Pediatric Audiology Productivity: Results From a Multicenter Survey.

Purpose This report focuses on audiology productivity measures and targets, impacting variables, and implementation of productivity in pediatric settings and "life span facilities" with a significant population of pediatric patients. Research Design A questionnaire was sent to 116 facilities in geographically diverse locations. Results Of the 116 surveys sent, 25 surveys were returned (response rate of 21.5%) and 22 had sufficient data to be included for analyses. The majority of the sites measure productivity at the individual and/or department level. No predominant productivity measurement method was reported. Measurement methods included the following: appointment length (n = 6), weighting Current Procedural Terminology codes (n = 6), relative value units (n = 5), a combination of methods (n = 1), and other specific parameters (n = 1). Thirteen sites measured productivity as a percentage of scheduled worked time with a target of 69% (ranged from 40% to 85%). Many variables thought to impact productivity were evaluated: age of the patient, scheduling, use of a 2nd tester, no-show rate, hearing aid status, components of auditory brainstem response evaluation, documentation time, and monaural or binaural services. Schedule review and adjustment, efficiency, and communication with staff were methods reported to increase productivity. Conclusion Most surveyed facilities measure productivity at the individual and/or department levels. However, there is no standard method. Current productivity measures include time-based measures based on appointment length, time-based measures assigned to Current Procedural Terminology codes, relative value units, combined approaches, and individual site developed programs. Many variables impact productivity measurements. Further study and discussion are needed to develop an optimal method for productivity measurement in audiology.

Longitudinal hearing loss in Wolfram syndrome.

BACKGROUND: Wolfram syndrome (WFS) is a rare autosomal recessive disease with clinical manifestations of diabetes mellitus (DM), diabetes insipidus (DI), optic nerve atrophy (OA) and sensorineural hearing loss (SNHL). Although SNHL is a key symptom of WFS, there is limited information on its natural history using standardized measures. Such information is important for clinical care and determining its use as an outcome measure in clinical trials. METHODS: Standardized audiologic measures, including pure-tone testing, tympanometry, speech perception, and the unaided Speech Intelligibility Index (SII) were assessed in patients with confirmed WFS annually. Mixed model analyses were used to examine main effects of age, time or interactions for pure tone average (PTA), high frequency average (HFA) and SII. RESULTS: Forty WFS patients were evaluated between 1 and 6 times. Mean age at initial enrollment was 13.5 years (SD = 5.6). Patients were classified as having normal hearing (n = 10), mild-to-severe (n = 24) or profound SNHL (n = 6). Mean age of diagnosis for SNHL was 8.3 years (SD = 5.1) with 75% prevalence. HFA worsened over time for both ears, and SII worsened over time in the worse ear, with greater decline in both measures in younger patients. Average estimated change over 1 year for all measures was in the subclinical range and power analyses suggest that 100 patients would be needed per group (treatment vs. placebo) to detect a 60% reduction in annual change of HFA over 3 years. If trials focused on just those patients with SNHL, power estimates suggest 55 patients per group would be sufficient. CONCLUSIONS: Most patients had a slow progressive SNHL emerging in late childhood. Change over time with standard audiologic tests (HFA, SII) was small and would not be detectable for at least 2 years in an individual. Relatively large sample sizes would be necessary to detect significant impact on hearing progression in a clinical trial. Hearing function should be monitored clinically in WFS to provide appropriate intervention. Because SNHL can occur very early in WFS, audiologists and otolaryngologists should be aware of and refer for later emerging symptoms.

Audiology Assistants: Results of a Multicenter Survey.

