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Oculo-auditory Syndromes

Thomas R. Hedges Jr, MD
JAMA. 1985;254(23):3373-3374. doi:10.1001/jama.1985.03360230105036.
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This unique volume gives us the best understanding to date of the two higher sensory systems, the ocular and the auditory, which are very complex but have common characteristics. Contributors from the interrelated fields of basic science clearly point out the overlapping anatomy, embryology, and physiology, which are liable to produce complicated and rare clinical syndromes.

The common ectodermal origin of the eye and ear logically leads to anatomic similarities, especially as regards circulation and neurovegetative function. This developmental pattern is responsible for oculoauditory syndromes due to genetic defects affecting the neuroectoderm, including Usher's syndrome (retinitis pigmentosa and dysacousia), Alstrom's syndrome (retinitis pigmentosa, obesity, deafness, and diabetes mellitus), Norrie's disease (oculoacoustic cerebral degeneration), Refsum's disease (phytanic acid storage disease), and Konigsmark's syndrome (congenital deafness and progressive optic atrophy). Genetic enzymatic defects affecting mesenchymal tissues are illustrated by Goldenhar's syndrome (oculoauriculovertebral dysplasia) and Waardenburg's syndrome, among others.

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