Sensitivity to Deaf Culture Among Otolaryngology and Audiology Trainees.

OBJECTIVE: The Deaf community is an ethnolinguistic minority group. Low sensitivity to Deaf culture contributes to health disparities among Deaf patients. This study determines the level of sensitivity to Deaf culture among otolaryngology-head and neck surgery (OHNS) and audiology trainees. METHODS: Cross-sectional survey study of OHNS and audiology trainees from 10 large US institutions. Trainees were queried on their exposure to and comfort with Deaf patients and their education on, attitude toward, and awareness and knowledge of Deaf culture. Sensitivity to Deaf culture was operationalized as awareness and knowledge of Deaf culture. These were assessed using a 35-item instrument that was previously developed using a d/Deaf community-based participatory approach to research. We used T-tests to compare the sample to previous samples of medical students with training in Deaf culture (MS-TDCs) and general practitioners (GPs). RESULTS: There were 91 completed surveys (response rate 44.5%). Almost all were aware of Deaf culture (97.8%). The mean knowledge score was 55.0% (standard deviation (SD) 13.4%), which was significantly higher than that for GPs at 43.0% (SD 15.0%) (95% confidence interval 8.1%, 15.8%, P < .0001) but significantly lower than that for MS-TDCs at 69.0% (SD 13.0%)(CI -20.3%, -7.6%, P < .0001). Knowledge scores were comparable for OHNS and audiology trainees (P = .09). CONCLUSION: This sample of OHNS and audiology trainees was more sensitive to Deaf culture than GPs but less sensitive than MS-TDCs. Developing specialty-specific education may be warranted. LEVEL OF EVIDENCE: 4.

Educational Environments and Secondary School Outcomes Among Students Who Are D/deaf and Hard of Hearing in Special Education.

PURPOSE: The purpose of this study is to provide updated national estimates on the annual number, educational environments, and secondary school outcomes of students who are D/deaf and hard of hearing (D/HH) receiving special education (SpEd) and related services in the United States. METHOD: We performed a retrospective cross-sectional descriptive analysis of Individuals with Disabilities Education Act, Part B, Section 618 data from 2012 to 2018. Participants included students 6-21 years old in SpEd with "hearing impairment" reported as their primary disability. The general population of students in secondary school served as a comparator, via Current Population Survey data. We described the annual number of students (a) overall, (b) by educational environment, and (c) by reason for exiting SpEd, including the proportion graduating from and dropping out of secondary school. We described variation over time. RESULTS: The median annual number of students was 67,655, with minimal variation by year. The proportion in general education (GenEd) for ≥ 80% of the day increased by 4.2% over 6 years from 57.8% to 62.0%, whereas the proportions in GenEd for < 40% and 40%-79% of the day decreased by 1.6% and 1.3%, respectively. Proportions in the remainder of the environments changed < 1.0% each. Of exiters, 86.8% of students graduated, whereas 3.9% dropped out, compared to a dropout rate of 5.0% in the general population. CONCLUSION: From 2012 to 2018, students who are D/HH receiving SpEd in the United States have spent increasingly more time in GenEd, most graduated from high school, and few dropped out, with dropout patterns appearing similar to the general population.

Defining Essential Services for Deaf and Hard of Hearing Children during the COVID-19 Pandemic.

COVID-19 is a rapidly growing global pandemic caused by a novel coronavirus. With no vaccine or definitive treatment, public health authorities have recommended a strategy of "social distancing," reducing individual interaction, canceling elective procedures, and limiting nonessential services. Health care providers must determine what procedures are considered "elective," balancing risk of treatment delays with that of coronavirus exposure to patient, family, and providers. Given critical periods for language development and the long-term impact of auditory deprivation, some audiologic and otologic services should be considered essential. In this article, we describe the experience of a quaternary referral pediatric hospital in Seattle, the epicenter of COVID-19 in the United States, and share strategies for risk minimization employed by Seattle Children's Hospital. We hope that this work can be a reference for other centers continuing care for children who are deaf and hard of hearing during the COVID-19 and future resource-limiting crises.

Reliability of Measuring Insertion Depth in Cochlear Implanted Infants and Children Using Cochlear View Radiography.

OBJECTIVES: Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography. STUDY DESIGN: Prospective, observational. SETTING: Tertiary referral pediatric hospital. SUBJECTS AND METHODS: Patients aged 11 months to 20 years (median, 4 years; interquartile range [IQR], 1-8 years) undergoing cochlear implantation at our institution were studied over 1 year. Children underwent cochlear view imaging on postoperative day 1. Films were deidentified and 1 image per ear was selected. Two cochlear implant surgeons and 2 radiologists evaluated each image and determined angular depth of insertion. Images were re-reviewed 6 weeks later by all raters. Inter- and intrarater reliability were calculated with intraclass correlation coefficients (ICCs). RESULTS: Fifty-seven ears were imaged from 42 children. Forty-nine ears (86%) had successful cochlear view x-rays. Median angular depth of insertion was 381° (minimum, 272°; maximum, 450°; IQR, 360°-395°) during the first round of measurement. Measurements of the same images reviewed 6 weeks later showed median depth of insertion of 382° (minimum, 272°; maximum, 449°; IQR, 360°-397°). Interrater and intrarater reliability ICCs ranged between 0.81 and 0.96, indicating excellent reliability. CONCLUSIONS: Postoperative cochlear view radiography is a reliable tool for measurement of cochlear implant depth of insertion in infants and children. Further studies are needed to determine reliability of intraoperatively obtained cochlear view radiographs in this population.

