Results
Of the 42 MPS patients, 32 (76.2%) underwent surgery (Table 2), including adenotonsillectomy (20 patients, 47.6%), adenoidectomy only (0 patients, 0.0%), tonsillectomy only (2 patient, 4.8%), insertion of middle ear ventilation tubes (24 patients, 57.1%), tracheotomy (3 patients, 7.1%), and CO2 laser supraglottoplasty (3 patients, total 9 times, 7.1%). All patients had at least one ENT symptom (Table 3). We also found that 15 patients (35.7%) had a history of chronic and recurrent OME (≥ 5 episodes) in a year.
Hearing loss (demonstrated by pure tone audiometry) was noted in 34 patients (81.0%, 5 in MPS I, 14 in MPS II, 4 in MPS IIIB, 8 in MPS IVA and 3 in MPS VI). Nineteen patients (42.5%) presented with mixed type hearing loss, 8 patients (19.0%) had conductive hearing loss, and 7 patients (16.7%) had sensorineural hearing loss. There were 34 cases (81.0%) with adenoid hypertrophy (degree ≥ 2), and 34 cases (81.0%) with tonsillar hypertrophy (degree ≥ 1). Twenty patients had been underwent polysomnography and eleven of them (55.0%) were diagnosed of OSA. The AHI ranged from 17.6 to 83.1.
Thirty-two patients (76.2%) underwent surgical treatment. Figure 1 showed the comparison between ENT manifestations before and after surgery. According to the infection score system, improvements in the severity of respiratory symptoms and ENT infections were seen after surgery (mean infection score before surgery 5.8 ± 1.6 vs . 3.8 ± 0.9 after surgery, p <0.05). Nine patient (60.0%) still showed evidence of OSA after adenotonsillectomy due to macroglossia and dysmorphism. No patients had died at the time of writing.