title: Audiological findings in children with mucopolysaccharidoses type i-iv.
abstract: OBJECTIVE
  The aim of our study is to reflect hearing impairment of 23children diagnosed with mucopolysaccharidosis (MPS) typeI, II, III and IV.
  
  
  METHODS
  Retrospective study of the clinical, audiological and treatment (medical vs surgical) findings of 23children diagnosed with MPS typeI, II, III or IV followed at a Tertiary Referral Hospital between 1997 and 2015.
  
  
  RESULTS
  Six cases of MPSI, 8 of MPSII, 4 of MPSIII and 5 of MPSIV 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 typesI 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 typesI and II. Periodic follow up of these patients is mandatory because of hearing impairment and consequences for their development and quality of life.
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España. Electronic address: mariafernandavargasg@gmail.com.
authors.forename: María F
authors.initials: MF
authors.lastname: Vargas-Gamarra
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España.
authors.forename: Carlos
authors.initials: C
authors.lastname: de Paula-Vernetta
authors.affiliation: Unidad de Nutrición y Metabolopatías Congénitas, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España.
authors.forename: Isidro
authors.initials: I
authors.lastname: Vitoria Miñana
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España.
authors.forename: Isabel
authors.initials: I
authors.lastname: Ibañez-Alcañiz
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España.
authors.forename: Laura
authors.initials: L
authors.lastname: Cavallé-Garrido
authors.affiliation: Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, España.
authors.forename: Agustín
authors.initials: A
authors.lastname: Alamar-Velazquez
mesh_terms: D002648:Child; D005260:Female; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D008297:Male; D009083:Mucopolysaccharidoses / Q000150:complications*; D012189:Retrospective Studies
keywords: Hearing loss; Hipoacusia; Mucopolisacaridosis; Mucopolysaccharidosis; Otitis