Abbey L. Berg

The New York State universal newborn hearing screening demonstration project: Ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention

Objective: To determine the ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention through a multi‐center, state‐wide universal newborn hearing screening project. Design: Universal newborn hearing screening was conducted at eight hospitals across New York State. All infants who did not bilaterally pass hearing screening before discharge were recalled for outpatient retesting. Inpatient screening and outpatient rescreening were done with transient evoked otoacoustic emissions and/or auditory brain stem response testing. Diagnostic testing was performed with age appropriate tests, auditory brain stem response and/or visual reinforcement audiometry. Infants diagnosed with permanent hearing loss were considered for hearing aids and early intervention. Ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention were investigated regarding nursery type, risk status, unilateral versus bilateral hearing loss, loss type, loss severity, and state regions. Results: The prevalence of infants diagnosed with permanent hearing loss was 2.0/1000 (85 of 43,311). Of the 85 infants with hearing loss, 61% were from neonatal intensive care units (NICUs) and 67% were at risk for hearing loss. Of the 36 infants fitted with hearing aids, 58% were from NICUs and 78% were at risk for hearing loss. The median age at identification and enrollment in early intervention was 3 mo. Median age at hearing aid fitting was 7.5 mo. Median ages at identification were less for infants from the well‐baby nurseries (WBNs) than for the NICU infants and for infants with severe/profound than for infants with mild/moderate hearing loss, but were similar for not‐at‐risk and at‐risk infants. Median ages at hearing aid fitting were less for well babies than for NICU infants, for not‐at‐risk infants than for at‐risk infants, and for infants with severe/profound hearing loss than for infants with mild/moderate hearing loss. However, median ages at early intervention enrollment were similar for nursery types, risk status, and severity of hearing loss. Conclusions: Early ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention can be achieved for infants from NICUs and WBNs and for infants at risk and not at risk for hearing loss in a large multi‐center universal newborn hearing screening program.

Newborn Hearing Screening in the NICU: Profile of Failed Auditory Brainstem Response/Passed Otoacoustic Emission

Objective. Incidence of a specific pattern of auditory responses, absent auditory brainstem responses (ABRs) and present otoacoustic emissions (OAEs), in newborn hearing screening in a regional perinatal center neonatal intensive care unit (NICU) is described. This profile, labeled auditory neuropathy or auditory dyssynchrony (AN/AD), is a dysfunction in neural/brainstem transmission that occurs in individuals whose outer hairs cells are functioning normally. Although the AN/AD profile has been associated with various risk factors, incidence and prediction are unknown. Method. Analysis of electrophysiologic measures and medical record reviews of the first 22 months of the universal newborn hearing–screening program was conducted. Association of the AN/AD profile was evaluated with the following factors: gender, gestational age, ototoxic drug regimen, low birth weight, hyperbilirubinemia, hydrocephalus, low Apgar score, anoxia, respiratory distress syndrome, pulmonary hypertension, intraventricular hemorrhage, multiple birth, seizure activity, and family history. Results. One hundred fifteen (24.1%) of the 477 infants failed the ABR in 1 or both ears and passed OAEs bilaterally. Comparisons of infants fitting the AN/AD profile with those not fitting the AN/AD profile were negative with 3 exceptions: those with hyperbilirubinemia and those who were administered vancomycin or furosemide. A logistic-regression analysis model failed to predict which infants would be at risk for the AN/AD profile either unilaterally or bilaterally. Conclusions. Screening of NICU infants should be conducted with ABR first, followed by OAE after failure on ABR. Because the incidence of the AN/AD profile was found to be 24% in this at-risk population, additional study is warranted.

The New York State universal newborn hearing screening demonstration project: outpatient outcome measures.

Objective: To investigate outpatient outcome measures of a multi‐center, state‐wide, universal newborn hearing screening project. Design: Eight hospitals participated in a 3‐yr, funded project. Each hospital designed its own protocol using common criteria for judging whether an infant passed a hearing screening. Infants were tested in the hospital, and those either failing the in‐hospital screening or who were not tested in the hospital (missed) were asked to return 4 to 6 wk after hospital discharge for outpatient rescreening. Those infants failing the outpatient rescreening were referred for diagnostic auditory brain stem response testing. Each hospital used its own audiological equipment and criteria to determine whether a particular infant had a hearing loss. All data were collected and analyzed for individual hospitals, as well as totaled across all hospitals. Data were analyzed in terms of year of program operation, nursery type, and geographic region. Results: Seventy‐two percent of infants who failed the in‐hospital screening returned for outpatient testing. The percentage of in‐hospital fails returning for retesting was significantly higher than the percentage of in‐hospital misses returning for retesting. The percentage of infants returning for retesting increased with successive years of program operation. Some differences were noted in the percentage of infants returning for retesting among hospitals and geographic regions of the state. Some differences in outpatient outcome measures also were noted between infants originally born into the neonatal intensive care unit (NICU) and the well‐baby nursery (WBN). The percentage of infants from the NICU who returned for retesting was slightly higher than that for infants from the WBN. The percentage of infants from the WBN passing the outpatient rescreening was higher than that for the NICU infants. The overall prevalence of hearing loss was 1.96/1000, with that in the NICU being 8/1000 and that in the WBN being 0.9/1000. Positive predictive value for permanent hearing loss based on inpatient screening was approximately 4% and based on outpatient rescreening was approximately 22%. Conclusions: Several outpatient outcome measures changed with successive years of program operation, suggesting that programs improve over time. Also, some outpatient outcome measures differ between NICU and WBN populations. The differences noted across regions of the state in the percentage of infants returning for outpatient retesting require further research to determine whether differences are due to demographic and/or procedural differences.

