Audiometric evaluation in individuals with mucopolysaccharidosis
Clinics
; 73: e523, 2018. tab, graf
Article
em En
| LILACS
| ID: biblio-974936
Biblioteca responsável:
BR1.1
ABSTRACT
OBJECTIVES:
To characterize the audiometric evaluation and acoustic immittance measures in different types of mucopolysaccharidosis.METHOD:
Fifty-three mucopolysaccharidosis patients were evaluated. The classification consisted of type I (Hurler syndrome, Hurler-Scheie and Scheie syndrome), type II (Hunter syndrome), type III (Sanfilippo syndrome), type IV (Morquio syndrome), and type VI (Maroteaux-Lamy syndrome). Immittance audiometry and play or conventional threshold tone audiometry were used to obtain auditory thresholds and were chosen according to the patient's chronological age and ability to understand/respond to the procedure. The findings were analyzed using descriptive statistics and considering the recommendations for research involving human beings contained in Resolution CNE N° 466/2012.RESULTS:
Fifty-one subjects (96.2%) had hearing loss, and the conductive type was the most frequent. Only two (3.8%) patients presented bilateral thresholds within normal limits, one with type IV mucopolysaccharidosis and the other with type VI. There were 11 individuals (20.8%) with mucopolysaccharidosis type I with mixed hearing loss, 9 (16.9%) individuals with type I with conductive hearing loss and 9 (16.9%) with type VI with conductive hearing loss. Mild hearing loss was most common (37.3%), followed by moderately severe hearing loss (36.3%). The type B tympanometric curve (80.4%) was the most frequent.CONCLUSIONS:
Most of the individuals with mucopolysaccharidosis types I, II, III, IV and VI presented mixed or conductive hearing losses of mild to moderately severe degree, type B tympanograms and an absence of contralateral acoustic reflexes.Palavras-chave
Texto completo:
1
Índice:
LILACS
Assunto principal:
Limiar Auditivo
/
Mucopolissacaridoses
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Perda Auditiva
Tipo de estudo:
Diagnostic_studies
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Observational_studies
/
Prevalence_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Clinics
Assunto da revista:
MEDICINA
Ano de publicação:
2018
Tipo de documento:
Article