Examining Barriers to Early Hearing Diagnosis.

PURPOSE: Many factors create barriers for early hearing detection and intervention (EHDI), especially those related to unfavorable social determinants of health (SDOH). The primary aim of this study was to evaluate diagnostic timing of infants at risk for congenital hearing loss in consideration of known barriers. Understanding the specific barriers to early diagnosis can inform interventions to improve timeliness of diagnosis and subsequent habilitation. METHOD: A retrospective chart review was completed for infants referred for diagnostic audiologic testing at a tertiary urban-setting Children's Hospital from 2018 to 2021. After exclusion criteria were applied, 1,488 infants were included in the analysis. Various factors were recorded from electronic medical records including those specific to SDOH. Time to diagnosis was derived and compared across five factors of interest that have previously been shown to impact diagnostic timeline, including (a) insurance type, (b) race/ethnicity, (c) presence of middle ear dysfunction at first auditory brainstem response (ABR), (d) proximity to diagnostic center, and (e) diagnostic timing before and during/after the COVID-19 pandemic. RESULTS: Across the study time period, 77% of infants referred for diagnostic testing had confirmed diagnosis by the EHDI benchmark of 3 months. Analysis of time to diagnosis across factors of interest revealed no clinically significant differences for insurance type, race/ethnicity, proximity to diagnostic center, or timing in reference to the COVID-19 pandemic. Presence of middle ear dysfunction on first ABR was found to significantly protract final diagnostic timing. CONCLUSIONS: Although some known barriers for EHDI can be universal, other factors may have a differential impact on an infant's timeline to diagnosis based on their specific location, which can interact differently with additional known barriers. Understanding local challenges will serve to better guide programs in implementing facilitators that will address their specific needs for improved outcomes.

Design of aided augmentative and alternative communication systems for children with vision impairment: psychoacoustic perspectives.

Children with complex communication needs often have multiple disabilities including visual impairments that impact their ability to interact with aided augmentative and alternative communication (AAC) systems. Just as the field benefited from a consideration of visual cognitive neuroscience in construction of visual displays, an exploration of psychoacoustics can potentially assist in maximizing the possibilities within AAC systems when the visual channel is either (a) not the primary sensory mode, or (b) is one that can be augmented to ultimately benefit AAC outcomes. The purpose of this paper is to highlight background information about psychoacoustics and present possible future directions for the design of aided AAC system technologies for children with visual impairments who rely on auditory information to learn and utilize AAC.

Barriers to and Facilitators of Early Hearing Detection and Intervention in the United States: A Systematic Review.

OBJECTIVES: Early hearing detection and intervention (EHDI) is guided by the 1-3-6 approach: screening by one month, diagnosis by 3 mo, and early intervention (EI) enrollment by 6 mo. Although screening rates remain high, successful diagnosis and EI-enrollment lag in comparison. The aim of this systematic review is to critically examine and synthesize the barriers to and facilitators of EHDI that exist for families, as they navigate the journey of congenital hearing loss diagnosis and management in the United States. Understanding barriers across each and all stages is necessary for EHDI stakeholders to develop and test novel approaches which will effectively reduce barriers to early hearing healthcare. DESIGN: A systematic literature search was completed in May and August 2021 for empirical articles focusing on screening, diagnosis, and EI of children with hearing loss. Two independent reviewers completed title and abstract screening, full-text review, data extraction, and quality assessments with a third independent reviewer establishing consensus at each stage. Data synthesis was completed using the Framework Analysis approach to categorize articles into EHDI journey timepoints and individual/family-level factors versus system-level factors. RESULTS: Sixty-two studies were included in the narrative synthesis. Results revealed that both individual/family-level (e.g., economic stability, medical status of the infant including middle ear involvement) and system-level barriers (e.g., system-service capacity, provider knowledge, and program quality) hinder timely diagnosis and EI for congenital hearing loss. Specific social determinants of health were noted as barriers to effective EHDI; however, system-level facilitators such as care coordination, colocation of services, and family support programs have been shown to mitigate the negative impact of those sociodemographic factors. CONCLUSIONS: Many barriers exist for families to obtain appropriate and timely EHDI for their children, but system-level changes could facilitate the process and contribute to long-term outcomes improvement. Limitations of this study include limited generalizability due to the heterogeneity of EHDI programs and an inability to ascertain factor interactions.