Cochlear Implantation in Children with Postlingual Progressive Steeply Sloping High-Frequency Hearing Loss.

BACKGROUND: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. PURPOSE: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). RESEARCH DESIGN: Retrospective case series. STUDY SAMPLE: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children's Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. DATA COLLECTION AND ANALYSIS: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. RESULTS: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. CONCLUSIONS: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.

Hearing Loss in Children With Craniofacial Microsomia.

OBJECTIVE: To evaluate the association between craniofacial phenotype and hearing loss in children with craniofacial microsomia. DESIGN: Retrospective cohort study. SETTING: Tertiary care children's hospital. PATIENTS: Individuals with craniofacial microsomia. MAIN OUTCOME MEASURES: Ear-specific audiograms and standardized phenotypic classification of facial characteristics. RESULTS: A total of 79 participants were included in the study. The mean age was 9 years (range, 1 to 23 years) and approximately 60% were boys. Facial anomalies were bilateral in 39 participants and unilateral in 40 participants (24 right, 16 left). Microtia (hypoplasia of the ear) was the most common feature (94%), followed by mandibular hypoplasia (76%), soft tissue deficiency (60%), orbital hypoplasia or displacement (53%), and facial nerve palsy (32%). Sixty-five individuals had hearing loss (12 bilateral and 53 unilateral). Hearing loss was conductive in 73% of affected ears, mixed in 10%, sensorineural in 1%, and indeterminate in 16%. Hypoplasia of the ear or mandible was frequently associated with ipsilateral hearing loss, although contralateral hearing loss occurred in 8% of hemifaces. CONCLUSIONS: Hearing loss is strongly associated with malformations of the ipsilateral ear in craniofacial microsomia and is most commonly conductive. Hearing loss can occur contralaterally to the side with malformations in children with apparent hemifacial involvement. Children with craniofacial microsomia should receive early diagnostic hearing assessments.

Psychoacoustic performance and music and speech perception in prelingually deafened children with cochlear implants.

The number of pediatric cochlear implant (CI) recipients has increased substantially over the past 10 years, and it has become more important to understand the underlying mechanisms of the variable outcomes in this population. In this study, psychoacoustic measures of spectral-ripple and Schroeder-phase discrimination, the Clinical Assessment of Music Perception, and consonant-nucleus-consonant (CNC) word recognition in quiet and spondee reception threshold (SRT) in noise tests have been presented to 11 prelingually deafened CI users, aged 8-16 years with at least 5 years of CI experience. The children's performance was compared to the previously reported results of postlingually deafened adult CI users. The average spectral-ripple threshold (n = 10) was 2.08 ripples/octave. The average Schroeder-phase discrimination was 67.3% for 50 Hz and 56.5% for 200 Hz (n = 9). The Clinical Assessment of Music Perception test showed that the average complex pitch direction discrimination was 2.98 semitones. The mean melody score was at a chance level, and the mean timbre score was 34.1% correct. The mean CNC word recognition score was 68.6%, and the mean SRT in steady noise was -8.5 dB SNR. The children's spectral-ripple resolution, CNC word recognition, and SRT in noise performances were, within statistical bounds, the same as in a population of postlingually deafened adult CI users. However, Schroeder-phase discrimination and music perception were generally poorer than in the adults. It is possible then that this poorer performance seen in the children might be partly accounted for by the delayed maturation in their temporal processing ability, and because of this, the children's performance may have been driven more by their spectral sensitivity.

Congenital cytomegalovirus infection in pediatric hearing loss.

OBJECTIVES: To compare the prevalence of congenital cytomegalovirus (CMV) infection in Washington State in children with hearing loss (HL) and the general population and to compare the characteristics of HL in children with and without congenital CMV infection. DESIGN: Matched case-control; case cohort. SETTING: Regional pediatric hospital, Washington State Department of Health (WSDOH). PATIENTS: Cases were children 4 years and older with HL born in Washington State. Control individuals matched for demographic characteristics were identified at random through the WSDOH. MAIN OUTCOME MEASURES: Congenital CMV status determined using quantitative polymerase chain reaction testing on newborn heel stick blood spots archived by the WSDOH. Audiologic data were used to characterize HL. RESULTS: Congenital CMV testing was performed for 222 matched cases and controls. Congenital CMV infection was detected in 1.4% of controls and in 9.9% of cases (odds ratio, 10.5; 95% confidence interval, 2.6-92.4). An estimated 8.9% of HL in children in Washington can be attributed to CMV infection. After inclusion of an additional 132 children with HL (for a total of 354 cases in the case cohort), we observed that children with congenital CMV had more severe HL (P < .001) and higher proportions of progressive (P = .02) and unilateral (P = .002) HL compared with children without congenital CMV infection. In the 35 children with congenital CMV infection, there was no relationship between neonatal CMV load and severity of HL. CONCLUSIONS: In Washington State, children with HL had a far higher prevalence of congenital CMV viremia than did the general pediatric population, and CMV infection seems to be responsible for an appreciable fraction of pediatric HL in Washington State.

