Audiometric results of bilateral bone-anchored hearing aid application in patients with bilateral congenital aural atresia. 1998

K T van der Pouw, and A F Snik, and C W Cremers
Department of Otorhinolaryngology, University Hospital Nijmegen, The Netherlands.

The effect of bilateral application of bone-anchored hearing aids (BAHAs) was examined in terms of directional hearing and speech recognition in quiet and in noise in four patients with bilateral congenital atresia who, out of pure necessity, had been using a unilateral bone-conduction hearing aid since early life. This study comprised a prospective clinical evaluation in a single subject design; four patients with bilateral congenital atresia originating from the Nijmegen BAHA series participated. Three patients had Treacher Collins syndrome. All four patients had conductive, most probably, symmetrical, hearing loss. Recently these patients had applied for a second BAHA and were subsequently fitted bilaterally. With two BAHAs, all four patients showed significant improvement in sound localization. Also, speech perception in quiet showed significant improvement with bilateral application, and a significant improvement was found in speech perception in noise in three patients. These results suggest that patients with congenital conductive, symmetrical hearing loss will benefit from bilateral BAHAs.

UI MeSH Term Description Entries
D008297 Male Males
D008342 Mandibulofacial Dysostosis A hereditary disorder occurring in two forms: the complete form (Franceschetti's syndrome) is characterized by a slant of the palpebral fissures, COLOBOMA of the lower lid, MICROGNATHIA and hypoplasia of the ZYGOMATIC ARCHES, and CONGENITAL MICROTIA. It is transmitted as an autosomal trait. The incomplete form (Treacher Collins syndrome) is characterized by the same anomalies in less pronounced degree. It occurs sporadically, but an autosomal dominant mode of transmission is suspected. (Dorland, 27th ed) MFD1 Mandibulofacial Dysostosis,Treacher Collins Syndrome,Franceschetti-Zwahlen-Klein Syndrome,Mandibulofacial Dysostosis (MFD1),Treacher Collins-Franceschetti Syndrome,Dysostoses, MFD1 Mandibulofacial,Dysostoses, Mandibulofacial,Dysostoses, Mandibulofacial (MFD1),Dysostosis, MFD1 Mandibulofacial,Dysostosis, Mandibulofacial,Dysostosis, Mandibulofacial (MFD1),Franceschetti Zwahlen Klein Syndrome,Franceschetti-Zwahlen-Klein Syndromes,MFD1 Mandibulofacial Dysostoses,Mandibulofacial Dysostoses,Mandibulofacial Dysostoses (MFD1),Mandibulofacial Dysostoses, MFD1,Mandibulofacial Dysostosis, MFD1,Syndrome, Franceschetti-Zwahlen-Klein,Syndrome, Treacher Collins,Syndrome, Treacher Collins-Franceschetti,Syndromes, Franceschetti-Zwahlen-Klein,Syndromes, Treacher Collins-Franceschetti,Treacher Collins Franceschetti Syndrome,Treacher Collins-Franceschetti Syndromes
D008416 Mastoid The posterior part of the temporal bone. It is a projection of the petrous bone. Mastoid Foramen,Mastoid Bone,Mastoid Process,Bone, Mastoid,Foramen, Mastoid,Mastoid Bones,Mastoid Processes,Mastoids,Process, Mastoid
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009622 Noise Any sound which is unwanted or interferes with HEARING other sounds. Noise Pollution,Noises,Pollution, Noise
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D001844 Bone Conduction Transmission of sound waves through vibration of bones in the SKULL to the inner ear (COCHLEA). By using bone conduction stimulation and by bypassing any OUTER EAR or MIDDLE EAR abnormalities, hearing thresholds of the cochlea can be determined. Bone conduction hearing differs from normal hearing which is based on air conduction stimulation via the EAR CANAL and the TYMPANIC MEMBRANE. Bone Conduction Hearing,Conduction Hearing, Bone,Conduction, Bone,Hearing, Bone Conduction
D003463 Cues Signals for an action; that specific portion of a perceptual field or pattern of stimuli to which a subject has learned to respond. Cue
D004424 Ear Canal The narrow passage way that conducts the sound collected by the EAR AURICLE to the TYMPANIC MEMBRANE. Auditory Canal, External,External Acoustic Canal,External Acoustic Meatus,External Auditory Canal,External Ear Canal,Acoustic Canal, External,Acoustic Canals, External,Acoustic Meatus, External,Auditory Canals, External,Canal, Ear,Canal, External Ear,Canals, Ear,Canals, External Ear,Ear Canal, External,Ear Canals,Ear Canals, External,External Acoustic Canals,External Auditory Canals,External Ear Canals
D004432 Ear, Middle The space and structures directly internal to the TYMPANIC MEMBRANE and external to the inner ear (LABYRINTH). Its major components include the AUDITORY OSSICLES and the EUSTACHIAN TUBE that connects the cavity of middle ear (tympanic cavity) to the upper part of the throat. Tympanic Cavity,Tympanum,Middle Ear,Cavities, Tympanic,Cavity, Tympanic,Ears, Middle,Middle Ears,Tympanic Cavities,Tympanums

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