Ototoxic impact of cisplatin in pediatric oncology patients.

Objective: To describe hearing changes in a group of 28 children (age range, 8‐180 mo) undergoing protocol‐based cisplatin therapy.

New York State universal newborn hearing screening demonstration project: effects of screening protocol on inpatient outcome measures.

Objective: To examine differences among various test protocols on the fail rate at hospital discharge for infants in the well‐baby nursery (WBN) and neonatal intensive care unit (NICU) who received hearing screening through a universal newborn hearing screening demonstration project. Design: The outcomes of several screening protocols were examined. Two technologies were used: transient evoked otoacoustic emissions (TEOAEs) alone or in combination with the auditory brain stem response (ABR). The performance of test protocols in both nurseries within eight hospitals was examined over a 2‐ to 3‐yr period. In the WBN, six hospitals used a screening protocol of TEOAE technology first followed by an ABR (automated or conventional) technology screening for newborns who referred on TEOAE screening. Two hospitals used TEOAE only in the WBN. Seven hospitals used screening protocols in the NICU that used a combination of TEOAE and ABR technologies (TEOAE technology administered first or second, before or after TEOAE, or TEOAE and ABR tests on all infants). Only one hospital used TEOAE technology exclusively for hearing screening. Results: Significant differences among screening protocols were found across hospitals in the first, second, and third years of the program. The combination of TEOAE technology and ABR technology (a two‐technology screening protocol) resulted in a significantly lower fail rate at hospital discharge than the use of a single‐technology (TEOAE). Fail rates at discharge were twice as high using the one‐technology protocol versus two‐technology protocol, even when the best outcomes from program year 3 were considered exclusively. Results of two‐technology versus one‐technology protocols were similar in the NICU. Use of a second technology for screening TEOAE fails significantly reduced every hospital that used the protocols fail rate at discharge. Conclusions: A two‐technology screening protocol resulted in significantly lower fail rates at hospital discharge in both the WBN and NICU nurseries than use of a single‐technology (TEOAE) hearing screening protocol.

Pervasive Developmental Delay in Children Presenting As Possible Hearing Loss

Objective/Hypothesis: Children who fail to develop adequate language skills and/or appropriate social skills by age 2 years often are referred to the department of otolaryngology for otolaryngologic examination and evaluation of possible hearing deficits. Discovering a gross disparity between hearing function and language ability often uncovers an underlying developmental disorder satisfying criteria for diagnosis on the spectrum of autism and pervasive developmental delay (PDD). The otolaryngologist has a unique opportunity to identify these autistic children and initiate their evaluation and management. Study Design: Retrospective review. Methods: Review of charts of children referred over the past 4 years to the Department of Otolaryngology for possible hearing loss identified 15 children who were later diagnosed with PDD. Results: Fifteen children initially referred for hearing evaluation were subsequently identified with a diagnosis of PDD. Males outnumbered females 4 to 1, with the average age of referral being 2 years. One third of the patients displayed middle ear disease that improved with PE tube placement. One third of the patients showed brainstem conduction dysfunction on auditory brainstem evoked response testing. Conclusions: Children with developmental delays, especially higher functioning ones, may present with a myriad of language and communication deficits that are often mistakenly attributed to hearing loss. Otolaryngologists and audiologists can assist in their early identification and appropriate referral for therapy.

Influence of Hearing Risk Information on the Motivation and Modification of Personal Listening Device Use.

Purpose The purpose of this study was (a) to investigate the behaviors, knowledge, and motivators associated with personal listening device (PLD) use and (b) to determine the influence of different types of hearing health risk education information (text with or without visual images) on motivation to modify PLD listening use behaviors in young adults. Method College-age students (N = 523) completed a paper-and-pencil survey tapping their behaviors, knowledge, and motivation regarding listening to music or media at high volume using PLDs. Participants rated their motivation to listen to PLDs at lower volume levels following each of three information sets: text only, behind-the-ear hearing aid image with text, and inner ear hair cell damage image with text. Results Acoustically pleasing and emotional motives were the most frequently cited (38%-45%) reasons for listening to music or media using a PLD at high volume levels. The behind-the-ear hearing aid image with text information was significantly (p < .0001) more motivating to participants than text alone or the inner ear hair cell damage image with text. Conclusions Evocative imagery using hearing aids may be an effective approach in hearing protective health campaigns for motivating safer listening practices with PLDs in young adults.

The Effectiveness of Parental Questionnaires in the Assessment of Speech-Language and Auditory Function in Children

Objective: This study investigates the use of a parental questionnaire to determine its effectiveness as a screening tool in identifying speech-language and/or auditory impairments in children. Patients and Methods: Parents completed speech-language (n = 36) or audiology (n = 23) questionnaires prior to their child’s speech-language and/or auditory evaluations. The speech-language and audiology evaluations were conducted by clinicians at three different universities not familiar with the administration and scoring of the parental questionnaires. A research assistant coded the results of the speech-language and audiology evaluations. Statistical analysis was used to determine if any aspects of the parents’ evaluation of their child’s speech, language, or hearing correlated with the speech-language pathologists’/audiologists’ assessment. The results of the analyses would indicate whether parental reports are a reliable alternative to professionals’ assessment. Results: The speech-language questionnaires for children aged 2–5 years revealed the parents’ perception of their children speaking 3-word sentences significantly (p < 0.05) corresponded to the children’s comprehension and expression skills. The results from the auditory questionnaires revealed that reports of a child’s ear pain were (p < 0.05) correlated with auditory dysfunction. Conclusions: These questionnaires can potentially be used in low-income countries where professional resources are scarce and there are barriers to identifying children with speech-language and/or auditory impairment.