Descriptions of Hearing Loss Severity Differentially Influence Parental Concern about the Impact of Childhood Hearing Loss.

PURPOSE: The purpose of this study was to measure how parent concern about childhood hearing loss varies under different description conditions: classification-based, audibility-based, and simulation-based descriptions. METHOD: We randomly allocated study participants (n = 143) to complete an online survey about expected child difficulties with listening situations with hearing loss. Our participants were parents of children with typical hearing in the 0- to 12-month age range. Participants were exposed to one type of description (classification-based, audibility-based, or simulation-based) and one level of hearing loss (slight, mild, and moderate or their audibility and simulation equivalents), producing nine total groups. Participants rated the level of expected difficulty their child would experience performing age-appropriate listening tasks with the given hearing loss. They also selected what they perceived as the most appropriate intervention from a list of increasingly intense options. RESULTS: Our findings revealed that audibility-based descriptions elicited significantly higher levels of parent concerns about hearing loss than classification-based strategies, but that simulation-based descriptions elicited the highest levels of concern. Those assigned to simulation-based and audibility-based groups also judged relatively more intense intervention options as appropriate compared to those assigned to classification-based groups. CONCLUSIONS: This study expands our knowledge base about descriptive factors that impact levels of parent concern about hearing loss after diagnosis. This has potentially cascading effects on later intervention actions such as fitting hearing technology. It also provides a foundation for developing and testing clinical applications of audibility-based counseling strategies.

The audiologic profile of patients with Charcot-Marie Tooth neuropathy can be characterised by both cochlear and neural deficits.

Objective: The primary goal of this study was to characterise the cochlear and neural components of hearing loss in a large cohort of people with Charcot-Marie Tooth neuropathy who reported hearing difficulties.Design: A full complement of audiologic measures including behavioral, physiologic and subjective assessments were administered.Study sample: Seventy-nine participants completed the study. Forty-four people had CMT1, 27 had CMT2 and four had CMT-INT.Results: Pure tone average was related to age and the absence of high-frequency DPOAE energy, suggesting a strong cochlear component. Acoustic reflexes were often elevated or absent and many participants exhibited abnormal ABR waveforms, suggesting additional neural hearing loss components. Participants with an abnormal or absent ABR wave V exhibited poorer speech perception abilities. There was an association between a prolonged ABR wave I latency and an abnormal or absent ABR wave V with a higher Charcot-Marie Tooth Neuropathy Score (indicating greater disability).Conclusions: The hearing abilities of people with CMT are highly variable. While there were strong neural hearing loss components, speech perception abilities were not disproportionately affected in most participants. Therefore, a hearing aid trial is recommended. ABR responses may be a useful tool for monitoring the progression of CMT over time.

Medical Referral Patterns and Etiologies for Children With Mild-to-Severe Hearing Loss.

OBJECTIVES: To (1) identify the etiologies and risk factors of the patient cohort and determine the degree to which they reflected the incidence for children with hearing loss and (2) quantify practice management patterns in three catchment areas of the United States with available centers of excellence in pediatric hearing loss. DESIGN: Medical information for 307 children with bilateral, mild-to-severe hearing loss was examined retrospectively. Children were participants in the Outcomes of Children with Hearing Loss (OCHL) study, a 5-year longitudinal study that recruited subjects at three different sites. Children aged 6 months to 7 years at time of OCHL enrollment were participants in this study. Children with cochlear implants, children with severe or profound hearing loss, and children with significant cognitive or motor delays were excluded from the OCHL study and, by extension, from this analysis. Medical information was gathered using medical records and participant intake forms, the latter reflecting a caregiver's report. A comparison group included 134 children with normal hearing. A Chi-square test on two-way tables was used to assess for differences in referral patterns by site for the children who are hard of hearing (CHH). Linear regression was performed on gestational age and birth weight as continuous variables. Risk factors were assessed using t tests. The alpha value was set at p < 0.05. RESULTS: Neonatal intensive care unit stay, mechanical ventilation, oxygen requirement, aminoglycoside exposure, and family history were correlated with hearing loss. For this study cohort, congenital cytomegalovirus, strep positivity, bacterial meningitis, extracorporeal membrane oxygenation, and loop diuretic exposure were not associated with hearing loss. Less than 50% of children underwent imaging, although 34.2% of those scanned had abnormalities identified. No single imaging modality was preferred. Differences in referral rates were apparent for neurology, radiology, genetics, and ophthalmology. CONCLUSIONS: The OCHL cohort reflects known etiologies of CHH. Despite available guidelines, centers of excellence, and high-yield rates for imaging, the medical workup for children with hearing loss remains inconsistently implemented and widely variable. There remains limited awareness as to what constitutes appropriate medical assessment for CHH.

