Prevalence, risk factors, and audiological characteristics of auditory neuropathy.

OBJECTIVE: The objective of this study was to determine the prevalence, risk factors, and audiological characteristics of auditory neuropathy spectrum disorder (ANSD) in the pediatric population. DESIGN: A retrospective review of medical charts was conducted for children visiting two hospitals in Saudi Arabia. STUDY SAMPLE: Medical records of 1025 patients with sensorineural hearing loss (SNHL) were reviewed. We analyzed the databases for results of audiological examinations, risk factors, and outcomes of intervention including hearing aid (HA) and cochlear implantation (CI). RESULTS: Out of 1025 children with SNHL, 101 patients (9.85%) were identified to have ANSD. Audiological characteristics of the ANSD group revealed a severe-to-profound degree of hearing loss, all showed type A tympanogram and absent reflexes, absent auditory brainstem response (ABR) findings with present cochlear microphonic while otoacoustic emissions were absent in 54.5% of patients. The most prevalent risk factors for ANSD in this group were family history of hearing loss, consanguinity, hyperbilirubinemia, and low birth weight. Pure tone and speech detection thresholds improved significantly with CI compared to HA use in this sample of patients with ANSD. CONCLUSION: This study shows that ANSD is not extremely rare among Saudi children with severe to profound hearing loss, with a prevalence of 9.85%.

Closed-Set Speech Discrimination Tests for Assessing Young Children.

OBJECTIVE: The main objective of this study is to obtain data assessing normative scores, test-retest reliability, critical differences, and the effect of age for two closed-set consonant-discrimination tests. DESIGN: The two tests are intended for use with children aged 2 to 8 years. The tests were evaluated using normal-hearing children within the appropriate age range. The tests were (1) the closed-set consonant confusion test (CCT) and (2) the consonant-discrimination subtest of the closed-set Chear Auditory Perception Test (CAPT). Both were word-identification tests using stimuli presented at a low fixed level, chosen to avoid ceiling effects while avoiding the use of background noise. Each test was administered twice. RESULTS: All children in the age range 3 years 2 months to 8 years 11 months gave meaningful scores and were able to respond reliably using a computer mouse or a touch screen to select one of four response options displayed on a screen for each trial. Assessment of test-retest reliability showed strong agreement between the two test runs (interclass correlation ≥ 0.8 for both tests). The critical differences were similar to those for other monosyllabic speech tests. Tables of these differences for the CCT and CAPT are provided for clinical use of the measures. Performance tended to improve with increasing age, especially for the CCT. Regression equations relating mean performance to age are given. CONCLUSIONS: The CCT is appropriate for children with developmental age in the range 2 to 4.5 years and the CAPT is appropriate as a follow-on test from the CCT. If a child scores 80% or more on the CCT, they can be further tested using the CAPT, which contains more advanced vocabulary and more difficult contrasts. This allows the assessment of consonant perception ability and of changes over time or after an intervention.