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.

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.

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.

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.

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.