Clinical Practice Patterns of Fitting Advanced Device Features in Children With Cochlear Implants.

PURPOSE: The purpose of this study was to identify common clinical practice patterns for providing advanced noise management features in children with cochlear implants (CIs) and evaluate trends in consideration of clinician experience and comfort with CI manufacturer-specific technology. METHOD: A mixed-model survey including quantitative and qualitative questions regarding providing advanced noise management features in the pediatric CI population was collected electronically via research electronic data capture. Survey questions spanned approach/philosophy toward provision of features, age of provision, and demographics of respondents. Descriptive statistics were completed to define common clinical practice patterns and demographic information. RESULTS: A total of 160 pediatric audiologists from 35 U.S. States and five Canadian provinces completed the survey. Most audiologists (73.8%) reported enabling automatic directional microphones, and a vast majority (91%) reported enabling advanced noise processing features such as automatic noise cancellers, wind noise cancellers, and impulse noise cancellers in recipients' main programs. Audiologists ranked features in terms of importance for a school-age child with the top three ranked as automatic noise reduction, automatic directional microphones, and concha-level microphones. Importance of child-specific factors varied depending upon the specific feature of interest. CONCLUSIONS: Variability exists among providers in enabling advanced noise management features for pediatric CI recipients. Multiple factors, including patient characteristics, provider characteristics, and limited evidence-based guidance, could account for much of the variation. Overall, there is a trend toward automaticity for noise management. Additional studies are warranted to provide the evidence base for confidently programming advanced features for children using CIs.

Assessing Adherence to Audiologic Parameters of Care for Children With Cleft Palate: A Quality Improvement Initiative.

OBJECTIVE: To evaluate and increase adherence to an evidence-based audiologic management protocol for children with cleft palate. DESIGN: Prospective, multidisciplinary quality improvement initiative. SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: Children with cleft palate (with or without cleft lip) between the ages of 0 and 5 years (n = 205). INTERVENTIONS: A multidisciplinary team identified key drivers for nonadherence to recommended audiological follow-up and implemented interventions to improve adherence. Key drivers included provider practices and preferences, clinic logistics and flow, and patient/family awareness and education. Several interventions were implemented between 2016 and 2020, including developing an evidence-based audiologic protocol, maximizing access to audiologic clinic visits across multiple departments, cleft team education, and improved team communication. MAIN OUTCOME MEASURE(S): Completion of recommended audiologic assessment at 5 separate care milestones. RESULTS: After implementation of interventions between 2016 and 2020, adherence to recommended audiologic follow-up increased from 59% to 84%. Analysis of individual care milestones revealed that increased access to audiologic testing during team clinics resulted in the largest increase in adherence to recommended follow-up. Additionally, cause-effect analysis revealed that nonadherence due to provider-related causes decreased over the project period to a greater extent than patient/family-related causes. CONCLUSIONS: Implementation of an evidence-based audiologic care protocol and improvements in access to early hearing care are feasible in a high-volume multidisciplinary cleft clinic. Adherence to recommended audiologic management can be improved by establishing strategies to improve access to care, team member and family education, and enhanced team communication.

Hearing Status of Children and Adolescents With Familial Adenomatous Polyposis.