High Risk Factors Associated With Early Childhood Hearing Loss: A 3-Year Review.

PURPOSE: In this study, we examined the association between risk factors for hearing loss and early childhood hearing status (normal hearing, congenital hearing loss, or delayed-onset hearing loss). Follow-up rates of audiologic care following passed or referred birth screens for children with risk factors were also examined. METHOD: A retrospective data review was completed on 115,039 children born from 2010 to 2012. Data analyses included prevalence rates, odds ratios, and Fisher exact tests of statistical significance. RESULTS: Ninety percent of children were born with no risk factors for hearing loss; of those, 99.9% demonstrated normal hearing by 3 years of age. Of the 10% of children born with risk factors, 96.3% demonstrated normal hearing by age 3, 1.4% presented with congenital hearing loss, and 2.3% demonstrated permanent hearing loss by age 3. Factors that placed children at the highest risk of congenital hearing impairment were neurodegenerative disorders, syndromes, and congenital infections. Factors that placed children at the highest risk of developing permanent postnatal hearing loss were congenital cytomegalovirus, syndromes, and craniofacial anomalies. CONCLUSIONS: Certain risk factors place a child at significantly greater risk of congenital hearing impairment or developing permanent hearing loss by age 3. Follow-up diagnostic testing should remain a priority for children with certain risk factors for hearing loss.

Service Delivery to Children With Mild Hearing Loss: Current Practice Patterns and Parent Perceptions.

PURPOSE: This study investigates clinical practice patterns and parent perception of intervention for children with mild hearing loss (HL). METHOD: Ages at and delays between service delivery steps (first diagnostic evaluation, confirmation of HL, hearing aid [HA] fitting, entry into early intervention) were investigated for 113 children with mild HL. Comparisons were made to children with moderate-to-severe HL. Parents of children with mild HL reported reasons for delays and their perceptions of intervention and amplification for their children. RESULTS: Seventy-four percent of children with mild HL were identified through the newborn hearing screen; 26% were identified later due to passing or not receiving a newborn hearing screen. Ninety-four percent of children with mild HL were fit with HAs, albeit at significantly later ages than children with moderate-to-severe HL. Most parents indicated that their children benefited from HA use, but some parents expressed ambivalence toward the amount of benefit. CONCLUSIONS: Audiologists appear to be moving toward regularly providing amplification for children with mild HL. However, delays in HA fittings indicate that further educating professionals and parents about the benefits of early amplification and intervention is warranted to encourage timely fitting and consistent use of HAs.

The Influence of Hearing Aid Use on Outcomes of Children With Mild Hearing Loss.

PURPOSE: This study examined the effects of consistent hearing aid (HA) use on outcomes in children with mild hearing loss (HL). METHOD: Five- or 7-year-old children with mild HL were separated into 3 groups on the basis of patterns of daily HA use. Using analyses of variance, we compared outcomes between groups on speech and language tests and a speech perception in noise task. Regression models were used to investigate the influence of cumulative auditory experience (audibility, early intervention, HA use) on outcomes. RESULTS: Full-time HA users demonstrated significantly higher scores on vocabulary and grammar measures compared with nonusers. There were no significant differences between the 3 groups on articulation or speech perception measures. After controlling for the variance in age at confirmation of HL, level of audibility, and enrollment in early intervention, only amount of daily HA use was a significant predictor of grammar and vocabulary. CONCLUSIONS: The current results provide evidence that children's language development benefits from consistent HA use. Nonusers are at risk in areas such as vocabulary and grammar compared with other children with mild HL who wear HAs regularly. Service providers should work collaboratively to encourage consistent HA use.

Speech Recognition and Parent Ratings From Auditory Development Questionnaires in Children Who Are Hard of Hearing.

