A importância da avaliação otorrinolaringológica de pacientes com mucopolissacaridose

Bicalho, Cibele Gomes; Rezende, Morena Morais; Nogueira, Ana Maria Carrilho Moinhos; Paulon, Roberta Melo Calvoso; Acosta, Angelina Xavier

doi:10.1590/s1809-48722011000300004

Cited by 7 publications

(7 citation statements)

References 6 publications

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“…There was a high frequency of tympanic membrane retraction (33%). As a result of the data findings and the possibility of irreversible auditory impairments, this study suggests early hearing diagnosis by audiometry and the use of brainstem auditory evoked potentials and otoacoustic emissions for follow-up and intervention in this population 22.…”

Section: Discussionmentioning

confidence: 62%

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Audiometric evaluation in individuals with mucopolysaccharidosis

Silveira¹,

Buriti²,

Martins³

et al. 2018

Clinics

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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.

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Section: Discussionmentioning

confidence: 62%

“…The incidence in Brazil is not known, but it has been considered that the population selected for this study is large compared with other studies 21,22 that included a much smaller number of cases or reported isolated cases. Hearing loss was observed in 96.6% of the population evaluated in the present study.…”

Section: Discussionmentioning

confidence: 99%

Audiometric evaluation in individuals with mucopolysaccharidosis

Silveira¹,

Buriti²,

Martins³

et al. 2018

Clinics

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“…Atualmente, além da equipe multidisciplinar, já há uma terapia específica para os tipos I, II e VI, a terapia de reposição enzimática (TRE) que consiste na administração periódica, por via venosa, da enzima específica deficiente no paciente. Este tratamento vem proporcionando bons resultados na melhora clínica e da qualidade de vida (BICALHO et al, 2011).…”

Section: Métodosunclassified

“…É necessário considerar que a criança portadora mucopolissacaridose apresenta diversos sintomas e manifestações clínicas, que variam conforme a tipologia, mas no geral apresentam comprometimento no sistema esquelético e cardiopulmonar, a córnea, a pele, o fígado, o baço, o cérebro e as meninges. Os sintomas são multissistêmicos, de curso crônico e progressivo(BICALHO et al, 2011). serviços de especialidades médicas, no entanto apesar das manifestações clínicas evidentes e das avaliações por diferentes profissionais da saúde, foram muitas as dificuldades encontradas para desvendar a patologia de base.…”

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Trauma Torácico Transfixante Por Acidente Doméstico Na Infância: Relato De Caso

Câncio¹,

Magalhães²,

Arnaud³

et al. 2019

Novos Paradigmas De Abordagem Na Medicina Atual 4

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“…In scientific literature, it's been registered that patients with MPS frequently present audiological alterations occasioned by the pathological GAGs deposition on the post-nasal space, the auditory tube, and the middle ear. Thus, these patients frequently need to be submitted to the insertion of ventilation tubes due to secretory or acute otitis media with effusion 3,4 . Conductive hearing loss is usually secondary to recurrent infections of the upper respiratory tract and serous otitis media, or to a deformity of the bony ossicles, wherefore the use of ventilation tubes is made necessary 5 .…”

Section: Introductionmentioning

confidence: 99%

Audiological characteristics in mucopolysaccharidosis: a systematic literature review

et al. 2019

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Purpose: to describe the audiological characteristics of patients with mucopolysaccharidosis. Methods: after formulating the research question, three databases were considered for the search (Science Direct, Virtual Health Library and Web of Science); the following descriptors were used: mucopolysaccharidoses, hearing loss, and audiology. Articles were included that were original and complete, presenting audiological evaluation data in patients with mucopolysaccharidosis. Each study was classified according to the degree of recommendation and the level of scientific evidence, based on the criteria established by the Oxford Center for Evidence-based Medicine, and the results obtained from the evaluations were analyzed. Results: of the 499 articles found, eight met the inclusion criteria. Pure tone audiometry and tympanometry were performed in all studies. The incidence of hearing loss in patients with MPS ranged from 54% to 100% of the cases, with conductive hearing loss corresponding to 30% and 58.33%, mixed hearing loss corresponding to 28.5% and 66.66% and sensorineural hearing loss corresponding to less than 14%. None of the studies described the responses of otoacoustic emissions and auditory evoked potentials. Conclusion: the prevalence of hearing loss among individuals with MPS is higher than 54% of the cases, of which conductive and mixed are predominant.

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A importância da avaliação otorrinolaringológica de pacientes com mucopolissacaridose

Cited by 7 publications

References 6 publications

Audiometric evaluation in individuals with mucopolysaccharidosis

Audiometric evaluation in individuals with mucopolysaccharidosis

Trauma Torácico Transfixante Por Acidente Doméstico Na Infância: Relato De Caso

Audiological characteristics in mucopolysaccharidosis: a systematic literature review

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