BACKGROUND/AIM: Chemopreventative therapeutics may be helpful in familial adenomatous polyposis (FAP) management; however, prospective chemopreventative studies are complicated by potential ototoxicity and pre-existing hearing loss. The aim of this study was to establish and compare baseline hearing status of children and adolescents with FAP and their unaffected siblings. PATIENTS AND METHODS: Twenty FAP pediatric patients with documented mutation of the adenomatous polyposis coli (APC) gene and nine unaffected sibling controls underwent baseline hearing evaluation, including audiometry, speech perception testing, and middle and inner ear physiologic measures. Results of the FAP cohort were compared to the unaffected sibling cohort. RESULTS: Two (5%) children with FAP presented with baseline hearing loss of unknown etiology, likely unrelated to their FAP diagnosis. No significant differences were found in any of the hearing measures between groups. CONCLUSION: Mutation of the APC gene is not necessarily indicative of higher risk for baseline hearing loss in the pediatric population.

Therapeutic challenges and clinical characteristics of single-sided deafness in children.

INTRODUCTION: The clinical implications of single-sided deafness (SSD) in children has historically been underappreciated by patients and providers alike, despite a large body of literature on the wide-ranging neurocognitive, language, scholastic, and functional impairments that occur. Conventional amplification options are marked by variable results and frequent loss of follow-up. METHODS: Retrospective case series for pediatric SSD from 2008 to 2018. RESULTS: 88 children with congenital SSD were identified. Seventeen (N = 17/88, 23.9%) passed their newborn hearing screen. Median age at first otolaryngology evaluation was 0.65 years (range 0.1-16.9 years). Most common etiologies included cochlear nerve deficiency (N = 39, CND, 44.3%), unknown (N = 30, 35.2%), inner ear malformation (N = 7, 8.0%), and congenital cytomegalovirus (N = 6, 6.8%). 32.5% of patients elected for continued observation only, followed by bone conduction hearing aid (27.7%), contralateral routing of sound aid (20.5%), conventional hearing aid (13.3%), or cochlear implant (6%). Lack of follow-up at ≥1 year was common (39.8%). Of those with device use data (N = 39), 84.7% reported either discontinued or <6 h of daily use. CONCLUSIONS: Despite early diagnosis and evaluation, the pediatric SSD cohort is characterized by high rates of loss of follow-up and amplification discontinuation. Cochlear nerve deficiency is commonly seen in congenital SSD. Early specialist referral is critical for habilitation evaluation. Patients and caregivers should be educated on the significant implications of unilateral hearing loss.

Audiometric findings in children with unilateral enlarged vestibular aqueduct.

OBJECTIVE: To evaluate the prevalence of bilateral hearing loss in children with unilateral enlarged vestibular aqueduct (EVA) at a single institution. METHODS: A retrospective case review was performed at a tertiary care pediatric referral center involving children with radiologic findings of unilateral EVA and normal labyrinthine anatomy of the contralateral ear diagnosed via CT and/or MRI. The main outcome measure of interest is the number of patients with unilateral EVA who were diagnosed with bilateral hearing loss. RESULTS: Sixty-one pediatric patients were identified. The mean audiometric follow-up was 48.2 months (0-150). Three (4.9%) patients with unilateral EVA were noted to have bilateral hearing loss, and this rate was not significantly different (p = 1.0) from the rate reported in a comparison group of patients with contralateral hearing loss (6.0%) without an EVA. The pure-tone average (defined as the average dB HL at 500, 1000, 2000, and 4000 Hz) in the group with bilateral hearing loss was 31.3 dB HL in the better hearing ear and 79.6 dB HL in the worse hearing ear, with the difference being statistically significant (p = 0.02). In the unilateral EVA patients without contralateral hearing loss (n = 56, 91.8%), the PTA was 9.4 dB HL in the better hearing ear and 51.9 dB HL in the worse hearing ear, with the difference being statistically significant (p < 0.001). Two patients (3.3%) with unilateral EVA were found to have hearing within normal limits bilaterally. The EVA was ipsilateral to the worse hearing ear in all cases. CONCLUSION: The prevalence of bilateral hearing loss in children with unilateral EVA appears to be low. Specifically, it may be no different than the rate of contralateral hearing loss in children with unilateral hearing loss without an EVA. The present report is somewhat different than the previously described prevalence in the literature. This difference could be related to the imaging type and diagnostic criteria used, the patients included, the source of the identified patents, and the overall population of patients studied.