BACKGROUND: Although audiologists have been using support personnel for over 45 yr, controversy and variability continue with respect to the entry-level education, training methods, and scope of practice. PURPOSE: As part of a larger clinical practices survey, this report focuses on use of audiology assistants (AAs) for pediatric settings and "life-span" facilities that had a significant population of pediatric patients. RESEARCH DESIGN: A questionnaire was sent to 116 facilities in geographically diverse locations. Of the 25 surveys returned, 22 had sufficient data to be included for analysis purposes. RESULTS: The majority of respondents assigned duties to AAs as follows: assisting with conditioned play audiometry and visual reinforcement audiometry, infection control, mail management, disposing of protected health information, ordering supplies, calling families, fielding family phone calls, and stocking supplies. In addition, of the nine pediatric facilities that used AAs and reported job duties, the majority assigned troubleshooting equipment and auditory brainstem response (ABR) screening. Two of the five life-span facilities that reported job duties assigned several duties not assigned by any of the pediatric facilities: pure-tone screening, earmold impressions, assisting with videonystagmography and ABR, and in-house hearing aid repairs. Of facilities that use AAs and reported staffing, the ratio of AAs to audiologists ranged from 0.03:1 to 1:0.37, with an average of 0.15 for life-span facilities and 0.17 for the pediatric facilities. Minimum educational levels required were reported as follows: high school (n = 8), college (n = 3), certificate (n = 1), and no requirement (n = 1). CONCLUSIONS: Within a small sample size of pediatric and life-span facilities, 14 of 22 centers used AAs to perform a variety of direct patient care, indirect patient care, and clerical duties. Based on the duties recommended within the American Speech-Language-Hearing Association guidelines and by many states, expanded employment of AAs, as well as expansion of assigned duties should be considered. Data are needed to determine the appropriate ratio of AAs to audiologists within different settings and to determine the impact of AAs for accessibility, productivity, and profitability.

Efficacy of earphones for 12- to 24-month-old children during visual reinforcement audiometry.

OBJECTIVE: Efficacy of insert and supra-aural earphones during visual reinforcement audiometry (VRA) was investigated for 12- to 24-month-old children. DESIGN: VRA testing began in the soundfield and transitioned to either insert or supra-aural earphones. Audiologists recorded threshold estimates, participant behaviors, and an overall subjective rating of earphone acceptance. STUDY SAMPLE: One hundred and eighty-six 12- to 24-month-old children referred to the Department of Audiology at St. Louis Children's Hospital for a variety of reasons. RESULTS: Subjective ratings indicated high acceptance of insert earphones (84%) and supra-aural earphones (80%) despite negative behaviors. There was no significant difference in the number of threshold estimates based on earphone type for 12- to 17-month-old participants. Participants in the 18- to 24-month-old age group provided significantly more threshold estimates with insert earphones (mean = 5.3 threshold estimates, SD = 3.5) than with supra-aural earphones (mean = 2.9 threshold estimates, SD = 2.9). All seven participants who rejected earphone placement were successfully reconditioned for soundfield testing. CONCLUSIONS: Data support the use of insert earphones during VRA, especially with 18-to 24-month-old children, to obtain ear-specific information.

Do audiologic characteristics predict outcomes in children with unilateral hearing loss?

OBJECTIVE: To determine whether audiologic characteristics of unilateral hearing loss in children were associated with language, cognitive, or achievement scores. STUDY DESIGN: Case-control study SETTING: Pediatric otolaryngology ambulatory practice PATIENTS: Cases (n = 109) were children aged 6 to 12 years with permanent unilateral hearing loss; controls (n = 95) were siblings with normal bilateral hearing. INTERVENTIONS: Audiologic characteristics measured included side and severity of hearing loss and word recognition scores in quiet and in noise. MAIN OUTCOME MEASURES: Cognitive abilities were measured using the Wechsler Abbreviated Scale of Intelligence; reading, math, and writing achievement was measured with the Wechsler Individual Achievement Test - Second Edition - Abbreviated; and oral language skills were measured with the Oral Written and Language Scales. RESULTS: Children with unilateral hearing loss had worse verbal cognitive and oral language scores than children with normal hearing, but there were no differences in achievement scores. Children with profound unilateral hearing loss tended to have worse cognitive scores and had significantly lower oral language scores. Higher word recognition scores of the normal hearing ear in quiet were associated with higher cognitive, oral language, and reading achievement scores. Higher word recognition scores in noise were slightly correlated with higher oral language scores. CONCLUSION: As expected, children with unilateral hearing loss had worse language scores than their siblings with normal hearing, with trends toward worse cognitive scores. Children with profound unilateral hearing loss tended to have worse outcomes than children with normal hearing or less severe unilateral loss. However, there were no differences in outcomes between children with right or left unilateral hearing loss.

