Khosla Shivun, Elliot Matthew, Donnelly Neil
a Department of Otolaryngology , Cambridge University Hospitals Trust , Cambridge , UK.
b Gonville and Caius College, University of Cambridge , Cambridge , UK.
Cochlear Implants Int. 2017 Mar;18(2):121-124. doi: 10.1080/14670100.2017.1289605. Epub 2017 Feb 20.
To illustrate our experience when managing a complex patient with potentially life-threatening bilateral otological disease facing multisensory compromise including complete loss of audiovestibular function and visual disturbance Clinical presentation: A 67 year old lady, presented with a large left vestibular schwannoma and extensive right cholesteatoma encircling the otic capsule. She underwent translabyrinthine resection of the vestibular schwannoma, resulting in profound sensorineural hearing loss, vestibular hypofunction and corneal scarring following an initial temporary facial palsy. Due to the extent of the disease and good right-sided bone conduction thresholds, the cholesteatoma was managed conservatively utilising a bone-anchored-hearing-aid with regular review by the Skull-Base team. However, following acute deterioration in hearing and disease extension threatening right facial nerve function, the decision was taken for surgical intervention with a view to staged cochlear implantation (CI).
Our patient underwent right lateral petrousectomy, total osseous labyrinthectomy, and implantation of an intracochlear 'dummy' with blind sac closure of the external auditory canal. Recovery was excellent with no further deterioration in balance and no loss of facial nerve function. CI occurred 3 months post-operatively with good functional outcome.
We present the management of bilateral complex otoneurological disease with significant risk of multisensory compromise. Size of the left vestibular schwannoma necessitated surgical resection with resultant loss of ipsilateral audiovestibular function. Due to residual right audiovestibular function and the risks of surgery, the extensive right cholesteatoma was managed conservatively until disease progression necessitated surgical intervention. Subsequently it was possible to restore access to open-set speech with a right CI.
阐述我们在管理一位患有可能危及生命的双侧耳科疾病且面临多感官功能受损（包括听觉前庭功能完全丧失和视觉障碍）的复杂患者时的经验。
一位67岁女性，患有巨大的左侧前庭神经鞘瘤和环绕听骨囊的广泛右侧胆脂瘤。她接受了前庭神经鞘瘤的经迷路切除术，术后出现严重的感音神经性听力损失、前庭功能减退以及初始短暂性面瘫后的角膜瘢痕形成。鉴于疾病范围以及右侧良好的骨导阈值，对胆脂瘤采取保守治疗，使用骨锚式助听器，并由颅底团队定期复查。然而，在听力急性恶化且疾病扩展威胁右侧面神经功能后，决定进行手术干预，以期分期进行人工耳蜗植入（CI）。
我们的患者接受了右侧岩骨次全切除术、全骨性迷路切除术，并植入了耳蜗内“假电极”，同时对外耳道进行盲袋封闭。恢复情况良好，平衡功能未进一步恶化，面神经功能也未丧失。术后3个月进行了人工耳蜗植入，功能结果良好。
我们展示了对双侧复杂耳神经疾病的管理，该疾病存在多感官功能受损的重大风险。左侧前庭神经鞘瘤的大小决定了需要进行手术切除，导致同侧听觉前庭功能丧失。由于右侧仍保留听觉前庭功能以及手术风险，广泛的右侧胆脂瘤在疾病进展需要手术干预之前一直采取保守治疗。随后，通过右侧人工耳蜗植入恢复了开放环境下的言语交流能力。
