Similar articles

Abstract: Objective: To describe the characteristics of the clinical presentation, diagnosis, surgical methods, and outcomes of patients with otogenic cerebrospinal fluid (CSF) leakage secondary to congenital inner ear dysplasia. Methods: A retrospective review was performed of 18 patients with otogenic CSF leakage secondary to inner ear dysplasia who underwent surgery in our group from 2007 to 2017 and had a follow-up of at least 4 months. The average length of follow-up was three years. The characteristics of the clinical presentations of all patients, such as self-reported symptoms, radiographic findings, surgical approaches and methods of repair, position of the leakage during surgery, and postoperative course, including the success rate of surgery, are presented. Results: The patients presented mostly with typical symptoms of meningitis, severe hearing impairment, and CSF otorrhea or rhinorrhea. All 18 patients had at least one previous episode of meningitis accompanied by a severe hearing impairment. The preoperative audiograms of 17 patients showed profound sensorineural hearing loss, and one patient had conductive hearing loss. Twelve patients presented with an initial onset of otorrhea, and two had accompanying rhinorrhea. Six patients complained of rhinorrhea, two of whom were misdiagnosed with CSF rhinorrhea and underwent transnasal endoscopy at another hospital. high-resolution computed tomography (HRCT) images can reveal developments in the inner ear, such as expansion of a vestibular cyst, unclear structure of the semicircular canal or cochlea, or signs of effusion in the middle ear or mastoid, which strongly suggest the possibility of CSF otorrhea. The children in the study suffered more severe dysplasia than adults. All 18 patients had CSF leakage identified during surgery. The most common defect sites were in the stapes footplates (55.6%), and 38.9% of patients had a leak around the oval window. One patient had a return of CSF otorrhea during the postoperative period, which did not re-occur following a second repair. Conclusions: CSF otorrhea due to congenital inner ear dysplasia is more severe in children than in adults. The most common symptoms were meningitis, hearing impairment, and CSF otorrhea or rhinorrhea. HRCT has high diagnostic accuracy for this disease. The most common fistula site was around the oval window, including the stapes footplates and the annular ligament.

18例先天性内耳发育不良继发脑脊液耳漏诊疗 总结

[1]Brown NE, Grundfast KM, Jabre A, et al., 2004. Diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea. Laryngoscope, 114(5):800-805.

[2]Hernandez RN, Changa AR, Bassani L, et al., 2015. Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report. Childs Nerv Syst, 31(9):1613-1616.

[3]Hoppe F, Hagen R, Hofmann E, 1997. Fistula of stapes footplate caused by pulsatile cerebrospinal fluid in inner ear malformation. ORL J Otorhinolaryngol Relat Spec, 59(2):115-118.

[4]Jackler RK, Luxford WM, House WF, 1987. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope, 97(S40):2-14.

[5]Janocha-Litwin J, Simon K, 2013. Recurrent meningitis—a review of current literature. Przegl Epidemiol, 67(1):41-45.

[6]Kou YF, Zhu VF, Kutz JW Jr, et al., 2016. Transcanal endoscopic management of cerebrospinal fluid otorrhea secondary to congenital inner ear malformations. Otol Neurotol, 37(1):62-65.

[7]Lim R, Brichta AM, 2016. Anatomical and physiological development of the human inner ear. Hear Res, 338:9-21.

[8]Lin CY, Lin HC, Peng CC, et al., 2012. Mondini dysplasia presenting as otorrhea without meningitis. Pediatr Neonatol, 53(6):371-373.

[9]Lue AJ, Manolidis S, 2004. Intrathecal fluorescein to localize cerebrospinal fluid leakage in bilateral Mondini dysplasia. Otol Neurotol, 25(1):50-52.

[10]Ohlms LA, Edwards MS, Mason EO, et al., 1990. Recurrent meningitis and Mondini dysplasia. Arch Otolaryngol Head Neck Surg, 116(5):608-612.

[11]Rao AK, Merenda DM, Wetmore SJ, 2005. Diagnosis and management of spontaneous cerebrospinal fluid otorrhea. Otol Neurotol, 26(6):1171-1175.

[12]Savva A, Taylor MJ, Beatty CW, 2003. Management of cerebrospinal fluid leaks involving the temporal bone: report on 92 patients. Laryngoscope, 113(1):50-56.

[13]Sennaroglu L, Saatci I, 2002. A new classification for cochleovestibular malformations. Laryngoscope, 112(12):2230-2241.

[14]Tebruegge M, Curtis N, 2008. Epidemiology, etiology, pathogenesis, and diagnosis of recurrent bacterial meningitis. Clin Microbiol Rev, 21(3):519-537.

[15]Teo DTW, Tan TY, Eng SP, et al., 2004. Spontaneous cerebrospinal fluid otorrhoea via oval window: an obscure cause of recurrent meningitis. J Laryngol Otol, 118(9):717-720.

[16]Tyagi I, Syal R, Goyal A, 2005. Cerebrospinal fluid otorhinorrhoea due to inner-ear malformations: clinical presentation and new perspectives in management. J Laryngol Otol, 119(9):714-718.

[17]Wilson MN, Simon LM, Arriaga MA, et al., 2014. The management of spontaneous otogenic CSF leaks: a presentation of cases and review of literature. J Neurol Surg B Skull Base, 75(2):117-124.

[18]Yi HJ, Guo H, Ch W, et al., 2013. Use of the translabyrinthine approach to repair congenital spontaneous cerebrospinal fluid leakage in five Chinese patients with Mondini dysplasia. Int J Pediatr Otorhinolaryngol, 77(12):1965-1968.