to report a rare or specific entity

Tapetoretinal degenerations are degenerative affections of the ocular fundus, of hereditary origin. The degenerative process found in the retina has, in many cases, a corresponding counterpart in other parts of the body. Combinations of retinal dystrophy, and for instance, deafness or mental retardation are well known and also about metabolic dyscrasia and CNS. But only few reports on the combination of retinal dystrophy and dermatological disorders have been found.

We have found cases of tapetoretinal degeneration combined with a rare form of hair disorder and deafness in females in an Iranian family.

. Since we have succeeded in finding a few similar cases in the literature we consider this apparently rare combination worth a somewhat closer analysis as it might constitute a specific entity.