OBJECTIVES: Progress has been made in recent years in the provision of amplification and early intervention for children who are hard of hearing. However, children who use hearing aids (HAs) may have inconsistent access to their auditory environment due to limitations in speech audibility through their HAs or limited HA use. The effects of variability in children's auditory experience on parent-reported auditory skills questionnaires and on speech recognition in quiet and in noise were examined for a large group of children who were followed as part of the Outcomes of Children with Hearing Loss study. DESIGN: Parent ratings on auditory development questionnaires and children's speech recognition were assessed for 306 children who are hard of hearing. Children ranged in age from 12 months to 9 years. Three questionnaires involving parent ratings of auditory skill development and behavior were used, including the LittlEARS Auditory Questionnaire, Parents Evaluation of Oral/Aural Performance in Children rating scale, and an adaptation of the Speech, Spatial, and Qualities of Hearing scale. Speech recognition in quiet was assessed using the Open- and Closed-Set Test, Early Speech Perception test, Lexical Neighborhood Test, and Phonetically Balanced Kindergarten word lists. Speech recognition in noise was assessed using the Computer-Assisted Speech Perception Assessment. Children who are hard of hearing were compared with peers with normal hearing matched for age, maternal educational level, and nonverbal intelligence. The effects of aided audibility, HA use, and language ability on parent responses to auditory development questionnaires and on children's speech recognition were also examined. RESULTS: Children who are hard of hearing had poorer performance than peers with normal hearing on parent ratings of auditory skills and had poorer speech recognition. Significant individual variability among children who are hard of hearing was observed. Children with greater aided audibility through their HAs, more hours of HA use, and better language abilities generally had higher parent ratings of auditory skills and better speech-recognition abilities in quiet and in noise than peers with less audibility, more limited HA use, or poorer language abilities. In addition to the auditory and language factors that were predictive for speech recognition in quiet, phonological working memory was also a positive predictor for word recognition abilities in noise. CONCLUSIONS: Children who are hard of hearing continue to experience delays in auditory skill development and speech-recognition abilities compared with peers with normal hearing. However, significant improvements in these domains have occurred in comparison to similar data reported before the adoption of universal newborn hearing screening and early intervention programs for children who are hard of hearing. Increasing the audibility of speech has a direct positive effect on auditory skill development and speech-recognition abilities and also may enhance these skills by improving language abilities in children who are hard of hearing. Greater number of hours of HA use also had a significant positive impact on parent ratings of auditory skills and children's speech recognition.

Longitudinal Predictors of Aided Speech Audibility in Infants and Children.

OBJECTIVES: Amplification is a core component of early intervention for children who are hard of hearing, but hearing aids (HAs) have unique effects that may be independent from other components of the early intervention process, such as caregiver training or speech and language intervention. The specific effects of amplification are rarely described in studies of developmental outcomes. The primary purpose of this article is to quantify aided speech audibility during the early childhood years and examine the factors that influence audibility with amplification for children in the Outcomes of Children with Hearing Loss study. DESIGN: Participants were 288 children with permanent hearing loss who were followed as part of the Outcomes of Children with Hearing Loss study. All of the children in this analysis had bilateral hearing loss and wore air-conduction behind-the-ear HAs. At every study visit, hearing thresholds were measured using developmentally appropriate behavioral methods. Data were obtained for a total of 1043 audiometric evaluations across all subjects for the first four study visits. In addition, the aided audibility of speech through the HA was assessed using probe microphone measures. Hearing thresholds and aided audibility were analyzed. Repeated-measures analyses of variance were conducted to determine whether patterns of thresholds and aided audibility were significantly different between ears (left versus right) or across the first four study visits. Furthermore, a cluster analysis was performed based on the aided audibility at entry into the study, aided audibility at the child's final visit, and change in aided audibility between these two intervals to determine whether there were different patterns of longitudinal aided audibility within the sample. RESULTS: Eighty-four percent of children in the study had stable audiometric thresholds during the study, defined as threshold changes <10 dB for any single study visit. There were no significant differences in hearing thresholds, aided audibility, or deviation of the HA fitting from prescriptive targets between ears or across test intervals for the first four visits. Approximately 35% of the children in the study had aided audibility that was below the average for the normative range for the Speech Intelligibility Index based on degree of hearing loss. The cluster analysis of longitudinal aided audibility revealed three distinct groups of children: a group with consistently high aided audibility throughout the study, a group with decreasing audibility during the study, and a group with consistently low aided audibility. CONCLUSIONS: The current results indicated that approximately 65% of children in the study had adequate aided audibility of speech and stable hearing during the study period. Limited audibility was associated with greater degrees of hearing loss and larger deviations from prescriptive targets. Studies of developmental outcomes will help to determine how aided audibility is necessary to affect developmental outcomes in children who are hard of hearing.

Timeliness of service delivery for children with later-identified mild-to-severe hearing loss.

PURPOSE: In this study, the authors examined diagnostic and intervention services for children identified with hearing loss (HL) after the newborn period. METHOD: The authors compared ages at service delivery and length of delays between service delivery steps for 57 later-identified children with HL and 193 children who referred for assessment from the newborn hearing screen (NHS). For only later-identified children, regression models were used to investigate relationships among predictor variables and dependent variables related to service delivery. RESULTS: Children who referred from the NHS received follow-up services at younger ages than later-identified children. Later-identified children had significantly longer delays from HL confirmation to entry into early intervention, compared to children who referred from the NHS. For later-identified children, degree of HL predicted ages at follow-up clinical services. Children with more severe HL received services at younger ages compared to children with milder HL. Gender predicted the length of the delay from confirmation to entry into early intervention, with girls demonstrating shorter delays. CONCLUSIONS: The current results lend support to the need for ongoing hearing monitoring programs after the neonatal period, particularly when children enter early intervention programs because of language/developmental delays.

