关于并发中耳和内耳异常的患者的报告很少见。这些患者对人工耳蜗植入提出了手术挑战。手术风险必须与患者的听力结果和随后的言语和语言发展及其生活质量的预期益处进行权衡。
13岁的男孩被送到耳科诊所接受听觉康复治疗。他有轻微的发育迟缓,是非语言的,通过美国手语进行交流。他出生时患有双侧耳廓闭锁,从未戴过扩音术。在考试中,他患有1级小耳畸形和双侧完全耳道闭锁。他的行为听力测试显示双耳严重的感觉神经性听力损失。计算机断层扫描显示双侧乳突和中耳不发达和完全混浊,耳道完全骨性闭锁,和一个分区不足的耳蜗,在其他异常中。向患者及其家人提供了可用的选择以及任何进一步研究的必要性的咨询。
为患者和家属提供咨询。
虽然文献中有报道对并发闭锁和耳蜗发育不良的患者进行耳蜗植入,这些患者的内耳异常程度相对较小,其听力学预后良好.所呈现的病例是患者的病例,对于该患者而言,仅对耳蜗进行手术治疗是困难的。更重要的是,根据预测的有限的听力和语言发展结果,他的生活质量不会显着提高,考虑到他内耳异常的严重程度,沟通能力有限,长时间的耳聋和发育迟缓。
Reports of patients with concurrent middle and inner ear anomalies are rare. These patients present a surgical challenge for cochlear implantation. The surgical risk must be weighed against the predicted benefit of the patient\'s hearing outcome and subsequent development of speech and language as well as their quality of life.
Thirteen-year-old boy presented to the Otology clinic for auditory rehabilitation options. He has mild developmental delay, is non-verbal and communicates via American Sign Language. He was born with bilateral aural atresia and never wore amplification. On exam he has grade 1 microtia and complete ear canal atresia bilaterally. His behavioural hearing test shows profound sensorineural hearing loss of both ears. The computed tomography scan shows bilateral underdeveloped and completely opacified mastoid and middle ear, complete bony atresia of the ear canals, and an under-partitioned cochlea with poorly defined modiolus, among other abnormalities. The patient and his family were counselled on the available options as well as the need for any further studies.
Counselling of patient and family.
While there have been
reports in the literature of performing cochlear implantations in patients with a concurrent atresia and cochlear dysplasia, these were patients whose degree of inner ear anomalies was relatively minor and their prognosis of a good audiological outcome was favourable. The presented
case is that of a patient for whom the surgical approach to the cochlea alone would be difficult. More importantly, his quality of life would not significantly improve in light of the predicted limited hearing and language development outcomes, given the severity of his inner ear abnormalities, limited communication abilities, prolonged period of deafness and developmental delays.