Tozzi Andrea, Castellucci Andrea, Ferrulli Giuseppe, Martellucci Salvatore, Malara Pasquale, Brandolini Cristina, Armato Enrico, Ghidini Angelo
Otorhinolaryngology-Head and Neck Surgery Department, University Hospital of Modena, 41125 Modena, Italy.
ENT Unit, Department of Surgery, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy.
Audiol Res. 2023 Nov 1;13(6):833-844. doi: 10.3390/audiolres13060073.
Low-frequency air-bone gap (ABG) associated with pulsatile tinnitus (PT) and normal impedance audiometry represents a common finding in patients with third window syndromes. Other inner disorders, including Meniere's disease (MD), perilymphatic fistula and intralabyrinthine schwannoma, might sometimes result in a similar scenario. On the other hand, PT is frequently associated with dural arteriovenous fistula (DAVF), while conductive hearing loss (CHL) is extremely rare in this clinical setting. A 47-year-old patient was referred to our center with progressive left-sided PT alongside ipsilateral fullness and hearing loss. She also experienced headache and dizziness. Otoscopy and video-oculographic examination were unremarkable. Conversely, a detailed instrumental audio-vestibular assessment revealed low-frequency CHL with normal impedance audiometry, slight left-sided caloric weakness, slightly impaired vestibular-evoked myogenic potentials on the left and normal results on the video-head impulse test, consistent with an MD-like instrumental profile. Gadolinium-enhanced brain MRI revealed an early enhancement of the left transverse sinus, consistent with a left DAVF between the left occipital artery and the transverse sinus, which was then confirmed by angiography. A trans-arterial embolization with Onyx glue was performed, resulting in a complete recession of the symptoms. Post-operatively, the low-frequency ABG disappeared, supporting the possible role of venous intracranial hypertension and abnormal pressure of inner ear fluids in the onset of symptoms and offering new insights into the pathomechanism of inner ear CHL.
与搏动性耳鸣(PT)相关且声阻抗测听正常的低频气骨导差(ABG)是第三窗综合征患者的常见表现。其他内耳疾病,包括梅尼埃病(MD)、外淋巴瘘和迷路内神经鞘瘤,有时也可能导致类似情况。另一方面,PT常与硬脑膜动静脉瘘(DAVF)相关,而在这种临床情况下传导性听力损失(CHL)极为罕见。一名47岁患者因进行性左侧PT伴同侧耳部胀满感和听力损失被转诊至我们中心。她还伴有头痛和头晕。耳镜检查和视频眼震图检查未见异常。相反,详细的仪器音频前庭评估显示存在低频CHL且声阻抗测听正常,左侧有轻微的冷热试验减弱,左侧前庭诱发肌源性电位轻度受损,视频头脉冲试验结果正常,这与类似MD的仪器检查结果相符。钆增强脑MRI显示左侧横窦早期强化,符合左侧枕动脉与横窦之间的左侧DAVF,随后血管造影证实了这一诊断。采用Onyx胶进行经动脉栓塞,症状完全缓解。术后,低频ABG消失,这支持了静脉性颅内高压和内耳液压力异常在症状发作中的可能作用,并为内耳CHL的发病机制提供了新的见解。