Karlberg M, Annertz M, Magnusson M
Department of Otorhinolaryngology - Head & Neck Surgery, Lund University Hospital, Sweden.
J Laryngol Otol. 2006 May;120(5):419-22. doi: 10.1017/S0022215106000934. Epub 2006 Mar 24.
In 2003, it was reported that superior semicircular canal dehiscence can mimic otosclerosis because of low-frequency bone conduction hearing gain and dissipation of air-conducted acoustic energy through the dehiscence. We report the case of a 17-year-old girl with left-sided combined hearing loss thought to be due to otosclerosis. Bone conduction thresholds were -10 dB at 250 and 500 Hz and she had a 40 dB air-bone gap at 250 Hz. When a tuning fork was placed at her ankle she heard it in her left ear. Acoustic reflexes and vestibular evoked myogenic potentials could be elicited bilaterally. Imaging of the temporal bones showed no otosclerosis, superior semicircular canal dehiscence or large vestibular aqueduct, but a left-sided, Mondini-like dysplasia of the cochlea with a modiolar deficiency could be seen. Mondini-like cochlear dysplasia should be added to the causes of inner-ear conductive hearing loss.
2003年，有报道称，由于低频骨导听力增益以及通过缺损处气导声能的消散，上半规管裂可模拟耳硬化症。我们报告了一例17岁的左侧混合性听力损失女孩，其听力损失被认为是由耳硬化症引起的。250Hz和500Hz时骨导阈值为-10dB，250Hz时气骨导差为40dB。当将音叉置于其脚踝处时，她左耳能听到声音。双侧均可引出声反射和前庭诱发肌源性电位。颞骨影像学检查未发现耳硬化症、上半规管裂或大前庭导水管，但可见左侧类似Mondini畸形的耳蜗发育异常，伴有蜗轴缺损。类似Mondini畸形的耳蜗发育异常应被列入内耳传导性听力损失的病因之中。