Early Hearing Detection and Intervention: Timely Diagnosis, Timely Management.

OBJECTIVE: A quality improvement study was completed to assess the impact of three clinical practice changes on the timing of diagnosis and intervention for congenital hearing loss. DESIGN: A retrospective chart review was conducted for 800 infants evaluated for congenital hearing loss before and after implementing three clinical practice changes: the use of Kalman-weighted signal averaging for auditory brainstem response testing, a tone burst-prioritized testing protocol, and expediting scheduling of initial assessment. The impact of middle ear involvement on age at diagnosis and history of neonatal intensive care unit stay on age at treatment was also examined. RESULTS: The use of Kalman-weighted signal averaging for auditory brainstem response testing, a tone burst-prioritized testing protocol, and expedited scheduling of initial assessment each resulted in a decrease of age at diagnosis. Ultimately, the age at initial assessment was the only significant predictor related to decreased timeline for diagnosis. Middle ear pathology significantly increased age at diagnosis, while history of time in the neonatal intensive care unit significantly increased the age at provision of amplification as a treatment for permanent hearing loss. CONCLUSIONS: The technology used for assessment, clinical protocol, and timing of assessment of infants can impact the timeline for diagnosis and treatment of congenital hearing impairment. Given the significant sequelae of delayed or missed diagnosis of hearing loss in infancy, implementing clinical practice changes should be considered at pediatric diagnostic centers.

Parent perspectives on multidisciplinary pediatric hearing healthcare.

INTRODUCTION: Family-centered healthcare demands that families provide input regarding the care of their children. Very little is known, however, about how families perceive their experience in different types of multidisciplinary team models, and specifically, in the multidisciplinary setting currently utilized in many pediatric hearing clinics. METHODS: Quantitative and qualitative parent survey responses were collected and analyzed in a tertiary care pediatric medical center after a one-day multidisciplinary assessment clinical appointment. Questions pertained to information across five domains, including overall experience, diagnosis, treatment plan formulation, additional testing, and resources. Quantitative responses were analyzed descriptively while qualitative responses were evaluated using content analysis to derive themes. Quantitative and qualitative data were evaluated separately and then compared to delineate themes for strengths and weaknesses. RESULTS: Overall, high satisfaction was evident in both quantitative and qualitative responses. Results suggested that a one-day multidisciplinary assessment appointment may contribute to parents feeling overwhelmed by information shared and not fully understanding which disciplines are providing care. Analysis revealed a specific area of weakness in our particular setting was inadequate provision of information about functional hearing (e.g., listening socially and academically). Results contributed to a change from a multidisciplinary team model to an interdisciplinary care coordination approach to pediatric hearing healthcare. CONCLUSIONS: Understanding parent perspectives and expectations is the corner stone of family-centered care and may ultimately influence a child's developmental outcome. A systematic way of evaluating parent perspectives on the clinical process can influence service delivery and help children with hearing loss meet their potential.

(Central) Auditory Processing Disorder Grand Rounds: Multiple Cases, Multiple Causes, Multiple Outcomes.

PURPOSE: The purpose of this article was to provide multiple examples of how (central) auditory processing disorder ([C]APD) is being evaluated and treated at various audiology clinics throughout the United States. METHOD: The authors present 5 cases highlighting the diagnosis and treatment of (C)APD in children and adults. Similarities and differences between these cases have been showcased through detailed histories, evaluation protocol, and treatment options. When possible, the rationale for evaluation procedures and intervention processes were described and compared with guidelines and findings within the literature. RESULTS AND CONCLUSIONS: These cases illustrate the varied processes and clinical protocols by which children and adults are evaluated, diagnosed, counseled, and treated for (C)APD. In addition, similarities and differences between the referral source, evaluation team, developmental history, comorbidities, test battery, recommendations, and remediations were described. The multiple clinic sites, diversity of clinical philosophies, variety of test measures, and diversity of patient populations make these cases ideal for showcasing the assortment of methodologies used with patients who present with histories and characteristics consistent with (C)APD.

