Almuzaini Hanan, Müller J, Wilhelm Flatz, Polterauer D, Schuster M
General and Specialized Surgery Department Medicine College, Taibah University Madinah, KS Saudi Arabia.
Department of Otorhinolaryngology, Head and Neck Surgery Ludwig-Maximilians-University of Munich Munich Germany.
Clin Case Rep. 2024 Sep 3;12(9):e9423. doi: 10.1002/ccr3.9423. eCollection 2024 Sep.
In young infants, under the age of one-year, cochlear malformation with profound hearing loss complicated by a perilymphatic fistula (PLF), presents a serious clinical challenge, warranting immediate audiological and surgical intervention. Timely PLF detection and closure, along with an early CI can significantly improve the prognosis of such patients and helps them in achieving their maximum hearing and developmental potential, in the long term.
Inner ear malformation (IEM) with incomplete partition and cystic cochlea is mostly accompanied by profound hearing loss. It gets further complicated with other malformations such as a perilymphatic fistula (PLF). This case concerns an 8-month-old child cochlear malformation and profound hearing loss. Surgical intervention identified a PLF at the stapedial footplate, which was successfully closed. The surgery also included the placement of a cochlear implant (CI) in the right ear, via the round window. The left ear was equipped with hearing aids, with persistent hearing thresholds at 70-80 db. At the age of 6 years, the child showed a good hearing outcome with the CI, with only moderate speech delay. Cochlear malformation accompanied by a perilymphatic leakage warrants immediate surgical closure of the PLF, to minimize the risk of bacterial meningitis. Wherever possible, the feasibility of a CI should be explored in such cases and a CI should be placed for treatment of hearing loss. Audiological and speech outcomes may vary with the use of the CI, especially in cases of IEM. However, an early CI coupled with timely PLF detection and closure can help children with profound hearing loss, in achieving their maximum hearing and developmental potential, in the long run.
对于一岁以下的幼儿，伴有外淋巴瘘（PLF）的耳蜗畸形合并重度听力损失是一项严峻的临床挑战，需要立即进行听力学和手术干预。及时检测并封闭PLF，同时尽早植入人工耳蜗（CI），可显著改善此类患者的预后，并有助于他们长期实现最大听力和发育潜能。
不完全分隔和囊性耳蜗的内耳畸形（IEM）大多伴有重度听力损失，并常伴有外淋巴瘘（PLF）等其他畸形。本病例为一名8个月大的儿童，患有耳蜗畸形和重度听力损失。手术干预发现镫骨足板处存在PLF，并成功将其封闭。手术还包括通过圆窗在右耳植入人工耳蜗（CI）。左耳佩戴助听器，听力阈值持续在70 - 80分贝。6岁时，该儿童使用CI后听力结果良好，仅存在中度语言发育迟缓。伴有外淋巴瘘的耳蜗畸形需要立即手术封闭PLF，以降低细菌性脑膜炎的风险。在这类病例中，应尽可能探索CI植入的可行性，并植入CI以治疗听力损失。使用CI后的听力学和言语结果可能会有所不同，尤其是在IEM病例中。然而，早期植入CI并及时检测和封闭PLF，从长远来看有助于重度听力损失儿童实现最大听力和发育潜能。