Phenotypic characteristics of early Wolfram syndrome.

BACKGROUND: Wolfram Syndrome (WFS:OMIM 222300) is an autosomal recessive, progressive, neurologic and endocrinologic degenerative disorder caused by mutations in the WFS1 gene, encoding the endoplasmic reticulum (ER) protein wolframin, thought to be involved in the regulation of ER stress. This paper reports a cross section of data from the Washington University WFS Research Clinic, a longitudinal study to collect detailed phenotypic data on a group of young subjects in preparation for studies of therapeutic interventions. METHODS: Eighteen subjects (ages 5.9-25.8, mean 14.2 years) with genetically confirmed WFS were identified through the Washington University International Wolfram Registry. Examinations included: general medical, neurologic, ophthalmologic, audiologic, vestibular, and urologic exams, cognitive testing and neuroimaging. RESULTS: Seventeen (94%) had diabetes mellitus with the average age of diabetes onset of 6.3 ± 3.5 years. Diabetes insipidus was diagnosed in 13 (72%) at an average age of 10.6 ± 3.3 years. Seventeen (94%) had optic disc pallor and defects in color vision, 14 (78%) had hearing loss and 13 (72%) had olfactory defects, eight (44%) had impaired vibration sensation. Enuresis was reported by four (22%) and nocturia by three (17%). Of the 11 tested for bladder emptying, five (45%) had elevated post-void residual bladder volume. CONCLUSIONS: WFS causes multiple endocrine and neurologic deficits detectable on exam, even early in the course of the disease. Defects in olfaction have been underappreciated. The proposed mechanism of these deficits in WFS is ER stress-induced damage to neuronal and hormone-producing cells. This group of subjects with detailed clinical phenotyping provides a pool for testing proposed treatments for ER stress. Longitudinal follow-up is necessary for establishing the natural history and identifying potential biomarkers of progression.

Audiologic and vestibular findings in Wolfram syndrome.

OBJECTIVES: Assessment of auditory and vestibular function in Wolfram Syndrome (WS) patients, using a standardized protocol. DESIGN: Prospective cohort study of 11 patients using otoscopic inspection, tympanometry, otoacoustic emissions, pure tones, speech in noise (SIN), the Speech Intelligibility Index, and rotational chair testing. RESULTS: Mean SNHL diagnosis was 7.3 years with 55% prevalence. Four patients with a Speech Intelligibility Index less than 0.75 (better ear) routinely used bilateral amplification devices. Two patients with normal-hearing sensitivity exhibited abnormal SIN scores. The only patient with significant vestibular dysfunction also had a distinctive low-frequency component to her SNHL. CONCLUSIONS: Hearing loss may occur earlier than previously suspected, and comprehensive testing including SIN testing may reveal deficits not apparent with pure-tone testing. Particular configurations of hearing loss may indicate a need for comprehensive vestibular assessment. Because SNHL can be the first symptom of WS, audiologists and otolaryngologists should be vigilant about referring patients with hearing loss for ophthalmologic examination.

Animated toys versus video reinforcement in 16-24-month-old children in a clinical setting.

PURPOSE: To compare the clinical efficacy of visual reinforcement audiometry (VRA) with animated toy animal reinforcement (AVRA) to the efficacy of VRA with video reinforcement (VVRA) in children age 16 to 24 months in a fast-paced medical practice. METHOD: The 145 participants (age 16 to 24 months) were referred by either their primary care physician or an otolaryngology practitioner (physician or nurse practitioner) for audiologic assessment. Children were assigned in a counterbalanced manner to either the AVRA or VVRA group. RESULTS: Significantly more threshold estimates were obtained with AVRA (M = 5.52) than with VVRA (M = 3.47). There were no significant differences in performance based on age, hearing status, or gender. CONCLUSIONS: Number and relative strength of the visual reinforcers used are posited as the main reasons for the disparate outcomes. Clinical practices that test large numbers of children with VRA would be well-served to have both AVRA and VVRA available to meet the needs of individual patients.

Unilateral hearing loss is associated with worse speech-language scores in children.

