1. Audiological Findings in Children With Mucopolysaccharidoses Type I–IV
- Author
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María F. Vargas-Gamarra, Isabel Ibañez-Alcañiz, Isidro Vitoria Miñana, Laura Cavallé-Garrido, Carlos de Paula-Vernetta, and Agustín Alamar-Velazquez
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,Pediatrics ,medicine.medical_specialty ,Hearing loss ,business.industry ,Mucopolysaccharidosis ,nutritional and metabolic diseases ,Retrospective cohort study ,General Medicine ,Audiology ,Tertiary referral hospital ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Otitis ,Quality of life ,medicine ,medicine.symptom ,skin and connective tissue diseases ,030223 otorhinolaryngology ,business ,030217 neurology & neurosurgery - 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.
- Published
- 2017
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