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Vol. 68. Issue 5.
Pages 262-268 (September - October 2017)
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Vol. 68. Issue 5.
Pages 262-268 (September - October 2017)
Original article
DOI: 10.1016/j.otoeng.2017.08.006
Audiological Findings in Children With Mucopolysaccharidoses Type I–IV
Hallazgos audiológicos en niños con mucopolisacaridosis tipos I–IV
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María F. Vargas-Gamarraa,
Corresponding author
mariafernandavargasg@gmail.com

Corresponding author.
, Carlos de Paula-Vernettaa, Isidro Vitoria Miñanab, Isabel Ibañez-Alcañiza, Laura Cavallé-Garridoa, Agustín Alamar-Velazqueza
a Servicio de Otorrinolaringología, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, Spain
b Unidad de Nutrición y Metabolopatías Congénitas, Hospital Universitario y Politécnico La Fe, Universidad de Valencia, Valencia, Spain
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Table 1. Classification and Main Characteristics of the Mucopolysaccharidoses.
Table 2. Mucopolysaccharidose and Hearing Loss.
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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.

Keywords:
Mucopolysaccharidosis
Hearing loss
Otitis
Resumen
Objetivo

Revisión y descripción de la afectación otoaudiológica en el seguimiento de 23niños con diagnóstico de mucopolisacaridosis (MPS) tipo I, II, III y IV.

Métodos

Estudio retrospectivo de los hallazgos clínicos, audiológicos y tratamiento (médico y/o quirúrgico) de 23 niños con diagnóstico de MPS tipo I, II, III o IV en seguimiento en un hospital terciario entre 1997 y 2015.

Resultados

Seis casos de MPS I, 8 de MPS II, 4 de MPS III y 5 de MPS IV fueron revisados. Al inicio del seguimiento el 71,2% de los pacientes presentaban otitis media serosa (OMS) y el 54% de los casos presentaban algún tipo de hipoacusia. El comportamiento de la hipoacusia fue fluctuante en cada uno de los subgrupos de MPS, encontrando mayor afectación y variabilidad en los tipos I y II.

Conclusiones

Los niños afectos de MPS tienen un alto riesgo de hipoacusia, siendo MPS tipo I y II los casos con mayor porcentaje de afectación audiológica y con un comportamiento menos homogéneo, mostrando un importante porcentaje de hipoacusias transmisivas que progresan a componentes mixtos o neurosensoriales. Se requiere un seguimiento periódico dada la importante repercusión de esta patología en la calidad de vida y en el desarrollo de estos pacientes.

Palabras clave:
Mucopolisacaridosis
Hipoacusia
Otitis

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