2018
DOI: 10.6061/clinics/2018/e523
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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 audito… Show more

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Cited by 14 publications
(17 citation statements)
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“…A recent comprehensive study of patients with MPS including 10 MPS I participants used different audiometry procedures, depending on age and cognitive function [48]. Hearing in children aged 6 months to 2 years was tested by visual reinforcement audiometry (VRA), whereas children aged 2-6 years underwent conditional tonal play audiometry and individuals aged 7 and older were tested through conventional tonal audiometry.…”
Section: Diagnostics For Auditory Manifestationsmentioning
confidence: 99%
“…A recent comprehensive study of patients with MPS including 10 MPS I participants used different audiometry procedures, depending on age and cognitive function [48]. Hearing in children aged 6 months to 2 years was tested by visual reinforcement audiometry (VRA), whereas children aged 2-6 years underwent conditional tonal play audiometry and individuals aged 7 and older were tested through conventional tonal audiometry.…”
Section: Diagnostics For Auditory Manifestationsmentioning
confidence: 99%
“…Hearing loss is common in both the severe and attenuated phenotypes [ 49 ]. Reported rates of hearing loss range from 76.2% to 100% [ 10 , 20 , 21 , 22 ]. Hearing loss can present as conductive, sensorineural, or mixed [ 20 ].…”
Section: Mucopolysaccharidosis Type I (Hurler Syndrome)mentioning
confidence: 99%
“…Hearing loss can present as conductive, sensorineural, or mixed [ 20 ]. Severity ranges from mild to severe, with mild and moderately severe being most common [ 10 ]. Chronic otitis media is also common, with an estimated rate of 89.1% [ 22 ].…”
Section: Mucopolysaccharidosis Type I (Hurler Syndrome)mentioning
confidence: 99%
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“…Progressive hearing loss is a common feature of all MPS disorders, and is mainly due to recurrent otitis media, middle ear effusion, ossicle deformity, inner ear (cochlea) abnormalities and alterations in the auditory nerve [3,9]. While young children with MPS generally present with conductive hearing loss, mixed hearing loss or pure sensorineural hearing loss tends to develop later in life as part of the natural history of MPS.…”
Section: Hearing and Speechmentioning
confidence: 99%