PubMed · 12391380
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Ken Johkura. 2002-10-22. Midbrain deafness with normal brainstem auditory evoked potentials.. https://doi.org/10.1212/wnl.59.8.1293-a
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Otosclerosis is a primary osteodystrophy which affects a localized area within the human temporal bone. Hearing loss is the most functional deficit caused by otosclerosis. However, tinnitus and vestibular disorders are frequently reported by otosclerotic patients, especially in those patients with inner ear involvement. The best therapy in achieving a significant improvement is surgery (stapedoplasty). In most patients if the operation is not carried out for pure middle ear type, the hearing impairment can progress to high-degree hearing loss. Recently, guidelines for the treatment of otosclerosis reported the 20 dbHL threshold as the minimum air-bone gap in performing stapedoplasty. We believe that stapedoplasty is an effective procedure for selected patients affected by otosclerosis with an air-bone gap which is smaller than 20 dbHL. An operation in the earlier phases of the disease can arrest the progression of otosclerosis, preserve inner ear structures and provide a complete auditory recovery, with increased satisfaction of the patient.
Final Syllable Lengthening (FSL) has been extensively examined in infant vocalizations in order to determine whether its basis is biological or learned. Findings suggest there may be a U-shaped developmental trajectory for FSL. The present study sought to verify this pattern and to determine whether vocal maturity and deafness influence FSL. Eight normally hearing infants, aged 0;3 to 1;0, and eight deaf infants, aged 0;8 to 4;0, were examined at three levels of prelinguistic vocal development: precanonical, canonical, and postcanonical. FSL was found at all three levels suggesting a biological basis for this phenomenon. Individual variability was, however, considerable. Reduction in the magnitude of FSL across the three sessions provided some support for a downward trend for FSL in infancy. Findings further indicated that auditory deprivation can significantly affect temporal aspects of infant speech production.
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