Audiologic results in patients with Moebiüs sequence

SummaryObjectiveMoebiüs sequence is a pathology not very well understood regarding to the hearing status. The main goal of this study was to describe the audiologic findings in children and adolescent who carry Moebiüs sequence.MethodParticipated in this study 17 children and adolescent, with age ranging from 3 to 13 years old. Prior to the testing, the family answered an interview. It was realized external auditory canal inspection, and the hearing testing (auditory instrumentation, pure tone audiometry, speech audiometry, immittanciometric measures, and otoacoustic emissions) on the participants.ResultsThe auditory instrumentation evaluation (n = 6) was present in all participants. The pure tone audiometry presented normal hearing levels in 75.0% of the tested ears, one ear with conductive hearing loss, two ears with sensory neural hearing loss, and one ear with mixed hearing loss. The tympanometric measures showed Type A tympanograms in 63.0% of the ears, Type B in 11.1%, Type C in 18.5%, and Type As in 7.4%. T the acoustic reflexes measure showed contralateral acoustic reflexes present in 50.0% of the ears, and ipsilateral acoustic reflexes present in 34.6% ears. The OAE results showed presence in 73.0%, for the TOAE, and 76.9% for the DPOAE.ConclusionThese results support the idea that there is no audiologic pattern for conductive hearing loss. The majority of the participants presented hearing in the normal range. Care should be taken in drawing conclusions regarding to auditory status of the individual with Moebiüs sequence, but what can be said is that not always those individuals present hearing loss.