Marques Letícia Helena de Sousa, Martins Daniela Vieira, Juares Gabriel Liria, Lorenzetti Fábio Tadeu Moura, Monsanto Rafael da Costa
Department of Otolaryngology, Head and Neck Surgery, Banco de Olhos de Sorocaba Hospital, Sorocaba, Brazil.
Department of Otolaryngology, Head and Neck Surgery, Banco de Olhos de Sorocaba Hospital, Sorocaba, Brazil; Department of Otorhinolaryngology, Head and Neck Surgery, Universidade Federal de São Paulo / Escola Paulista de Medicina (UNIFESP / EPM), São Paulo, Brazil.
Int J Pediatr Otorhinolaryngol. 2017 Oct;101:223-229. doi: 10.1016/j.ijporl.2017.08.020. Epub 2017 Aug 22.
To describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature.
We performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome.
Fifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome.
Although rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients.
基于一例病例报告及对文献的系统综述，描述和讨论拉森综合征的耳科表现。
我们在PubMed数据库进行了检索，并选择了报告拉森综合征继发的耳鼻喉科表现的研究。对所选文章进行全文阅读，三位研究人员独立从这些研究中提取数据。同时，我们报告一例14岁因拉森综合征继发听力损失的患者。
15项研究符合我们的选择标准。7项研究报告了拉森综合征患者存在听力损失（4例为传导性听力损失，3例为混合性听力损失）。传导性听力损失可能继发于听骨畸形和/或中耳积液。传导性听力损失的其他原因包括中耳间充质残余、咽鼓管功能障碍和腭裂。在某些特定病例中，听力损失的手术治疗是可行的，不过应考虑手术和麻醉风险。助听器似乎是治疗拉森综合征继发听力损失的安全有效的选择。
尽管罕见，但拉森综合征患者可能出现听力损失。最常见的听力缺陷类型是传导性的，继发于听小骨畸形或中耳积液。这些异常的可能手术矫正应与这些患者的麻醉风险相权衡。
