Frontonasal dysplasia: clinical evaluation on audiological and brainstem electrophysiological profiles.

Abstract:

UNLABELLED:Frontonasal dysplasia (FND) is a rare malformative complex affecting the frontal portion of the face, the eyes and the nose; it may occur singly or associated with other clinical signs. No systematic studies describing hearing in this condition were found. AIM:To evaluate hearing sensitivity and sound stimulus conduction from cochlea to brainstem in patients with clinical signs of FND. METHODS:21 patients with isolated or syndromic FND were submitted to a clinical (otological/vestibular antecedents and otoscopy) and instrumental (pure tone and speech audiometry, tympanometry and brainstem auditory evoked response) hearing evaluation. DESIGN:A clinical, cross-sectional observational prospective study. RESULTS:Hearing thresholds were normal in 15 (70%) patients, abnormal in 5 (25%), mostly with conductive hearing loss; one patient did not cooperate with testing. The tympanometric curve was type A in 30 (72%) ears, type C in 5 (12%), type As in 4 (9%) and type B in 3 (7%). The auditory brainstem response (ABR) showed no abnormalities. CONCLUSION:Patients with FND showed no abnormalities in the auditory system from cochlea to brainstem in this study. Mild conductive hearing loss found in some is probably related to cleft palate. Further evaluation of hearing pathways at higher levels is recommended.

authors

Antoneli MZ,Giacheti CM,Genaro KF,Zorzetto NL,Richieri-Costa A

doi

10.1590/s1808-86942011000500013

subject

Has Abstract

pub_date

2011-09-01 00:00:00

pages

611-5

issue

5

eissn

1808-8694

issn

1808-8686

pii

S1808-8694(15)30707-2

journal_volume

77

pub_type

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