Audiometric evaluation in individuals with mucopolysaccharidosis
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 I...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Elsevier España
2018-12-01
|
| Series: | Clinics |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322018000100297&lng=en&tlng=en |
| _version_ | 1828295760685301760 |
|---|---|
| author | Marcela Rosana Maia da Silveira Ana Karina Lima Buriti Ana Maria Martins Daniela Gil Marisa Frasson de Azevedo |
| author_facet | Marcela Rosana Maia da Silveira Ana Karina Lima Buriti Ana Maria Martins Daniela Gil Marisa Frasson de Azevedo |
| author_sort | Marcela Rosana Maia da Silveira |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T12:00:27Z |
| format | Article |
| id | doaj.art-74e87f1bf9654f65a6e2ccb5a054206c |
| institution | Directory Open Access Journal |
| issn | 1980-5322 |
| language | English |
| last_indexed | 2024-04-13T12:00:27Z |
| publishDate | 2018-12-01 |
| publisher | Elsevier España |
| record_format | Article |
| series | Clinics |
| spelling | doaj.art-74e87f1bf9654f65a6e2ccb5a054206c2022-12-22T02:47:47ZengElsevier EspañaClinics1980-53222018-12-0173010.6061/clinics/2018/e523S1807-59322018000100297Audiometric evaluation in individuals with mucopolysaccharidosisMarcela Rosana Maia da SilveiraAna Karina Lima BuritiAna Maria MartinsDaniela GilMarisa Frasson de AzevedoOBJECTIVES: 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322018000100297&lng=en&tlng=enMucopolysaccharidosisHearingHearing LossChild |
| spellingShingle | Marcela Rosana Maia da Silveira Ana Karina Lima Buriti Ana Maria Martins Daniela Gil Marisa Frasson de Azevedo Audiometric evaluation in individuals with mucopolysaccharidosis Clinics Mucopolysaccharidosis Hearing Hearing Loss Child |
| title | Audiometric evaluation in individuals with mucopolysaccharidosis |
| title_full | Audiometric evaluation in individuals with mucopolysaccharidosis |
| title_fullStr | Audiometric evaluation in individuals with mucopolysaccharidosis |
| title_full_unstemmed | Audiometric evaluation in individuals with mucopolysaccharidosis |
| title_short | Audiometric evaluation in individuals with mucopolysaccharidosis |
| title_sort | audiometric evaluation in individuals with mucopolysaccharidosis |
| topic | Mucopolysaccharidosis Hearing Hearing Loss Child |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322018000100297&lng=en&tlng=en |
| work_keys_str_mv | AT marcelarosanamaiadasilveira audiometricevaluationinindividualswithmucopolysaccharidosis AT anakarinalimaburiti audiometricevaluationinindividualswithmucopolysaccharidosis AT anamariamartins audiometricevaluationinindividualswithmucopolysaccharidosis AT danielagil audiometricevaluationinindividualswithmucopolysaccharidosis AT marisafrassondeazevedo audiometricevaluationinindividualswithmucopolysaccharidosis |