Church Michael W, Parent-Jenkins Leslie, Rozzelle Arlene A, Eldis Frances E, Kazzi S Nadya J
Department of Obstetrics and Gynecology, Wayne State University School of Medicine, Detroit, Michigan, USA.
Pediatrics. 2007 Jun;119(6):e1351-60. doi: 10.1542/peds.2006-3009. Epub 2007 May 21.
Craniosynostosis is a devastating disorder characterized by premature closure of the cranial plates before or shortly after birth. This results in an abnormally shaped skull, face, and brain. Little is known about hearing disorders in such patients, and nothing has been published about their auditory brainstem responses. Our objective was to evaluate such patients for auditory brainstem response and hearing disorders with the long-term goal of improving patient evaluation and management.
We evaluated the auditory brainstem responses, hearing, and brain images of children with fibroblast growth factor receptor 2 craniosynostosis (n = 11).
Prolongation of the auditory brainstem response I-to-III interpeak latency was a frequent characteristic of fibroblast growth factor receptor 2 craniosynostosis, occurring in 91% of our patients. Prolongation of the III-to-V interpeak latency was an occasional characteristic, occurring in 27% of our patients. Whenever the I-to-III interpeak latency was prolonged, wave II was always abnormal. Associated morbidities included sensorineural hearing loss (27%), recurrent otitis media (100%), and Arnold-Chiari malformation (27%). Cranial decompression improved the interpeak latencies of 2 children.
These previously undocumented auditory brainstem response abnormalities reflect abnormal neural transmission, which could cause peripheral and central auditory processing disorders. We speculate that the major pathogenic basis of the I-to-III interpeak latency and wave II abnormalities is compression of the auditory nerve as it passes through the internal auditory meatus and posterior fossa, which would explain the auditory nerve hearing loss, tinnitus, and vertigo that affect these children. Awareness of these abnormalities could lead to important advancements in the auditory and neurosurgical assessment and management of this overlooked patient group. We provide recommendations for the improved assessment and management of these patients. In particular, we recommend that auditory brainstem response diagnostics become standard clinical care for this patient group as the best way to detect auditory nerve compression.
颅缝早闭是一种严重的疾病，其特征是颅骨板在出生前或出生后不久过早闭合。这会导致颅骨、面部和大脑形状异常。对于此类患者的听力障碍知之甚少，且尚无关于其听觉脑干反应的报道。我们的目的是评估此类患者的听觉脑干反应和听力障碍，长期目标是改善患者评估和管理。
我们评估了11例成纤维细胞生长因子受体2型颅缝早闭患儿的听觉脑干反应、听力和脑部影像。
听觉脑干反应I波至III波峰间潜伏期延长是成纤维细胞生长因子受体2型颅缝早闭的常见特征，在我们91%的患者中出现。III波至V波峰间潜伏期延长是偶尔出现的特征，在27%的患者中出现。每当I波至III波峰间潜伏期延长时，II波总是异常。相关疾病包括感音神经性听力损失（27%）、复发性中耳炎（100%）和阿诺德 - 奇亚里畸形（27%）。颅骨减压改善了2例患儿的峰间潜伏期。
这些先前未记录的听觉脑干反应异常反映了神经传导异常，可能导致外周和中枢听觉处理障碍。我们推测I波至III波峰间潜伏期和II波异常的主要致病基础是听神经在通过内耳道和后颅窝时受到压迫，这可以解释影响这些患儿的听神经听力损失、耳鸣和眩晕。认识到这些异常可能会在对这个被忽视的患者群体的听觉和神经外科评估及管理方面取得重要进展。我们提供了改善这些患者评估和管理的建议。特别是，我们建议听觉脑干反应诊断应成为该患者群体的标准临床护理，作为检测听神经受压的最佳方法。
