Introduction
Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder caused by TSC1 or TSC2 mutations is characterized by hamartomas in various organs (e.g., skin, brain, lungs, and kidneys) and appears with an incidence rate of approximately 1 in 5000–10,000 [1]. Angiofibromas are the most frequent skin lesions occurred in patients with TSC older than 5 years and characteristically consist of numerous pink to reddish papules or nodules that are typically located on the cheeks, nose, and chin [2]. The traditional and current treatment modalities for facial angiofibromas include topical sirolimus, topical rapamycin cryosurgery, curettage, dermabrasion, chemical peeling, excision, and laser therapies. Treatment options generally vary according to the clinical presentation of the disease and the location of the TSC related lesions. Treatments for skin lesions in the face and head and neck area are usually intended for cosmetical issues, except for ocular manifestations, and the treatment decision is made according to the severity of the disease (e.g. Facial Angiofibroma Severity Index). Auricula and external auditory canal (EAC) involvement in TSC related angiofibroma a rare and specific pathology due to the aesthetic and functional feature of the ear.
The obstruction of the EAC causes recurrent infections by preventing aeration and excretion of cerumen. It can also cause conductive hearing loss. Adequate meatoplasty is important to achieve a dry, self-cleaning EAC canal to protect recurrent external otitis. Bony canaloplasty, cartilage excision, Z-plasty techniques, or V-Y flaps are available for EAC meatoplasty (3).