Otology & Neurotology 35:1592Y1595 Ó 2014, Otology & Neurotology, Inc.

Novel Repair of Stapedial Footplate Defect Associated with Recurrent Meningitis *†Shinji Urata, *Akinori Kashio, *Takashi Sakamoto, *Akinobu Kakigi, and *Tatsuya Yamasoba *Department of Otolaryngology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan; and ÞDepartment of Otolaryngology, JR Tokyo General Hospital, Tokyo, Japan

Objective: Recurrent meningitis resulting from a partial defect of the stapedial footplate is most frequently treated by complete obliteration of the inner ear. Herein, we report the use of a partial obliteration technique in which the fistula is plugged with a shaped incus. Patients: A 5-year-old girl and a 7-year-old boy who had congenital inner ear malformations both developed recurrent meningitis. High-resolution computed tomography (HRCT) revealed soft tissue opacities between the mastoid air cells and the mesotympanum. Exploratory tympanotomies were performed, revealing cerebrospinal fluid (CSF) leakages from fistulae at the stapes footplate in both patients. Interventions: In each case, the vestibule was partially obliterated with temporalis fascia and modified incus remnant. Bone dust and fibrin were also applied.

Main Outcome Measures: Recurrence of CSF leakage and patient symptoms. Results: None of the patients exhibited vertigo or dizziness after surgery, and meningitis did not recur during the follow-up period. Conclusions: Compete obliteration of the inner ear space can be avoided by using this technique, thereby leaving room for future cochlear implantation in addition to reducing direct injury to the vestibular system. Key Words: Cochlear implantationVInner ear deformityVMeningitisVMondini dysplasia.

Recurrent meningitis can be caused by congenital inner ear malformations with continuous cerebrospinal fluid (CSF) leakage (1). Cochlear dysplasia associated with a defect in the stapes footplate is usually the cause of recurrent meningitis (2). Treatment of the CSF leakage in these cases is usually accomplished by complete obliteration of the vestibule and the cochlea with muscle and/or fascia via the oval window after removal of the stapes footplate. However, this method occludes the inner space, sacrifices any residual hearing or vestibular function, and makes future cochlear implant surgery challenging or impossible because of fibrosis and or ossification after the repair. Moreover, it has been reported that complete obliteration after removal of the stapes footplate has a high CSF leak recurrence of 30 to 60% (1Y3). In this clinical capsule, we describe an alternative method to successfully repair a CSF leak in two children using a

modified incus as a stopper, bone dust, and fibrin glue for partial obliteration of the vestibule.

Otol Neurotol 35:1592Y1595, 2014.

PATIENTS Case 1 A girl (5.8 years of age) had been diagnosed with bilateral profound sensorineural hearing loss (SNHL) with inner ear malformation. When she was 2 years old, her mother suspected that she had hearing impairment. The auditory brainstem-evoked response (ABR) showed profound hearing loss. HRCT revealed bilateral inner ear malformations (right ear, common cavity deformity; left ear, cochlear hypoplasia). She did not respond to sound with hearing aids and she had been communicating by using the finger alphabet. She developed meningitis twice from the ages of 3 to 5 years and was referred to our institution for evaluation of the recurrent bacterial meningitis. Otoscopic examination of both ears showed slightly red tympanic membranes without bulging. Conditioned orientation response audiometry revealed profound SNHL and vestibular testing demonstrated absent calorics in both ears.

Address correspondence and reprint requests to Shinji Urata, MD, Department of Otorhinolaryngology, JR General Hospital, 2-1-3 Yoyogi, Shibuya, Tokyo, 151-8528, Japan; E-mail: [email protected] The authors disclose no conflicts of interest.

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FISTULA PLUG USING TRIMMED INCUS FOR RECURRENT MENINGITIS However, cervical vestibular-evoked myogenic potential (cVEMP) were seen bilaterally; the threshold of the cVEMP using tone burst was 90 dB. HRCT showed opacification of the left middle ear and mastoid suggesting otitis media or CSF with no gross evidence of a bony fistula (Fig. 1, A, B). We performed an exploratory tympanotomy. The middle ear mucosa was thickened, and CSF had been leaking gradually from gaps in that tissue. After debriding the inflammatory disease, the stapes was seen and appeared normal. A bony crista was found in the center of the stapes footplate and a small fistula in the bony crista, from which CSF had also been leaking, was observed (Fig. 2, A). At this point, the incudostapedial joint was separated and the incus was removed. The fistula was widened using a hand drill applied against the center of the footplate. Temporalis fascia was then used to plug the bony defect. The CSF stopped. We measured the distance between the stapes footplate and the upper edge of the obturator foramen of the stapes by using a depth gauge, and we used a diamond bur to trim the removed incus like a cork. Subsequently, we plugged the fistula in the stapes footplate with one side of the incus trimmed as a cork, and the superstructure of the stapes held the other side of the trimmed incus (Fig. 2, B). Next, bone dust was placed around the plugged incus, and fibrin glue was applied for sealing. The patient had an uneventful postoperative course and remained disease-free during the 28-month follow-up period after the surgery.

