Medical Scientist Training Program, Vanderbilt University School of Medicine, Nashville, TN.
Department of Hearing and Speech Sciences, Vanderbilt University Medical Center, Nashville, TN.
Vanderbilt Brain Institute, Vanderbilt University, Nashville, TN.
Frist Center for Autism and Innovation, Vanderbilt University, Nashville, TN.
Graduate Program in Biomedical Sciences, Vanderbilt University, Nashville, TN.
Vanderbilt Kennedy Center, Vanderbilt University Medical Center, Nashville, TN.
目的 听觉过敏是一种复杂且知之甚少的听觉障碍，其特征是对声音的耐受性降低，而这种水平不会给大多数人带来麻烦。最近，有人提出，对日常声音有反应而出现耳痛（称为
痛觉过敏
）的人在临床上可能与那些主要症状是感觉日常声音过大（称为
响度过敏）的人在临床上有所不同。
）。尽管存在这种理论上的区别，但没有直接比较这两种听觉过敏患者群体的实证研究。方法 使用来自多国患者登记处（Sanford Registry 的罕见病协调）的数据，我们检查了 243 名患有听觉过敏症的成年人样本中自我报告的人口统计数据、症状、合并症和对治疗的反应，其中 152 人被归类为患有基于报告的症状的疼痛听觉过敏。贝叶斯统计检验用于调查响度和疼痛听觉过敏患者之间是否存在组间差异。结果 患有疼痛听觉过敏症的个体表现出更严重的临床表型，报告了更高频率的暂时症状恶化（即“挫折”），随着时间的推移感知症状改善较少，更严重的合并头痛疾病，并减少声音治疗的益处。然而，这两种假设的听觉过敏亚型表现出更多的相似之处而不是差异，大多数症状和合并症在各组中同样普遍。通常观察到多种合并症，包括耳鸣、原发性头痛疾病、精神疾病和功能性躯体综合征。还经常报告对其他方式的感觉刺激不耐受。结论 虽然这项研究几乎没有提供证据表明响度和疼痛听觉过敏是病理生理学上不同的疾病，但我们的研究结果表明，以疼痛为主的表型可能是听觉过敏患者的一个有意义的预后标志物。这两种假设的听觉过敏亚型表现出更多的相似性而不是差异，大多数症状和合并症在各组中同样普遍。通常观察到多种合并症，包括耳鸣、原发性头痛疾病、精神疾病和功能性躯体综合征。还经常报告对其他方式的感觉刺激不耐受。结论 虽然这项研究几乎没有提供证据表明响度和疼痛听觉过敏是病理生理学上不同的疾病，但我们的研究结果表明，以疼痛为主的表型可能是听觉过敏患者的一个有意义的预后标志物。这两种假设的听觉过敏亚型表现出更多的相似性而不是差异，大多数症状和合并症在各组中同样普遍。通常观察到多种合并症，包括耳鸣、原发性头痛疾病、精神疾病和功能性躯体综合征。还经常报告对其他方式的感觉刺激不耐受。结论 虽然这项研究几乎没有提供证据表明响度和疼痛听觉过敏是病理生理学上不同的疾病，但我们的研究结果表明，以疼痛为主的表型可能是听觉过敏患者的一个有意义的预后标志物。通常观察到多种合并症，包括耳鸣、原发性头痛疾病、精神疾病和功能性躯体综合征。还经常报告对其他方式的感觉刺激不耐受。结论 虽然这项研究几乎没有提供证据表明响度和疼痛听觉过敏是病理生理学上不同的疾病，但我们的研究结果表明，以疼痛为主的表型可能是听觉过敏患者的一个有意义的预后标志物。通常观察到多种合并症，包括耳鸣、原发性头痛疾病、精神疾病和功能性躯体综合征。还经常报告对其他方式的感觉刺激不耐受。结论 虽然这项研究几乎没有提供证据表明响度和疼痛听觉过敏是病理生理学上不同的疾病，但我们的研究结果表明，以疼痛为主的表型可能是听觉过敏患者的一个有意义的预后标志物。
Purpose Hyperacusis is a complex and poorly understood auditory disorder characterized by decreased tolerance to sound at levels that would not trouble most individuals. Recently, it has been suggested that individuals who experience otalgia in response to everyday sounds (termed
pain hyperacusis
) may differ clinically from those whose primary symptom is the perception of everyday sounds as excessively loud (termed
loudness hyperacusis
). Despite this theoretical distinction, there have been no empirical studies directly comparing these two populations of hyperacusis patients. Method Using data from a multinational patient registry (the Coordination of Rare Diseases at Sanford Registry), we examined self-reported demographics, symptoms, comorbidity, and response to treatment in a sample of 243 adults with hyperacusis, 152 of whom were classified as having pain hyperacusis based on reported symptoms. Bayesian statistical tests were used to investigate both the presence and absence of group differences between patients with loudness and pain hyperacusis. Results Individuals with pain hyperacusis presented with a more severe clinical phenotype, reporting a higher frequency of temporary symptom exacerbations (i.e., "setbacks"), less perceived symptom improvement over time, more severe comorbid headache disorders, and reduced benefit from sound therapy. However, the two hypothesized hyperacusis subtypes exhibited more similarities than differences, with the majority of symptoms and comorbidities being equally prevalent across groups. Multiple comorbidities were commonly observed, including tinnitus, primary headache disorders, psychiatric disorders, and functional somatic syndromes. Intolerance of sensory stimuli in other modalities was also frequently reported. Conclusion Although this study provides little evidence that loudness and pain hyperacusis are pathophysiologically distinct conditions, our findings indicate that a pain-predominant phenotype may be a meaningful prognostic marker in patients with hyperacusis.