Measuring sound detection and reaction time in infant and toddler cochlear implant recipients using an observer-based procedure: a first report.

OBJECTIVES: First, to establish the feasibility of the observer-based psychophysical procedure (OPP) in measuring sound detection in infant and toddler cochlear implant (CI) recipients. Second, to measure the psychometric function for detection (PFD) from individual subjects. Third, to determine whether reaction time (RT) provides information about the auditory sensitivity of young CI users. DESIGN: Twelve CI recipients, 11 to 32 mo old, participated in our study. Initially, tones were presented in sound field, and children learned to respond when they heard tones but not at other times. Once an 80% correct criterion was met in sound field, a novel stimulation paradigm was used to present stimuli to a single electrode while the child listened to acoustic input on most other electrodes using their usual map. The PFD and RT were measured using this single-electrode stimulation paradigm. RESULTS: Eleven subjects met criterion, 6 within the minimum possible number of trials. For eight subjects, the asymptotic level of detecting single-electrode stimuli averaged 86% correct, similar to levels achieved by normal-hearing infants and toddlers detecting pure tones. The PFD slope of infant and toddler CI recipients was less than or equal to the slope for adult CI users reported in previous studies. RT decreased significantly with stimulus level in four children. CONCLUSIONS: These preliminary results suggest that psychophysical detection data can be obtained from infant and toddler CI recipients using OPP. The PFD of young CI users may be shallower than that of adult CI users. Relatively good asymptotic detection performance implies that young CI users are more attentive to sound than has been suggested in previous studies. RT tended to be a less reliable measure of detection, but methodological changes could improve its utility.

The effects of aging and hearing loss on distortion product otoacoustic emissions.

OBJECTIVE: We sought to demonstrate the rate of change in distortion product otoacoustic emission (DPOE) amplitude with age in relation to hearing loss in an unselected adult population. Study design and setting We conducted a cross-sectional observation study involving the Framingham Offspring Cohort. Age changes in DPOE amplitude for frequencies of f2 from 1 to 8 kHz adjusted for pure-tone threshold level were assessed by multivariate linear regression. RESULTS: The women showed a mean hearing threshold-adjusted loss in high-frequency DPOE amplitude of 0.6, 2.1, 2.6, and 1.1 dB/per decade at the f2 frequencies of 1, 2, 4, and 8 kHz, respectively. In contrast, the men showed no effect of age on the DPOE amplitude independent of hearing loss. Emissions were reduced or absent in the noise notch frequencies. The rate of change with age in DPOE amplitude was significantly less than the rate of change in pure-tone thresholds in both the men and the women. CONCLUSION: Women lose DPOE amplitude from both age and hearing threshold loss. Men lose more DPOE amplitude than do women, and the loss is proportional to the degree of loss of hearing threshold sensitivity. The differential effect whereby age-related hearing loss affects thresholds more than emissions suggests that strial atrophy may be a pathophysiologic factor. SIGNIFICANCE: The use of DPOE measures for screening and monitoring cochlear status of adult women should take into account the age, pure-tone thresholds, and noise exposure status of the subjects.

Changes in Otoacoustic Emissions and Auditory Brain Stem Response after C/s-Platinum Exposure in Gerbils.

Ototoxicity associated with cis-platinum administration commonly presents as hearing loss and tinnitus. The hearing loss is usually an irreversible, high-frequency sensorineural loss. Histologic studies in humans and animals suggest that the outer hair cells (OHCs) are most susceptible to cis-platinum. Evoked otoacoustic emissions (EOAE), as a measure of outer hair cell function, are potentially useful in following ototoxic insults involving OHCs. Distortion-product otoacoustic emissions (DPOAE) test frequency-specific regions of the cochlea and therefore may be particularly well suited for monitoring ototoxic injuries. We measured distortion product otoacoustic emissions, at f2 = 2, 4, 6, 8, 10, and 12 kHz, in gerbils after a single large dose of cis-platinum. Animals treated with saline served as controls. The findings were compared to auditory brain stem evoked response (ABR) thresholds, using tone pips of the same frequencies. The DPOAE and ABR thresholds were measured before treatment and again 2, 5, and 14 days after drug administration. The changes in DPOAE were compared with the changes in ABR. No treatment effect was noted in the 2-day group. Animals treated with c/s-platinum demonstrated significant elevation of DPOAE and ABR thresholds compared with control animals at 5 and 14 days. There was no significant difference between the threshold changes in the 5-and 14-day groups.