FIG. 1. Temporal CT findings in Case 1 (A and B), and Case 2 (C and D). In Case 1, a common cavity deformity on the right side and cochlear hypoplasia on the left (arrowhead) were observed, and soft tissue opacity was noted from the mastoid air cell to the mesotympanum on the left (arrow). In Case 2, incomplete partition was observed (arrowhead ) on both sides, and soft tissue opacity was noted from the mastoid air cell to the mesotympanum on the left side (arrow).

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FIG. 2. Surgical findings of Case 1. A fistula (arrow) was noted in the center of the stapes (A). The temporal muscle was inserted in the fistula (B), and the trimmed incus (arrowhead ) was subsequently inserted between the superstructure and the footplate of the stapes.

Case 2 A 7-year-old boy had bilateral congenital inner ear malformations classified as incomplete partition. Newborn hearing screening and follow-up ABR testing at 7 months of age showed profound hearing loss. At the age of 3 years, he underwent right cochlear implantation (CI). The postoperative course was uneventful. However, from the age of 4 to 7 years, he was hospitalized three times in a local pediatric unit because of bacterial meningitis. To investigate the etiological factors responsible for the recurrent meningitis, the previous pediatrician again referred him to our institution. The initial finding of the otoscopic examination was bulging of the left eardrum. He did not have a fever or central nervous system symptoms. Opacification was seen in the left middle ear and mastoid (Fig. 1, C and D). We performed a left exploratory tympanotomy. CSF leakage from the center of the stapes footplate was found after the membranous tissue in the tympanic cavity was removed. The stapes superstructure was almost normal, but a small fistula was present in the bony crista in the stapes footplate. We detached the incudostapedial articulation and removed the incus. We partially obliterated the vestibule by using pieces of temporal fascia via the fistula. When we confirmed that the CSF leakage had stopped, we plugged the fistula in the stapes footplate with a trimmed incus, similar to Case 1. Finally, we placed bone dust in the surrounding area and affixed the plug with fibrin glue. The patient did not exhibit dizziness or vertigo, and meningitis did not recur during the 48-month follow-up period. These studies were conducted in accordance with the Helsinki Declaration and were approved by the University of Tokyo Human Ethics Committee. DISCUSSION Congenital cochlear malformations are developmental abnormalities of the otic capsule. Cochlear malformations are classified into five general types based on imaging findings: Michel deformity, cochlear aplasia, cochlear hypoplasia, incomplete partition (Mondini deformity), and Otology & Neurotology, Vol. 35, No. 9, 2014

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common cavity, by the process of fetal inner ear development (4,5). Congenital cochlea malformations are suspected when severe hearing impairment, a history of perilymphatic fistula, CSF otorrhea, and recurrent meningitis are observed (5). Teruegge provided a comprehensive literature review on recurrent meningitis (6), identifying 363 cases published in the preceding 20 years. These reports included 214 (59%) cases of anatomical problems (including CSF fistulas), which included 55 (15%) cases of inner ear malformations. Recurrent meningitis in children can occur because of abnormal communication between the subarachnoid space and the middle ear space caused by inner ear malformation. Abnormal communication routes are divided into the subarachnoid space to the vestibular space and the vestibular space to the middle ear space. Schuknecht (7) indicated that the former abnormal routes involve abnormal patency of the cochlear aqueduct and the lamina cribrosa between the internal auditory canal and vestibule. Gacek et al. (8) indicated that the latter abnormal route involved abnormality of the stapes footplate. The stapes footplate is commonly malformed because of either a common embryologic defect or erosion from continuous pulsatile CSF pressure (9). Harada et al. (10) reported that microfissures in the oval window were observed in 25% of 331 temporal bones studied, but defects of the stapes superstructure have not been described. Recurrent meningitis due to inner ear deformity has primarily been treated by obliteration of the middle ear (and the mastoid) and/or the inner ear. Obliteration of the middle ear with and without mastoid obliteration preserves the vestibular space, but this structure may not be able to withstand high-pressure CSF leakage due to the fistula of the stapes, resulting in a high recurrence rate

