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.

OBJETIVO: verificar a influência do tipo de transdutor na quantidade de mascaramento necessária para obtenção de limiares por vias aérea e óssea em indivíduos com deficiência auditiva condutiva e mista. MÉTODO: foram avaliados 16 indivíduos com presença de deficiência auditiva condutiva ou mista bilateral simétrica com gap aéreo-ósseo de no mínimo 15dBNA. Os indivíduos foram submetidos à anamnese, meatoscopia, medidas de imitância acústica, audiometria tonal liminar e audiometria vocal, realizadas com fones supra-aurais TDH-39 e com os fones de inserção ER-3A. RESULTADOS: não houve diferença estatisticamente significante entre as orelhas independentemente do tipo de transdutor. A quantidade de mascaramento utilizada com o fone de inserção ER-3A para testar a via aérea, nas frequências de 250Hz e 500Hz, foi menor do que a utilizada com o fone supra-aural, com significância estatística. CONCLUSÕES: há influência do tipo de transdutor na quantidade de mascaramento utilizada para obtenção dos limiares de via aérea e via óssea, principalmente nas frequências baixas, sendo que a quantidade de mascaramento com o fone de inserção é menor tanto para testar a via aérea como a via óssea.

PURPOSE: this study aimed at examining the influence of transducer's type in the amount of masking needed to obtain air and bone conducted thresholds in subjects with conductive and mixed hearing losses. METHOD: 16 patients with symmetric bilateral conductive hearing deficiency or mixed hearing loss with air-bone gap of at least 15dBNA were examined. The subjects underwent clinical history, otoscopy and acoustic immittance measures (tympanometry and acoustic reflex), and subsequently, pure tone and speech audiometry (SRT and WRS). Pure tone threshold and speech audiometry were performed both with supra-aural TDH-39 and insertion earphones ER-3A. RESULTS: there was no statistically significant difference between the ears regardless of the type of transducer. The amount of masking used with the insertion earphone ER-3A in order to test the air thresholds, in the frequencies of 250Hz and 500Hz, was lower than the one used with supra-aural headset with statistical significance. CONCLUSIONS: transducer's type influences in the amount of masking used in order to obtain air and bone conducted thresholds, mainly in the low frequencies, whereas the amount of masking with the insertion phone is smaller, both for testing the air as well as the bone thresholds.

OBJETIVO: Determinar a eficácia dos fones de inserção em casos de suspeita de colabamento de meato acústico externo. MÉTODOS: Vinte e um idosos com intervalo aéreo-ósseo superior a 10 dBNA nas frequências de 3 kHz e/ou 4 kHz foram submetidos à anamne se, audiometria tonal liminar, logoaudiometria e imitanciometria. A audiometria tonal e a logoaudiometria foram realizadas com os fones supra-aurais e com fones de inserção. RESULTADOS: Verificou-se que os limiares auditivos por via aérea obtidos com os fones de inserção foram menores em todas as frequências com exceção de 1 kHz. O intervalo aéreo-ósseo também foi menor com o fone de inserção, havendo um acoplamento aéreo-ósseo com este transdutor. CONCLUSÃO: Os fones de inserção ER-3A possibilitam melhora significativa nos limiares de audibilidade por via aérea em idosos, diminuindo ou até eliminando o intervalo aéreo-ósseo, sobretudo nas frequências altas, nos casos de colabamento do meato acústico externo.

PURPOSE: To determine the efficacy of insert earphones in cases of collapsed external auditory canals. METHODS: Twenty-one elderly individuals with air-bone gaps greater than 10 dBHL at frequencies of 3 kHz and/or 4 kHz underwent anamnesis, pure tone audiometry, speech audiometry, and immittance test. Pure tone audiometry and speech audiometry were performed with both supra-aural and insert earphones. RESULTS: Air conduction thresholds were lower with insert earphones at all frequencies except 1 kHz. The magnitude of the air-bone gap was also lower with the insert earphones, and there was a air-bone coupling with this transducer. CONCLUSION: Insert earphones ER-3A allowed significant improvement in air conduction hearing thresholds in elderly individuals, reducing or even eliminating the air-bone gap in cases of collapsed external auditory canals, especially at higher frequencies.

TEMA: audição e mitocondriopatia. PROCEDIMENTOS: este relato de caso teve como objetivo descrever os resultados da avaliação audiológica de duas crianças atendidas no ambulatório de audiologia clínica da Universidade Federal de São Paulo - Escola Paulista de Medicina (UNIFESP/EPM), com diagnóstico de doença mitocondrial, correlacionando os achados desta avaliação com a fisiopatologia subjacente. As crianças deste estudo, filhas de pais consangüíneos, foram encaminhadas para o ambulatório de audiologia clínica da UNIFESP-EPM pelo ambulatório de doenças metabólicas da mesma instituição em março de 2006 para realizar avaliação audiológica completa. As crianças foram submetidas à audiometria tonal, audiometria vocal, observação comportamental, medidas da imitância acústica e emissões otoacústicas. RESULTADOS: as avaliações comportamental e fisiológica revelaram que as crianças apresentam deficiência auditiva neurossensorial de grau leve a moderadamente severo e presença de sobressalto com ausência de habituação, indicando assim, comprometimento auditivo central. CONCLUSÃO: estes relatos de casos ressaltam a importância de ser considerada a associação entre os fatores de risco para deficiência auditiva no planejamento da reabilitação dos pacientes.

BACKGROUND: hearing and mitochondriopathy. PROCEDURE: this case report aims at describing audiological evaluation results of two children diagnosed with mitochondrial disease referred to Clinical Audiology Out-patient Clinic of UNIFESP/EPM. Children were referred to audiology department from metabolic diseases out-patient clinic of UNIFESP. Both have undergone pure tone audiometry, behavioral observation, immittance measures and otoacoustic emissions. RESULTS: either behavioral or physiological evaluations revealed abnormal results evidencing mild to moderate-to-severe sensorineural hearing loss and presence of startle reflex without habituation, indicating central auditory involvement. CONCLUSION: these case reports reinforce the importance of considering the association of risk factor for hearing loss in the rehabilitation planning of these patients.