Audiological findings in children with mucopolysaccharidoses type i-iv.

Autors de IIS La Fe

• Maria Fernanda Vargas Gamarra

  Autor

• Carlos De Paula Verneta

  Autor

• Isidro Vitoria Miñana

  Autor

• Isabel Ibañez Alcañiz

  Autor

• Laura Cavalle Garrido

  Autor

Autors aliens a IIS La Fe

• Alamar-Velazquez, A

Grups d'Investigació

• Biomedicina Molecular, Celular y Genómica

  

  Leader

  José María Millán Salvador

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.

Keywords

• Hearing loss; Hipoacusia; Mucopolisacaridosis; Mucopolysaccharidosis; Otitis