Audiometric evaluation in individuals with mucopolysaccharidosis
Clinics
;
73: e523, 2018. tab, graf
Article
in English
| LILACS
| ID: biblio-974936
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Auditory Threshold
/
Mucopolysaccharidoses
/
Hearing Loss
Type of study:
Diagnostic study
/
Observational study
/
Prevalence study
/
Risk factors
Limits:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
English
Journal:
Clinics
Journal subject:
Medicine
Year:
2018
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Federal de Sao Paulo (UNIFESP)/BR
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