6533b836fe1ef96bd12a1c1d

RESEARCH PRODUCT

Audiological Findings in Children With Mucopolysaccharidoses Type I–IV

María F. Vargas-gamarraIsabel Ibañez-alcañizIsidro Vitoria MiñanaLaura Cavallé-garridoCarlos De Paula-vernettaAgustín Alamar-velazquez

subject

congenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtyHearing lossbusiness.industryMucopolysaccharidosisnutritional and metabolic diseasesRetrospective cohort studyGeneral MedicineAudiologyTertiary referral hospitalmedicine.disease03 medical and health sciences0302 clinical medicineOtitisQuality of lifemedicinemedicine.symptomskin and connective tissue diseases030223 otorhinolaryngologybusiness030217 neurology & neurosurgery

description

Abstract Objective The aim of our study is to reflect hearing impairment of 23 children diagnosed with mucopolysaccharidosis (MPS) type I, II, III and IV. Methods Retrospective study of the clinical, audiological and treatment (medical vs surgical) findings of 23 children diagnosed with MPS type I, II, III and IV followed at a Tertiary Referral Hospital between 1997 and 2015. Results Six cases of MPS I, 8 of MPS II, 4 of MPS III and 5 of MPS IV were reviewed. 71.2% of patients had secretory otitis media (SOM) and 54% of patients had some type of hearing loss (HL). The behaviour of hearing loss was variable in each of the subgroups of MPS, finding greater involvement and variability in types I and II. Conclusions Children with MPS have a high risk of hearing loss. A significant percentage of transmissive HL progressing to mixed or sensorineural HL was observed. This was more common in types I and II. Periodic follow up of these patients is mandatory because of hearing impairment and consequences for their development and quality of life.

yearjournalcountryeditionlanguage
2017-09-01Acta Otorrinolaringologica (English Edition)
https://doi.org/10.1016/j.otoeng.2017.08.006