (bottom panels). The 90th percentile (light gray) and 95th percentile (dark gray) ranges of threshold differences obtained from repeated audiograms in a single session for participants with Down syndrome is shown for comparison (top panels only). DS = Down syndrome; %ile = percentile. Discussion Given evidence of high rates of permanent hearing loss, transient hearing loss, and potentially progressive hearing loss starting in childhood (e.g., Buchanan, 1990 ; Kreicher et al., 2018 ; Nightengale et al., 2017 ), pediatric and adult health care recommendations for individuals with Down syndrome advise repeated audiometric evaluation throughout the lifespan (e.g., Bull et al., 2022 ; Capone et al., 2017 ). Current standards for test–retest threshold reliability are based on studies of individuals without described intellectual impairment, prompting uncertainty regarding expected reliability of threshold estimation for repeated auditory assessments for individuals with diverse intellectual abilities. Uncertainty regarding expected response reliability for repeated auditory threshold assessments confounds the ability to determine true changes in threshold. As such, this study was undertaken to describe variability in pure-tone threshold measurements for a community-based sample of participants with Down syndrome for repeated measurements obtained during a single test session as part of a larger effort to improve hearing health care and outcomes for individuals with Down syndrome across the lifespan. These data were then compared to previously obtained consecutive audiograms obtained over a period of > 3 years from a subset of the same cohort of participants with Down syndrome. Across frequencies, intrasession reliability for most (94%–100%) participants with Down syndrome met the current clinical standard of acceptable threshold variability of ≤ 10 dB for repeated auditory assessment (ANSI S3.21-2004). In addition, intrasession reliability for participants with Down syndrome and neurotypical participants was similar, with between-run threshold differences ≤ 20 dB observed for both groups. Data from the current study broadly replicate historic work evaluating intrasession reliability of auditory sensitivity measurements in individuals without described intellectual disability (e.g., Atherley & Dngwall-Fordyce, 1963 ; Harris, 1946 ), despite some methodological differences. For example, Atherley and Dingwall-Fordyce (1963) compared audiometric assessments for medical students tested at intervals separated by 24 hr to 1 week and reported differences in successive threshold measurements of up to 25 dB. Results from the present study are also consistent with the ≤ 5 dB variability described in Harris (1946) , who compared audiometric thresholds in adults obtained successively by two technicians who varied in amount of training in audiometric assessment methodology. Like Schmuziger et al. (2004) , intrasession variability in the present study tended to be higher for higher frequencies compared to lower frequencies. That study reported intrasession variability ≤ 10 dB for 97%–100% of participants for tones up to 12.5 kHz and for 94%–99% of participants from 14 to 16 kHz. In the present study, data from participants with Down syndrome suggest similar intrasession variability, with 100% of response differences ≤ 10 dB for 1 and 4 kHz, and 94.3% and 96.6% at 8 and 11.2 kHz, respectively. Counter to historic concern for a potential relationship between intellectual ability and response variability (e.g., Lemkens et al., 2002 ), results from this study show intrasession variability within currently accepted clinical standards for repeated auditory assessment for individuals with Down syndrome despite having mild-to-moderate intellectual disability. Between-session threshold differences increased as time between sessions increased for previously obtained audiograms, which likely reflects true changes in audiometric thresholds. Across frequencies, 15%–36% of threshold differences for audiograms repeated within 11.2 months were greater than 10 dB, the range of the 90th percentile range defined during intrasession reliability assessment in the present study and the current clinical standard. Of the 19 participants with repeated audiograms separated by months, 10 had changes greater than 10 dB in one or both ears at one or more frequencies. However, changes in hearing greater than 10 dB were observed for some participants within a shorter time frame. Specifically, six of 12 participants who received repeated audiometric evaluation within 6 months and 4/5 participants who received repeated audiometric evaluation within 3 months had threshold changes greater than 10 dB in one or both ears at one or more frequencies. Although from a limited data set, this provides further evidence supporting the need for frequent audiometric assessment for individuals with Down syndrome. Additional longitudinal work is needed to begin to describe changes in hearing over time considering specific variables, such as patient age and test frequency. Not surprisingly, the proportion of threshold differences greater than 10 dB was larger for audiograms separated by years compared to those separated by months, occurring for 37.5%–62.5% of threshold differences across frequencies. Whereas the maximum difference between runs was ±20 dB for repeated audiograms in a single session, that range increased to −20 to 40 dB for audiograms obtained an average of 5.8 months apart and −50 to 60 dB for audiograms obtained an average of 3.4 years apart. Based on intrasession variability findings, these between-sessions threshold differences greater than 10 dB should be considered as true changes in audiometric threshold. Interestingly, differences in hearing thresholds between sessions do not seem to be closely associated with tympanometric classification. The average (absolute value) of threshold differences for audiograms separated by an average of 5.8 months was similarly broad whether participants had the same tympanometric classification across sessions (range: 0.0–30.0 dB, average = 9.4, SD = 6.6) or different tympanometric classification across sessions (range: 1.7–30.0 dB, average = 12.9, SD = 12.1). Likewise, the average (absolute value) of threshold differences for audiograms separated by an average of 3.4 years was broad whether participants had the same tympanometric classification across sessions (range: 3.3–41.7 