The Role of Cognitive Evaluation in Predicting Successful Audiometric Testing among Children.

OBJECTIVE: To determine the role of cognitive testing in predicting age-appropriate audiometric responses among children aged 30 to 42 months. STUDY DESIGN: Prospective. SETTING: Tertiary care audiology clinic. SUBJECTS AND METHODS: Subjects included primary English-speaking children aged 30 to 42 months. A certified pediatric audiologist performed the cognitive aspect of the Developmental Assessment of Young Children-Second Edition (DAYC-2). A second, blinded audiologist performed age-appropriate audiometry. The raw, age-equivalent, percentile, and standard DAYC-2 scores were compared by agreement between speech reception threshold (SRT) and pure tone average (PTA). Optimal DAYC-2 thresholds were also calculated for prediction of SRT-PTA agreement and assessed for sensitivity, specificity, and positive and negative predictive values. P < .05 was considered significant. RESULTS: Complete data were obtained from 37 children. The mean age was 34.9 months (95% CI, 33.5-36.2), and 15 (41%) were female. Among the 37 children, 24 (65%) and 13 (35%) underwent visual reinforcement audiometry and conditioned play audiometry, respectively. SRT-PTA agreement was seen in 32 (87%) tests. Mean DAYC-2 raw score grouped by SRT-PTA agreement was 39.4 versus 33.4 for nonagreement (2.8-9.3, P < .001). The mean age-equivalent score grouped by SRT-PTA agreement was 29.6 versus 23.0 for nonagreement (2.7-10.6, P = .002). Optimal cut points based on DAYC-2 scores achieved moderate overall prediction performance (area under the curve, 0.73-0.77) with a positive predictive value of 100%. CONCLUSION: The DAYC-2 is a useful screen to identify children likely to complete an age-appropriate audiogram.

SLC26A4 genotype, but not cochlear radiologic structure, is correlated with hearing loss in ears with an enlarged vestibular aqueduct.

OBJECTIVES/HYPOTHESIS: Identify correlations among SLC26A4 genotype, cochlear structural anomalies, and hearing loss associated with enlargement of the vestibular aqueduct (EVA). STUDY DESIGN: Prospective cohort survey, National Institutes of Health, Clinical Center, a federal biomedical research facility. METHODS: Eighty-three individuals, 11 months to 59 years of age, with EVA in at least one ear were studied. Correlations among pure-tone hearing thresholds, number of mutant SLC26A4 alleles, and the presence of cochlear anomalies detected by computed tomography or magnetic resonance imaging were examined. RESULTS: Linear mixed-effects model indicated significantly poorer hearing in ears with EVA in individuals with two mutant alleles of SLC26A4 than in those with EVA and a single mutant allele (P = .012) or no mutant alleles (P = .007) in this gene. There was no detectable relationship between degree of hearing loss and the presence of structural cochlear anomalies. CONCLUSIONS: The number of mutant alleles of SLC26A4, but not the presence of cochlear anomalies, has a significant association with severity of hearing loss in ears with EVA. This information will be useful for prognostic counseling of patients and families with EVA.