Otologic manifestations of Hunter syndrome and their relationship with speech development

Audiology & Neuro-otology
Yang-Sun ChoDong-Kyu Jin

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.

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Citations

Sep 26, 2013·Medicina clínica·Encarna Guillén-NavarroUNKNOWN grupo de trabajo Hunter España
Sep 14, 2010·International Journal of Pediatric Otorhinolaryngology·Mahmood F Bhutta
Aug 1, 2012·American Journal of Medical Genetics. Part a·Sung Hwa HongDong-Kyu Jin
Jul 16, 2011·Acta otorrinolaringológica española·Saturnino SantosM Jesús Domínguez
Apr 27, 2016·Molecular Genetics and Metabolism Reports·Elsa G ShapiroChester B Whitley
Jun 15, 2016·European Journal of Internal Medicine·John MitchellKlane White
Nov 18, 2018·Italian Journal of Pediatrics·Pier Marco BianchiSilvano Vitale
Apr 21, 2020·Orphanet Journal of Rare Diseases·Roberto GiuglianiMaurizio Scarpa
Aug 12, 2021·International Archives of Otorhinolaryngology·Cibele Gomes BicalhoAngelina Xavier Acosta

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