OBJECTIVE: To determine whether children with unilateral hearing loss (UHL) demonstrate worse language skills than their siblings with normal hearing, and whether children with UHL are more likely to receive extra assistance at school. PATIENTS AND METHODS: We conducted a case-control study of 6- to 12-year-old children with UHL compared with sibling controls (74 pairs, n = 148). Scores on the oral portion of the Oral and Written Language Scales (OWLS) were the primary outcome measure. Multivariable analysis was used to determine whether UHL independently predicted OWLS scores after we controlled for potential confounding variables. RESULTS: Children with UHL had worse scores than their siblings on language comprehension (91 vs 98; P = .003), oral expression (94 vs 101; P = .007), and oral composite (90 vs 99; P < .001). UHL independently predicted these OWLS scores when multivariable regression was used with moderate effect sizes of 0.3 to 0.7. Family income and maternal education were also independent predictors of oral expression and oral composite scores. No differences were found between children with right- or left-ear UHL or with varying severity of hearing loss. Children with UHL were more likely to have an individualized education plan (odds ratio: 4.4 [95% confidence interval: 2.0-9.5]) and to have received speech-language therapy (odds ratio: 2.6 [95% confidence interval: 1.3-5.4]). CONCLUSIONS: School-aged children with UHL demonstrated worse oral language scores than did their siblings with normal hearing. These findings suggest that the common practice of withholding hearing-related accommodations from children with UHL should be reconsidered and studied, and that parents and educators should be informed about the deleterious effects of UHL on oral language skills.

Late onset hearing loss: a significant complication of cancer survivors treated with Cisplatin containing chemotherapy regimens.

Cisplatin is a known ototoxic agent and has been associated with late onset hearing loss (LOHL) in children beyond completion of treatment. We completed a retrospective review of 160 patients yielding 59 who received cisplatin and had sufficient data to determine the presence of LOHL. LOHL was defined as a significant change in hearing thresholds 6 months past the last cisplatin therapy. A significant change was defined as a decrease of >15 dB in a frequency from 1 to 8 kHz in either ear, or a decrease of 10 dB at 2 or more frequencies in the same ear, compared with the previously entered audiogram. Hearing loss was classified using the Brock grading system for each ear. Of the 59 patients evaluated, 51% exhibited LOHL. Univariate analysis indicated LOHL was significantly associated with age of diagnosis (P=0.031), diagnosis of medulloblastoma (P=0.035), hearing aids (P=0.010), and cranial radiation (P=0.044), particularly to the posterior fossa (P=0.023). Multivariate analysis revealed only radiation to the posterior fossa (P=0.02) and the use of hearing aids (P=0.01) were significantly associated with LOHL. LOHL is a significant complication in childhood cancer survivors who receive cisplatin. Long-term audiologic monitoring after therapy is needed to identify the affected patients.

Hearing loss in pediatric oncology patients receiving carboplatin-containing regimens.

Carboplatin is a well-established chemotherapeutic agent used to treat a variety of pediatric malignancies. Platinum analogues such as cisplatin are known to be ototoxic, but little is known regarding the ototoxicity of carboplatin. We performed a single institution retrospective chart review of pediatric oncology patients who received platinum-containing regimens from 1993 to 2002. Ninety-nine patients with sufficient medical and audiologic data were identified. Significant hearing loss was defined as a Brock grade 1 or higher. The incidence was compared among 3 treatment groups (carboplatin only, carboplatin and cisplatin, and cisplatin only). Significant hearing loss in patients receiving carboplatin only was rare, observed in only 1/25 (4%) of patients. In contrast, 19/27 (70%) of patients receiving carboplatin and cisplatin possessed significant hearing loss, as did 27/47 (57%) of those patients receiving cisplatin only P<0.001. The difference in hearing loss could not be explained by different cumulative exposures of the platinum agents. Carboplatin, when used without cisplatin, is rarely associated with severe hearing loss, even at high cumulative doses.

Initial audiologic assessment of infants referred from well baby, special care, and neonatal intensive care unit nurseries.