dB, average = 20.0, SD = 13.8) or different tympanometric classification across sessions (range: 5.0–15.0, average = 8.9, SD = 5.4). Standard 226-Hz tympanometry has limited ability to predict the impact of middle ear status on hearing sensitivity in individuals who are neurotypical (e.g., Keefe & Simmons, 2003 ; Prieve et al., 2013 ), and failure to find an association between tympanometry and thresholds for individuals with Down syndrome does not rule out an effect of middle ear status. In individuals who are neurotypical, wideband acoustic immittance may better reflect middle ear status and any related impact on hearing sensitivity compared to tympanometry (e.g., Keefe & Simmons, 2003 ; Merchant et al., 2021 ; Prieve et al., 2013 ). However, with the exception of one study evaluating wideband acoustic immittance in children with Down syndrome (i.e., Hunter et al., 2017 ), this is a relatively underexplored area in individuals with Down syndrome across the lifespan that we are currently working to address. Limitations This study is the first to evaluate intrasession reliability for repeated audiometric assessment in individuals with Down syndrome, but it is not without limitations. The small sample size may not adequately capture variability for a larger group, especially intrasession reliability data for 1-kHz pure-tones. Greater intrasession variability may be expected for lower frequency pure-tones due to the effects of self-generated noise (e.g., Buss et al., 2016 ), which may be elevated due to the occlusion effect (e.g., Porter et al., 2020 ) and exacerbated for individuals with Down syndrome considering the high prevalence of narrow and small ear canals observed in this population (e.g., Dy & Lapeña, 2018 ; Saliba et al., 2014 ; Schwartz & Schwartz, 1978 ). Work is planned to evaluate intrasession reliability across a broader frequency range in a larger cohort of participants. In addition, although participants with Down syndrome in the current study were diverse with respect to hearing abilities, having hearing sensitivity ranging from normal to severe-to-profound hearing loss across test frequencies, all participants with Down syndrome in this study had mild-to-moderate intellectual impairment as described by SB-5 nonverbal IQ standard scores. This finding is consistent with findings from Stancliffe et al. (2012) , who surveyed a large sample of adults with Down syndrome from across the United States ( n = 1,199) and reported most (68%) had mild or moderate intellectual ability; however, it is possible that intrasession reliability might differ for those listeners with more severe intellectual disability. In our sample, no association was observed for average intrasession or across-session threshold differences and SB-5 nonverbal IQ raw scores ( r = −.216–.227, p s ≥ .196) or standard scores ( r = −.143 to .193, p s ≥ .420). Although this could be related to a lack of association between nonverbal intelligence quotient and test–retest reliability for audiometric assessment, it could also be due to the small n in this sample. Conclusions Intrasession reliability for pure-tone threshold assessment in quiet was within 10 dB for 94.3%–100% for 1, 4, 8, and 11.2 kHz stimuli for participants with Down syndrome across a wide range of ages. This result is aligned with the current clinical standards, which allow variability of up to 10 dB between repeated audiologic assessments before diagnosing a change in hearing. A greater proportion of between-sessions threshold differences fell outside of the 90th percentile range for intrasession reliability (i.e., ≤ 10 dB) for repeated assessments separated by months or years. Differences in repeated audiometric thresholds > 10 dB likely reflect true changes in hearing sensitivity. Data Availability Statement All of the data used in preparation for this article are presented in graphical or tabular format herein. Supplementary Material Supplemental Material S1. A social story depicting all study activities was used to help participants prepare for their visit and familiarize them with study procedures. Each page of the social story includes a figure with a short sentence describing the activity being shown, in this example otoscopy. AJA-34-896-s001.pdf (558KB, pdf) Supplemental Material S2. A sample visual schedule depicting major study activities. Visual schedules serve as an illustrated checklist and were used to help guide study participation. AJA-34-896-s002.pdf (254.8KB, pdf) Acknowledgments This study was funded by National Institute on Deafness and Other Communication Disorders Grants T35DC008757-17 (awarded to K. Janky), R21DC020024 (awarded to H. Porter), and R01DC020229 (awarded to L. Leibold), and the National Institute of General Medical Sciences Grant P20GM109023 (awarded to L. Leibold). We are grateful for the ongoing support provided by the participants and their families and the members of the Project INCLUDE team at Boys Town National Research Hospital. Funding Statement This study was funded by National Institute on Deafness and Other Communication Disorders Grants T35DC008757-17 (awarded to K. Janky), R21DC020024 (awarded to H. Porter), and R01DC020229 (awarded to L. Leibold), and the National Institute of General Medical Sciences Grant P20GM109023 (awarded to L. Leibold). References American National Standards Institute. (1999). Maximum permissible ambient noise levels for audiometric test rooms (ANSI/ASA S3.1-1999). [ Google Scholar ] American National Standards Institute. (2004). Methods manual pure-tone threshold audiometry (ANSI/ASA S3.21-2004). Anshu, K., Kristensen, K., Godar, S. P., Zhou, X., Hartley, S. L., & Litovsky, R. Y. (2024). 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A social story depicting all study activities was used to help participants prepare for their visit and familiarize them with study procedures. Each page of the social story includes a figure with a short sentence describing the activity being shown, in this example otoscopy. AJA-34-896-s001.pdf (558KB, pdf) Supplemental Material S2. A sample visual schedule depicting major study activities. Visual schedules serve as an illustrated checklist and were used to help guide study participation. AJA-34-896-s002.pdf (254.8KB, pdf) Data Availability Statement All of the data used in preparation for this article are presented in graphical or tabular format herein. 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