The Effect of Lexical Content on Dichotic Speech Recognition in Older Adults.

BACKGROUND: Age-related auditory processing deficits have been shown to negatively affect speech recognition for older adult listeners. In contrast, older adults gain benefit from their ability to make use of semantic and lexical content of the speech signal (i.e., top-down processing), particularly in complex listening situations. Assessment of auditory processing abilities among aging adults should take into consideration semantic and lexical content of the speech signal. PURPOSE: The purpose of this study was to examine the effects of lexical and attentional factors on dichotic speech recognition performance characteristics for older adult listeners. RESEARCH DESIGN: A repeated measures design was used to examine differences in dichotic word recognition as a function of lexical and attentional factors. STUDY SAMPLE: Thirty-five older adults (61-85 yr) with sensorineural hearing loss participated in this study. DATA COLLECTION AND ANALYSIS: Dichotic speech recognition was evaluated using consonant-vowel-consonant (CVC) word and nonsense CVC syllable stimuli administered in the free recall, directed recall right, and directed recall left response conditions. RESULTS: Dichotic speech recognition performance for nonsense CVC syllables was significantly poorer than performance for CVC words. Dichotic recognition performance varied across response condition for both stimulus types, which is consistent with previous studies on dichotic speech recognition. Inspection of individual results revealed that five listeners demonstrated an auditory-based left ear deficit for one or both stimulus types. CONCLUSIONS: Lexical content of stimulus materials affects performance characteristics for dichotic speech recognition tasks in the older adult population. The use of nonsense CVC syllable material may provide a way to assess dichotic speech recognition performance while potentially lessening the effects of lexical content on performance (i.e., measuring bottom-up auditory function both with and without top-down processing).

Dichotic speech recognition using CVC word and nonsense CVC syllable stimuli.

BACKGROUND: The effects of stimulus material, lexical content, and response condition on dichotic speech recognition performance characteristics were examined for normal-hearing young adult listeners. No previous investigation has systematically examined the effects of stimulus material with constant phonetic content but varied lexical content across three response conditions typically used to evaluate binaural auditory processing abilities. PURPOSE: To examine how dichotic speech recognition performance varies for stimulus materials with constant phonetic content but varied lexical content across the free recall, directed recall right, and directed recall left response conditions. RESEARCH DESIGN: Dichotic speech recognition was evaluated using consonant-vowel-consonant (CVC) word and nonsense CVC syllable stimuli administered in the free recall, directed right, and directed left response conditions, a repeated measures experimental design. STUDY SAMPLE: Thirty normal-hearing young adults (15 male, 15 female) served as participants. Participants ranged in age from 18 to 31 yr and were all right-handed. DATA COLLECTION AND ANALYSIS: Participants engaged in monaural speech recognition and dichotic speech recognition tasks. Percent correct recognition per ear, as well as ear advantage for dichotic speech recognition, were calculated and evaluated using a repeated measures analysis of variance (ANOVA) statistical procedure. RESULTS: Dichotic speech recognition performance for nonsense CVC syllables was significantly poorer than performance for CVC words, suggesting that lexical content impacts performance on dichotic speech recognition tasks. Performance also varied across response condition, which is consistent with previous studies of dichotic speech recognition. CONCLUSIONS: Lexical content of stimulus materials impacts performance characteristics for dichotic speech recognition tasks in the normal-hearing young adult population. The use of nonsense CVC syllable material may provide a way to assess dichotic speech recognition performance while potentially lessening the effects of lexical content on performance.