PURPOSE: The purpose of the study was to evaluate the effectiveness of a 2-hr initial audiologic assessment appointment for infants referred from area universal newborn hearing screening (UNHS) programs to a clinical audiology department in an urban hospital. METHOD: A prospective auditory brainstem response (ABR)-based protocol, including clicks, frequency-specific tone bursts, and bone-conducted stimuli, was administered by 10 audiologists to 375 infants. Depending on the ABR findings, additional test options included distortion product otoacoustic emissions (DPOAEs), high-frequency tympanometry, and/or otologic examination. RESULTS: In 88% of the 2-hr test sessions, at least 4 ABR threshold estimates were obtained (i.e., bilateral clicks and either a 500- or 1000-Hz tone burst and a 4000-Hz frequency tone burst for the better ear). The incidence of hearing loss was significantly different across nursery levels: 18% for Level I (well baby), 29% for Level II (special care), and 52% for Level III (neonatal intensive care unit). Hearing loss type was defined at the initial assessment for 35 of the 51 infants with bilateral hearing loss based on bone-conduction ABR, latency measures, DPOAEs, high-frequency tympanometry, and/or otologic examination. CONCLUSIONS: Our findings indicate that a 2-hr test appointment is appropriate for all nursery levels to diagnose severity and type of hearing loss in the majority of infants referred from UNHS. Examination by an otolaryngologist within 24-48 hr further defines the hearing loss and facilitates treatment plans.

Hearing screening in the neonatal intensive care unit: follow-up of referrals.

PURPOSE: The goal of this study was to examine the rate of diagnostic testing after newborn hearing screening (NHS) referral, evaluate timeliness of follow-up, and evaluate the use of multilevel auditory brainstem response (ABR) in screening of high-risk infants. METHOD: Telephone interviews were conducted with parents of infants who had been admitted to a neonatal intensive care unit from 1999 to 2002 and referred on NHS. An ABR screen was combined with a multilevel ABR (40, 70, and 90 dB nHL) for referrals. RESULTS: Parents of 206 infants participated; 69% of the infants underwent diagnostic follow-up. Of those with follow-up, 37% had normal hearing, 38% had hearing loss, and parents were unsure of hearing test results for 25%. Follow-up by 6 months of age occurred for 13% in 1999, increasing to 31% by 2002. Infants who did not pass their screening in both ears had confirmed hearing loss in 56% vs. 25% in those who passed in 1 ear. Also, 67% of infants with bilateral pass levels of 90 dB nHL or more had confirmed hearing loss, vs. 32% in all others. CONCLUSIONS: Timely follow-up after NHS referral in our program has improved over time. Multilevel ABR may facilitate allocation of appropriate resources to track and ensure follow-up in infants at high risk for hearing loss.

Otologic and audiologic features of Nager acrofacial dysostosis.

OBJECTIVE: To describe the otologic and audiologic characteristics of pediatric patients with Nager acrofacial dysostosis. DESIGN: Retrospective case series. SETTING: Multidisciplinary clinic in a tertiary care children's hospital. SUBJECTS: Patients less than 18 years of age with Nager acrofacial dysostosis. METHODS: Nager syndrome is a mandibulofacial dysostosis associated with preaxial limb abnormalities and multiple craniofacial anomalies. Ten patients with Nager syndrome were reviewed. Relevant literature, 1966 to the present, was reviewed with the assistance of Medline. RESULTS: External and middle ear abnormalities are common in Nager syndrome. All non-atretic ears had significant difficulty with otitis media, requiring an average of two sets of tympanostomy tubes. Cholesteatoma was diagnosed in one patient. Pure conductive hearing loss was identified in eight patients with mixed hearing loss noted in two patients. Conductive hearing loss greater than 30 dB HL was noted in 90% (9/10) of patients, with 40% (4/10) having 55-70 dB HL loss. Although amplification was effective, results of surgical interventions to correct conductive hearing loss were inconsistent. Two patients with mixed hearing loss developed the sensorineural component in later childhood, indicating that progressive or fluctuating sensorineural hearing loss is also possible in this population. CONCLUSIONS: Pediatric patients with Nager acrofacial dysostosis exhibit conductive hearing loss due to middle and external ear pathology. Prolonged ventilation of the middle ear via tympanostomy tubes and amplification with hearing aids are often required. Some patients also demonstrate mixed hearing loss that may be progressive and should be monitored carefully. Early and aggressive management in a multidisciplinary team approach is recommended.