The excessive storage of mucopolysaccharide in Hunter syndrome leads to various otologic manifestations. We interviewed 19 patients with Hunter syndrome to assess their otologic problems, and conducted audiologic tests and temporal bone CT. Patients with the intermediate or severe form exhibited severe speech delay by more than 2 years (12/14 patients). However, in patients with the mild form (5/5), speech development was not much disturbed (2/5), although otoscopic findings were similar. The hearing threshold determined by the auditory brainstem response differed significantly between the mild and intermediate/severe forms (p &lt; 0.05). Therefore, patients with the mild form may benefit from active otologic intervention such as VT insertion, amplification, and speech therapy.

Yang-Sun Cho

Joon Ho Kim

Tae Wook Kim

Soo Chan Chung

Son-A Chang

Dong-Kyu Jin

Audiology and Neurotology

“听力学和神经病学”为发表与听觉和前庭系统以及耳部疾病的基础科学和临床方面相关的最先进和最严谨的科学研究提供了一个论坛。该杂志寻求提交前沿研究，开辟新的和创新的研究领域，这可能会提高我们对听觉和前庭系统疾病患者的理解和治疗，他们的中枢连接和他们在中枢神经系统中的感知。除了原创论文外，该杂志还提供由该领域领先专家撰写的关于当前主题的受邀评论文章。该杂志对所有对听力学、耳科和神经病学、听觉神经科学和相关学科感兴趣的科学家和从业者都至关重要。

"Audiology and Neurotology" provides a cutting-edge academic forum for fundamental and clinical research related to the auditory and vestibular systems, as well as ear diseases. The journal aims to publish innovative studies that explore new research areas, enhancing our understanding and treatment of patients with auditory and vestibular system disorders, particularly regarding their central connections and perceptions within the central nervous system. In addition to original research articles, the journal features invited review articles written by leading experts in the field, discussing current topics of interest. This journal is essential for all scientists and practitioners interested in audiology, otology, neurology, auditory neuroscience, and related disciplines.

《Audiology and Neurotology》为听觉和前庭系统以及耳部疾病的基础科学和临床研究提供了一个前沿的学术论坛。该杂志致力于发表创新性研究，探索新的研究领域，以增进我们对听觉和前庭系统疾病患者的理解和治疗，特别是他们的中枢连接及其在中枢神经系统中的感知。此外，杂志还刊登由该领域顶尖专家撰写的受邀评论文章，讨论当前的研究主题。对于所有关注听力学、耳科、神经病学、听觉神经科学及相关学科的科学家和从业者来说，该杂志具有重要的学术价值。

Otologic Manifestations of Hunter Syndrome and Their Relationship with Speech Development

The hearing threshold determined by the auditory brainstem response differed significantly between the mild and intermediate/severe forms, and patients with the mild form may benefit from active otologic intervention such as VT insertion, amplification, and speech therapy.

Category	Quartile
医学	4
医学, 听力学与言语病理学	4
医学, 神经科学	4
医学, 耳鼻喉科学	4

Otologic Manifestations of Hunter Syndrome and Their Relationship with Speech Development

ivySCI AI Smartly Parses PDF, Answers Researchers' Questions, and Helps You Understand Papers in Seconds

Journal Info