Zanetti Diego, Di Berardino Federica
Audiology Unit, Department of Clinical Science and Community Health, University of Milano, IRCCS Fondazione Cà Granda, Ospedale Maggiore Policlinico, Milano, Italy.
Am J Case Rep. 2018 Jan 23;19:82-89. doi: 10.12659/ajcr.904907.
BACKGROUND Implantable devices have been proposed as an alternative to hearing aids and auditory canal reconstruction in patients with microtia (congenital aural atresia), which includes a malformation of the external and middle ear. This report is of two rare cases of microtia associated with congenital stapes ankylosis treated with an implantable device and describes the treatment outcomes. CASE REPORT Two siblings from Ecuador, a 29-year-old woman, and her 35-year-old brother, were born with unilateral type II microtia with bilateral external auditory canal atresia and conductive hearing loss. Pre-operatively, high-resolution computed tomography (HRCT) imaging was performed using FastView software to allow placement of a bone conduction-floating mass transducer (BC-FMT) to couple a Bonebridge bone conduction implant (BCI) system in both patients. Pure-tone audiometry (PTA) testing and speech audiology were performed. The Abbreviated Profile of Hearing Aid Benefit (APHAB) and the Speech, Spatial and Qualities (SSQ) of hearing scale questionnaires and scoring systems were used. Following activation of the implantable device, both patients achieved improved bilateral conductive hearing with sound-field (field-free) thresholds >25 dB, and speech recognition scores >90%. In both cases, hearing improvement remained at three years following surgery. CONCLUSIONS To our knowledge, these are the first reported cases of microtia with congenital stapes ankylosis successfully treated with a bone conduction implantable device. Patients with microtia and stapes ankylosis who are reluctant to undergo surgery may benefit from unilateral or bilateral, short-term or long-term use of a Bonebridge bone conduction implantable device.
对于小耳畸形（先天性外耳道闭锁）患者，可植入设备已被提议作为助听器和外耳道重建的替代方案，小耳畸形包括外耳和中耳的畸形。本报告介绍了两例罕见的先天性镫骨固定合并小耳畸形患者接受可植入设备治疗的情况，并描述了治疗结果。病例报告：来自厄瓜多尔的一对姐弟，29岁女性和35岁男性，出生时患有单侧II型小耳畸形，双侧外耳道闭锁及传导性听力损失。术前，使用FastView软件进行高分辨率计算机断层扫描（HRCT）成像，以便在两名患者中放置骨传导浮动质量传感器（BC-FMT）来耦合骨桥骨传导植入物（BCI）系统。进行了纯音听力测定（PTA）测试和言语听力检查。使用了助听器效益简易量表（APHAB）以及听力量表问卷和评分系统中的言语、空间和质量（SSQ）部分。激活可植入设备后，两名患者双侧传导性听力均得到改善，声场（无场）阈值>25 dB，言语识别得分>90%。在这两个病例中，术后三年听力改善情况持续存在。结论：据我们所知，这是首次报道的先天性镫骨固定合并小耳畸形成功使用骨传导可植入设备治疗的病例。不愿接受手术的小耳畸形合并镫骨固定患者可能受益于单侧或双侧、短期或长期使用骨桥骨传导可植入设备。
