A Rare Case of Epidermodysplasia Verruciformis with Non Syndromic Hearing Loss

Abstract

The onset of Non Syndromic Hearing Loss (NSHL) typically occurs without any other symptoms and can vary from person to person, even within the same family. Hearing loss can be unilateral or bilateral and can range from mild to profound degrees of hearing loss. Epidermodysplasia Verruciformis (EDV) is a dermatologic condition in which patients show reduced immunologic ability to defend and eradicate certain types of Human Papillomavirus (HPV), leading to persistent infection and an increased lifetime risk of developing cutaneous dysplasia and malignancy. Both conditions have a genetic background. However, their concurrent occurrence is very rare. Therefore, a case study is presented of a four-year-old female child who visited the dermatology outpatient department with a chief complaint of hearing loss and white-coloured lesions covering her arms, neck, back, and chest for the last two years. The hearing loss was gradual, not apparent at birth, and progressed slowly to complete hearing loss. A final diagnosis of EDV with Non Syndromic Sensorineural Hearing Loss (NSSNHL) was made based on clinical and histological examination.

Keywords

• bilateral hearing loss
• gene mutation
• hypopigmented macules
• inherited disorder
• pityriasis versicolour
• vitiligo