Brown Jason R, Milgraum D Micah, Riyaz Farhaad R, Jahnke Marla N, Thottam Prasad John
1 Department of Otolaryngology - Head and Neck Surgery, Detroit Medical Center, Detroit, MI, USA.
2 Drexel University College of Medicine, Philadelphia, PA, USA.
Ann Otol Rhinol Laryngol. 2017 Nov;126(11):778-780. doi: 10.1177/0003489417729833. Epub 2017 Sep 12.
Epidermolysis bullosa (EB) is a spectrum of mechanobullous disorders characterized by blistering following minor trauma or traction to the skin. Hearing loss in this population is poorly described in the otolaryngology literature, and its treatment oftentimes results in external auditory canal skin irritation.
We present the case of a 26-year-old female with EB and mixed hearing loss unable to wear conventional hearing aids due to sequelae of the external auditory canals. An osseointegrated implant was used as other hearing aids were deemed to be too destructive of the external auditory canal skin. Management and Outcome: Our patient underwent placement of a right bone-anchored hearing aid with minimal disruption of the surrounding skin using a minimally invasive punch technique. Over 1 year of follow-up, her course was complicated by 1 simple cellulitic infection at the surgical site treated successfully with oral antibiotics.
The literature regarding the otolaryngologic manifestations of EB is sparse. The otologic sequelae are particularly overlooked in the workup and management. Based on the results of this case study, it appears that an osseointegrated implant can be safely utilized to treat significant mixed or conductive hearing loss in patients with EB.
大疱性表皮松解症（EB）是一系列机械性大疱性疾病，其特征是皮肤受到轻微创伤或牵拉后出现水疱。耳鼻喉科文献中对该人群听力损失的描述较少，而且其治疗常常会导致外耳道皮肤刺激。
我们报告一例26岁患有EB和混合性听力损失的女性病例，由于外耳道后遗症，她无法佩戴传统助听器。由于其他助听器被认为对外耳道皮肤破坏太大，因此使用了骨整合植入物。治疗与结果：我们的患者采用微创打孔技术植入了右侧骨锚式助听器，对周围皮肤的干扰最小。在超过1年的随访中，她的病程因手术部位出现1次单纯蜂窝织炎感染而复杂化，经口服抗生素治疗成功。
关于EB耳鼻喉科表现的文献很少。在检查和治疗中，耳部后遗症尤其被忽视。根据本病例研究的结果，骨整合植入物似乎可以安全地用于治疗EB患者的严重混合性或传导性听力损失。
