Hearing loss is a prominent feature in multiple genodermatoses. Underappreciation of auditory deficits can misdirect proper diagnosis by the treating dermatologist. This review reviews the anatomic, developmental, and embryologic aspects that characterize the ear and summarizes genodermatoses that have aberrant auditory findings. The latter are classified into neural crest, metabolic, pigmentary, craniofacial, and a miscellaneous category of disorders lacking specific cutaneous findings. The algorithms provided in this review enable treating dermatologists to better recognize and manage genodermatoses with ear involvement.
Keywords: cutaneous manifestations; deafness; genodermatoses; inherited skin disorders; syndromic hearing loss.
Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.