By James B. Snow John Jacob Ballenger
Univ. of Pennsylvania, Philadelphia. finished connection with the present wisdom within the fields of otology/neurotology, rhinology, facial plastic and reconstructive surgical procedure, and head and neck surgical procedure. Stresses the $64000 function of molecular biology within the pathogenesis of sickness. for college kids, citizens, fellows, and practitioners. past version: c1996.
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The axons, which are fine axons, converge with the olivocochlear bundle as the vestibular root is reached in the brainstem. They travel with the olivocochlear bundle efferents through the vestibular nerve to the point where the vestibular ganglia are reached. At this point, the vestibular efferents diverge from the parent efferent bundle to break up into fascicles and individual axons that then disperse throughout the ampullary and macular nerve branches. They branch and ramify richly along their course within the nerve trunks and after penetration of the basement membrane in the neuroepithelium of the vestibular sense organs.
The middle ear cleft is commonly hypoplastic in BOR syndrome, as can be the middle ear ossicles. In the inner ear, aplasia or hypoplasia of the cochlea has been reported as a BOR phenotype (Figure 2–9). The vestibular portions of the inner ear similarly show absent or malformed semicircular canals. 16,19 The spectrum of malformations seen in BOR syndrome suggests a defect in the EYA1 molecular pathway occurring sometime between the fourth and the tenth week of gestation. 33 At later embryonic time points, Eya1 is expressed along the neuroepithelium that eventually gives rise to key cochlear structures, including the inner and outer hair cells.
Formation of the ear canal is typically complete by the twenty-eighth week of gestation (C). Note that the medial-most ectoderm and endoderm of the tubotympanic recess interface in such a way that these two layers combine to form the tympanic membrane (C). Proliferation of these ectodermal cells generates a meatal “plug” that expands medially. Resorption of cells in the center of this meatal plug leaves a hollow tube-like structure representing the external meatus and ear canal. Failure of this canalization process results in aural atresia.