(11). In contrast, although obliteration of the inner ear has a lower rate of recurrence, it still has a 30 to 60% failure rate and results in inner ear destruction (1Y3). Recent reports have described successful repair of congenital CSF fistulae by using fibrin glue (1,12Y15). Savva et al. invented a multilayer technique combining allogenic materials (fibrin glue) with free autologous grafts (13). Tyagi et al. successfully repaired defects by using fibrin glue to pack the vestibule (1). These conventional methods may be highly effective for stopping CSF leakage, but packing the inner ear cavity produces inner dysfunction. CI may become an option in the future for some cases with profound SNHL. Therefore, partial obliteration of the inner ear offers some advantages compared to conventional methods. Our new method (Fig. 3) for stopping CSF leakage has three main advantages compared to conventional methods. First, by plugging the shaped incus into the fistula, it is possible to eliminate gaps between CSF leaks. Second, fastening the shaped incus by the superstructure of the stapes allows the superstructure to have a bracing function to prevent leakage due to CSF pressure in the horizontal direction. Finally, spreading bone dust affixed with fibrin glue can reduce the CSF escape pressure to an ambient level. Our method can stop CSF leakage with a lesser quantity of fasciae than is usually used, thereby avoiding complete obliteration of the inner ear cavity. Moreover, this method not only leaves room for future CI but can also preserve vestibular function. Our new method has advantages even if fistula closure and CI must be performed simultaneously because, if CI failure occurs, our method can be assumed to preserve more inner-ear cavity and re-insertion of the CI would be more likely compared to complete inner-ear obstruction. Because we have reported our experience

FIG. 3. Steps of partial obliteration of the inner ear. After identifying the fistula, the defect was enlarged by drilling for placement of the fascia (A). The fascia was inserted, which stopped the CSF leakage (B). A portion of the trimmed incus was inserted to plug the fistula and was fastened by the superstructure of the stapes (C). Ultimately, bone dust was spread in the surrounding area and was affixed with fibrin glue (D). Otology & Neurotology, Vol. 35, No. 9, 2014

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FISTULA PLUG USING TRIMMED INCUS FOR RECURRENT MENINGITIS with only two cases, we cannot definitively conclude that our method is very effective and is superior to the previously reported techniques. However, the good outcome in our cases suggests that our method can be considered as one of the choices for closure of CSF fistulae due to a defect in the stapes footplate. Further study is required in additional cases. CONCLUSION Partial obliteration of the inner ear, as described in the current paper, can be an effective treatment modality in cases of CSF leakage due to recurrent meningitis with a fistula of the stapes footplate. Further study is required to confirm the efficacy of this technique in additional cases. REFERENCES 1. Tyagi I, Syal R, Goyal A. Cerebrospinal fluid otorhinorrhoea due to inner-ear malformations: clinical presentation and new perspectives in management. J Laryngol Otol 2005;119:714Y18. 2. Ohlms LA, Edwards MS. Recurrent meningitis and Mondini dysplasia. Arch Otolaryngol Head Neck Surg 1990;116:608Y12. 3. Spetzler RF, Wilson SB. Management of recurrent CSF rhinorrhea of the middle and posterior fossa. J Neurosurg 1978;49:393Y7. 4. Jackler RK, Luxford WM, House WF. Congenital malformations of the inner ear: a classification based on embryogenesis. Laryngoscope 1987;40:2Y14.

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5. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope 2002;112:2230Y41. 6. Kakigi A, Tomofuji S, Takeda T, Sawada S. Cerebrospinal fluid otorrhea in a patient with congenital inner ear dysplasia. Auris Nasus Larynx 2001;28:357Y9. 7. Tebruegge M, Curtis N. Epidemiology, etiology, pathogenesis, and diagnosis of recurrent bacterial meningitis. Clin Microbiol Rev 2008; 21:519Y37. 8. Schuknecht HF. Mondini dysplasia; a clinical and pathological study. Ann Otol Rhinol Laryngol 1980;89:11Y23. 9. Gacek RR, Leipzig B. Congenital cerebrospinal otorrhea. Ann Otol 1979;88:358Y65. 10. Weider DJ, Geurkink NA, Saunders RL. Spontaneous cerebrospinal fluid otorhinorrhea. Am J Otol 1985;6:416Y22. 11. Harada T, Sando I, Myers EN. Microfissure in the oval window area. Ann Otol Rhinol Laryngol 1981;90:174Y80. 12. Stool S, Leeds NE, Shulman K. The syndrome of congenital deafness and otic meningitis: diagnosis and management. J Pediatr 1967;71:547Y52. 13. Ronald AH. Cerebrospinal fluid leak of temporal bone origin. In: Jackler RK, Brackmann DE, eds. Neurotology. London: Mosby, 1994:919Y28. 14. Savva A, Taylor MJ, Beatty CW. Management of cerebrospinal fluid leaks involving the temporal bone: report on 92 patients. Laryngoscope 2003;113:50Y6. 15. Kulkarni GB, Roopa S, Madhu N, et al. Cystic cochleovestibular anomaly presenting with congenital deafness and recurrent bacterial meningitis in childhood. Ann Indian Acad Neurol 2013;16:272Y5. 16. Lien TH, Fu CM, Hsu CJ, Lu L, Peng SS, Chang LY. Recurrent bacterial meningitis associated with Mondini dysplasia. Pediatr Neonatol 2011;52:294Y6.

Otology & Neurotology, Vol. 35, No. 9, 2014

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Novel repair of stapedial footplate defect associated with recurrent meningitis.

Recurrent meningitis resulting from a partial defect of the stapedial footplate is most frequently treated by complete obliteration of the inner ear. ...
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