APPARENTLY NEW SYNDROME OF SENSORINEURAL HEARING-LOSS, RETINAL-PIGMENT EPITHELIUM LESIONS, AND DISCOLORED TEETH

We report on a family with early-onset sensorineural hearing loss, abn ormal retinal pigment epithelium granularity, accumulation of creamy-w hite lesions at the level of the retinal pigment epithelium particular ly superior to the arcade, and selective discoloration (brown) of mola rs or canine deciduous teeth that follows an apparent autosomal recess ive inheritance pattern, This appears to be a new syndrome that can be distinguished from the known otodental, oculo-acoustic and flecked re tina syndromes by the occurrence of distinct dental and ocular abnorma lities, (C) 1998 Wiley-Liss, Inc.