Meleca Joseph B, Stillitano Gina, Lee Maxwell Y, Lyle Whitney, Carol Liu Yi-Chun, Anne Samantha
Cleveland Clinic, Head and Neck Institute, USA.
Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
Int J Pediatr Otorhinolaryngol. 2020 Feb;129:109757. doi: 10.1016/j.ijporl.2019.109757. Epub 2019 Oct 31.
Auditory Neuropathy Spectrum Disorder (ANSD) is characterized by hearing loss ranging from normal to profound. Additionally, results are confounded by commonly fluctuating hearing thresholds in ANSD. As such, we sought to evaluate results of audiometric testing on children with ANSD and the impact of age and time on testing results.
Retrospective chart review on children <18 years of age diagnosed with ANSD at two tertiary care academic institutions. Data analyzed included initial audiogram with speech detection thresholds (SDT) and pure tone averages (PTA) as well as most recent unaided audiogram SDT and PTA.
75 ANSD patients were analyzed, of which 32 (42.7%) were female. Bilateral and unilateral ANSD was seen in 55 (73.3%) and 20 (26.7%), respectively. A total of 130 ears with ANSD were assessed with 80 (61.5%) meeting inclusion criteria. Of these patients, the median age in years at first audiogram and most recent audiogram were 1.94 (0.45-13.68) and 4.22 (0.97-14.61), respectively. The median ages at which an SDT and PTA could first be acquired on the audiogram in ANSD patients were 1.94 (0.50-13.68) and 2.86 (0.45-13.68), respectively. The average SDT/PTA at the initial and most recent audiogram were 47.5/45.7 and 49.4/53.0 dB, respectively. SDT to PTA within the same audiogram exhibited strong correlation (r = 0.82, p < 0.001). Similarly, comparison of initial SDT to SDT at a later time interval showed strong correlation (r = 0.73, p < 0.001). SDT and PTA at initial audiogram and PTA at later time demonstrated lower correlation but was still statistically significant (r = 0.49, p < 0.009 and r = 0.51, p < 0.044, respectively). Individual PTA was associated with age (r = -0.56, p < 0.001).
SDT and PTA within the same audiogram and initial SDT to SDT acquired at a later time correlate strongly in audiometric testing in children with ANSD. Although not as strong, initial SDT and PTA still correlate with PTA at a later time interval. These findings suggest that audiometric results yielding a reliable SDT and frequency specific information necessary to calculate the PTA is not typically obtained until 2-3 years old. However, once this information is obtained, the child's hearing is fairly stable but may fluctuate over time.
听觉神经病谱系障碍（ANSD）的特征是听力损失范围从正常到重度。此外，ANSD中常见的听力阈值波动会混淆检测结果。因此，我们试图评估ANSD患儿的听力测试结果以及年龄和时间对测试结果的影响。
对两家三级医疗学术机构中诊断为ANSD的18岁以下儿童进行回顾性病历审查。分析的数据包括初始听力图的言语检测阈值（SDT）和纯音平均值（PTA）以及最近的未助听听力图的SDT和PTA。
分析了75例ANSD患者，其中32例（42.7%）为女性。双侧和单侧ANSD分别见于55例（73.3%）和20例（26.7%）。共评估了130只患有ANSD的耳朵，其中80只（61.5%）符合纳入标准。在这些患者中，首次听力图和最近听力图的年龄中位数分别为1.94岁（0.45 - 13.68岁）和4.22岁（0.97 - 14.61岁）。ANSD患者听力图上首次获得SDT和PTA的年龄中位数分别为1.94岁（0.50 - 13.68岁）和2.86岁（0.45 - 13.68岁）。初始和最近听力图的平均SDT/PTA分别为47.5/45.7和49.4/53.0 dB。同一听力图内的SDT与PTA呈现出强相关性（r = 0.82，p < 0.001）。同样，初始SDT与稍后时间间隔的SDT比较显示出强相关性（r = 0.73，p < 0.001）。初始听力图的SDT和PTA与稍后时间的PTA相关性较低，但仍具有统计学意义（分别为r = 0.49，p < 0.009和r = 0.51，p < 0.044）。个体PTA与年龄相关（r = -0.56，p < 0.001）。
在ANSD患儿的听力测试中，同一听力图内的SDT和PTA以及稍后时间获得的初始SDT与SDT之间具有强相关性。虽然相关性没有那么强，但初始SDT和PTA与稍后时间间隔的PTA仍具有相关性。这些发现表明，通常在2至3岁之前无法获得产生可靠SDT和计算PTA所需的频率特异性信息的听力测试结果。然而，一旦获得这些信息，儿童的听力相当稳定，但可能会随时间波动。
