title: Otologic manifestations of Hunter syndrome and their relationship with speech development.
abstract: 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 < 0.05). Therefore, patients with the mild form may benefit from active otologic intervention such as VT insertion, amplification, and speech therapy.
authors.affiliation: Department of Otorhinolaryngology - Head and Neck Surgery, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, South Korea.
authors.forename: Yang-Sun
authors.initials: YS
authors.lastname: Cho
authors.forename: Joon Ho
authors.initials: JH
authors.lastname: Kim
authors.forename: Tae Wook
authors.initials: TW
authors.lastname: Kim
authors.forename: Soo Chan
authors.initials: SC
authors.lastname: Chung
authors.forename: Son-A
authors.initials: SA
authors.lastname: Chang
authors.forename: Dong-Kyu
authors.initials: DK
authors.lastname: Jin
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004432:Ear, Middle / Q000473:pathology; D006311:Hearing Disorders / Q000209:etiology*; D006801:Humans; D007360:Intelligence; D007805:Language Development Disorders / Q000209:etiology* / Q000503:physiopathology; D007859:Learning Disabilities / Q000209:etiology; D008297:Male; D008416:Mastoid / Q000473:pathology; D016532:Mucopolysaccharidosis II / Q000235:genetics / Q000473:pathology / Q000503:physiopathology* / Q000523:psychology; D010641:Phenotype; D011446:Prospective Studies; D013065:Speech Intelligibility / Q000502:physiology*