Clinical and genetic features of hearing loss in facioscapulohumeral muscular dystrophy.

OBJECTIVE: To describe the hearing loss in facioscapulohumeral muscular dystrophy (FSHD) and examine the relationship to genotype. METHODS: Medical records of all individuals with FSHD seen at the University of Iowa neuromuscular clinic between July 2006 and July 2012 (n = 59) were reviewed. Eleven had significant hearing loss and no non-FSHD cause. All available audiology records for these individuals were analyzed. The relationship between the FSHD mutation (EcoRI/BlnI fragment size) and hearing loss was evaluated using a logistic regression analysis. RESULTS: In patients with hearing loss, recalled age at onset of facial weakness ranged from birth to 5 years and shoulder weakness was 3 to 15 years. The age at diagnosis of hearing loss ranged from birth to 7 years. Only 2 were identified by newborn hearing screen. Most audiograms demonstrated a bilateral, sloping, high-frequency sensorineural hearing loss. Of the 4 patients with more than 5 years of data, 3 had progression of hearing loss. Logistic regression showed statistically significant negative association between the presence of hearing loss and EcoRI/BlnI fragment size (p = 0.0207). CONCLUSIONS: FSHD with a small EcoRI/BlnI fragment is associated with a bilateral, progressive, sloping, high-frequency hearing loss with onset in childhood. Patients with FSHD and small EcoRI/BlnI fragment sizes should have hearing screened, even if the child passed newborn hearing screening.

Factors influencing follow-up to newborn hearing screening for infants who are hard of hearing.

PURPOSE: To document the epidemiological characteristics of a group of children who are hard of hearing, identify individual predictor variables for timely follow-up after a failed newborn hearing screening, and identify barriers to follow-up encountered by families. METHOD: The authors used an accelerated longitudinal design to investigate outcomes for children who are hard of hearing in a large, multicenter study. The present study involved a subgroup of 193 children with hearing loss who did not pass the newborn hearing screening. The authors used available records to capture ages of confirmation of hearing loss, hearing aid fitting, and entry into early intervention. Linear regression models were used to investigate relationships among individual predictor variables and age at each follow-up benchmark. RESULTS: Of several predictor variables, only higher levels of maternal education were significantly associated with earlier confirmation of hearing loss and fitting of hearing aids; severity of hearing loss was not. No variables were significantly associated with age of entry into early intervention. Each recommended benchmark was met by a majority of children, but only one third met all of the benchmarks within the recommended time frame. CONCLUSION: Results suggest that underserved communities need extra support in navigating steps that follow failed newborn hearing screening.

Tympanometry in newborn infants--1 kHz norms.

With the rapid implementation of universal newborn hearing screening (UNHS) programs, a test of middle-ear function for infants is urgently needed. Recent evidence suggests that 1 kHz tympanometry may be effective. Normative data are presented for newborn intensive care unit (NICU) graduates tested at a mean age of 3.9 weeks (Study 1) and full-term infants tested at 2-4 weeks (Study 2) who passed an otoacoustic emissions (OAE) screen. Nearly all infants tested had single-peaked tympanograms. The norms are evaluated for a group of full-term infants who were screened with OAE (Study 3) and two groups of infants (NICU patients and well babies) who were not screened by OAE (Study 4). The 5th percentile for static admittance for NICU and full-term babies was identical, allowing a sinngle pass-fail criterion. Using that criterion, well babies who passed an OAE screen (Study 3) yielded a 91% pass rate. Those who passed the OAE screen had substantially higher 1 kHz static admittance than those who failed, suggesting a strong relationship between middle-ear transmission characteristics and OAE responses. The pass rate was lower for newborn well babies and NICU graduates who were not screened by OAE (Study 4).

Early childhood hearing loss: a frequently overlooked cause of speech and language delay.

The explosion of information regarding the genetics of hearing loss, the extraordinary effectiveness of early intervention, and the widespread practice of universal newborn hearing screening make for exciting times for those who serve young children who are deaf or hard-of-hearing and their families. These rapid changes in the knowledge base and practice standards also require the cooperation and help of pediatricians to enable children to take full advantage of available opportunities to optimize development of early communication.