This Article  • References  • Full text PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

William B. Reed, MD; Vean M. Stone, MD; Elena Boder, MD; Leo Ziprkowski, MD

Arch Dermatol. 1967;95(5):456-461.

Abstract
Congenital deafness may be associated with hereditary disorders of keratinization. Pili torti, twisted hair, was first reported by Bjornstad with sensorineural deafness. We have found four similar patients with partial alopecia from pili torti. The inheritance appears to be autosomal dominant in character. Congenital deafness of the sensorineural type also has been noted with onychodystrophy, ainhum, and keratosis palmaris et plantaris.

Hereditary disorders with important dermatological neurological complications may show an associated deafness. These include Refsum's, Cockayne's, Hunter's, and Laurance-Moon-Bardet-Biedl syndromes, xeroderma pigmentosa, and hidrotic ectodermal dysplasia.

Congenital malformations sometimes from hereditary causes may show abnormal implantations of hair (Treacher-Collins syndrome), nail dystrophy (Turner's syndrome), and malformed ears with ear pits and sinus tracts. One patient with Klein-Waardenburg syndrome was noted to have malformed ears with postauricular sinus tracts.

Author Affiliations
Burbank, Calif; Riverside, Calif; Beverly Hills, Calif; Tel Aviv, Israel

From the Department of Dermatology, Long Beach (Calif) Veterans Administration Hospital. Dr. Reed is now at the University of California at Los Angeles Medical Center, Los Angeles.

Footnotes
Accepted for publication Jan 20, 1967.

Reprint requests to University of California at Los Angeles Medical Center, Los Angeles 91506 (Dr. Reed).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Congenital Deafness, Spiny Hyperkeratosis, and Universal Alopecia
Myers et al.
Arch Otolaryngol Head Neck Surg 1971;93:68-74.
ABSTRACT  

Generalized Spiny Hyperkeratosis, Universal Alopecia, and Deafness: A Previously Undescribed Syndrome
Morris et al.
Arch Dermatol 1969;100:692-698.
ABSTRACT  

Hereditary Congenital Deafness With Onychodystrophy
Goodman et al.
Arch Otolaryngol Head Neck Surg 1969;90:474-477.
ABSTRACT  

Sensorineural Deafness
Perlman
Arch Otolaryngol Head Neck Surg 1969;90:232-234.
ABSTRACT  

Familial Neural Hearing Loss and Atopic Dermatitis
Konigsmark et al.
JAMA 1968;204:953-957.
ABSTRACT