title: Permanent t-tube insertion in two patients with Hurler's syndrome.
abstract: Hearing loss in mucopolysaccharidosis is usually both conductive and sensorineural. The conductive component is attributable to serous otitis media secondary to dysfunction of the eustachian tube and chronic thickening of the mucosa of the middle ear. The conductive component may persist after myringotomy and insertion of short-term or long-term ventilation tubes. In Hurler's syndrome, death usually occurs in the first decade of life. In our study, we present two cases, a three-year-old girl and a four-year-old boy, who were diagnosed with Hurler's syndrome. Both children have a history of otitis media with effusion requiring repeated short-term ventilation tube insertions that were unsuccessful. Permanent t-tubes were inserted in both cases. Results showed an approximate 20 dB improvement in hearing sensitivity postoperatively for each patient.
authors: Council of Forensic Medicine, ENT Department, Istanbul, Turkey. fatihoghan@hotmail.com
authors: Fatih
authors: F
authors: Oghan
authors: Ugur
authors: U
authors: Harputluoglu
authors: Ender
authors: E
authors: Guclu
authors: Ali
authors: A
authors: Guvey
authors: Nursen
authors: N
authors: Turan
authors: Ozcan
authors: O
authors: Ozturk
mesh_terms: D000158:Acoustic Impedance Tests; D002675:Child, Preschool; D018450:Disease Progression; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008876:Middle Ear Ventilation / Q000295:instrumentation / Q000379:methods*; D009083:Mucopolysaccharidoses / Q000150:complications; D008059:Mucopolysaccharidosis I / Q000601:surgery*; D010034:Otitis Media with Effusion / Q000150:complications / Q000601:surgery*; D011474:Prosthesis Design; D012720:Severity of Illness Index