Original research paper
Cerebrospinal fluid gusher in cochlear implantation Ali Eftekharian, Maryam Amizadeh Department of Otolaryngology, Shahid-Beheshti University of Medical Sciences, Tehran, Iran Objectives: To share our experience of cerebrospinal fluid (CSF) gusher in cochlear implantation. Methods: Demographic, radiological, and surgical results of patients with CSF gusher in 523 consecutive cochlear implant recipients including children and adults as well as our management technique were evaluated and a review of the literature has been included. Results: Fifteen (2.87%) cases had CSF gusher. Two patients (13.3%) were adults with post-lingual hearing loss and the rest 12 (86.7%) were children with congenital hearing loss. Twelve patients (80%) had various types of inner ear malformation. Three patients (20%) had no predictable risk of CSF gusher from history or pre-operative imaging. In all patients, CSF gushers were controlled with our technique of packing the electrode entrance site with no additional measures. Conclusion: CSF gusher may occur with post-lingual hearing loss and in children with apparently unremarkable imaging and history. Thus, surgeons should always be ready to manage it. Management of CSF gusher can be mainly performed during the initial surgery by precise tight packing of the electrode entrance site. Furthermore, non-surgical or surgical measures are rarely required to stop a persistent leak. Our results show that our management technique may be recommended as well. Keywords: Cochlear implant, CSF gusher, Management, Inner ear malformation, Complication
Introduction
Materials and methods
The incidence of cerebrospinal fluid (CSF) gusher in cochlear implantation is reported between 1 and 5% in large series (Wootten et al., 2006; Brito et al., 2012; Kempf et al., 1999; Ding et al., 2009; Kim et al., 2004). Most of the literature addresses CSF gushers in the pediatric population during cochleostomy and there are few reports of its occurrence in adults (Wootten et al., 2006). Failure to create a watertight seal around the cochlear implant array would create a risk for future CSF leakage and produce a potential hazard for meningitis. Various techniques have been reported to seal a CSF leak around a cochlear implant array. These methods include packing muscle or connective tissue around the implant, sealing the muscle with fibrin glue, packing with several strips of bone-waxed silk suture material plus muscle (Marks, 2004), and the use of a specially designed electrode array (Sennaroglu, 2010). Here, we report our radiological, surgical, and management experience in CSF gusher at cochlear implantation.
Between July 2008 and May 2013, 523 cochlear implant procedures including children and adults have been done in our department. Their clinical records were reviewed and the data of patients who had CSF gusher were enrolled in this study. Only those who had profuse CSF outflow upon opening the cochlea which usually last several minutes were considered. Cases with pulsatile perilymph, oozing, or leaks that are sometimes found on opening the cochlea, for example, in those with an isolated large vestibular aqueduct, were not enrolled in this study. Demographic, radiological, and surgical results of these patients as well as our management technique are evaluated.
Correspondence to: Ali Eftekharian, Department of Otolaryngology, Loghman Hospital, Kamali Street, South Kargar Ave. 13336-35445, Tehran, Iran. E-mail: [email protected]
© W. S. Maney & Son Ltd 2014 DOI 10.1179/1754762814Y.0000000069
Results Out of 523 cochlear implantations including children and adults, 15 (2.87%) cases had CSF gusher who also met our inclusion and exclusion criteria. Their data are shown in Table 1. Two patients (13.3%) were adults with post-lingual hearing loss and the rest 12 (86.7%) were children with congenital hearing loss. Highresolution computed tomography (CT) scans in three patients (20%) did not show any obvious anomaly to predict CSF gusher occurrence in the operation. Twelve patients (80%) had various types of inner ear
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Table 1
Patient’s demographics
Patient
Sex
Age (year)
Type of deafness
Op side
1
M
42
Post
L
2 3 4 5 6 7 8 9 10 11 12 13 14 15
F M F F F F M M M F F M F M
8 9 9 5 53 14 2 5 6 6 2 4 2 2
Pre Pre Pre Pre Post Pre Pre Pre Pre Pre Pre Pre Pre Pre
R R R L R L R R L R L L R L
CT scan findings Right side: total ossification Left side: just erosion of lateral semicircular canal Incomplete partition type I Incomplete partition type III Incomplete partition type I Common cavity Normal Incomplete partition type I Normal Incomplete partition type I Incomplete partition type I Incomplete partition type I Mondini deformity Mondini deformity Incomplete partition type I Incomplete partition type I
Electrode entrance
Device
Follow-up (months)
R.W
HiRes90k1j
46
C R.W R.W C R.W R.W R.W C R.W R.W R.W R.W R.W R.W
CI24R(ST) CI24RE HiRes90k1j HiRes90k1j HiRes90k1j CI24RE CI24RE HiRes90k1j HiRes90k1j HiRes90k1j CI24RE HiRes90k1j HiRes90k1j CI24RE
42 33 19 19 18 18 15 9 8 6 5 3 3 2
M, male; F, female; Op side, operated side; L, left; R, right; Post, post-lingual; Pre, pre-lingual; R.W, round window; C, cochleostomy.
Figure 1 Patient 11: Incomplete partition type I.
malformation. Eight cases (66.7% of malformations) were incomplete partition type I. Fig. 1 shows the CT scan of one of them (patient 11). Two patients had a Mondini deformity (Fig. 2 shows the CT scan of one of them; patient 12). One child had common cavity deformity and one child had X-linked deafness or incomplete partition type III (patient 3, Fig. 3). One child with congenital deafness ( patient 8) had imaging that seems to be normal (Fig. 4). Patient 6 was a 53-year-old female. She suddenly lost her left-
Figure 2 Patient 12: Mondini deformity.
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side hearing 28 years ago during her second pregnancy without any clear reason. Seven years later, her rightside hearing was abruptly lost. She claimed that it occurred after her father’s funeral. There is also no obvious reason for this side deafness. Fig. 5 demonstrates one of her magnetic resonance imaging (MRI) images. Patient 1 was a 42-year-old man with bilateral profound hearing loss. His right side was operated on 30 years ago possibly for a cholesteatoma but there are no clear data about his hearing loss. His left side was operated on for an acquired cholesteatoma in another center 6 months before admission in our center. His clinical history revealed that his left hearing was abruptly deteriorated after its operation. On the right side, the cochlea was nearly completely ossified and on the left side, the only apparent defect was an erosion of the lateral semicircular canal (Fig. 6). In our department, the preferred technique of electrode insertion is the round window approach. Cochleostomy is chosen just when anatomically adequate view of the round window does not exist. In 12 patients (80%), round window was the route of
Eftekharian and Amizadeh
Figure 5
Figure 3
Patient 3: Incomplete partition type III.
electrode insertion. In three remaining ones, cochleostomy was used anteroinferior to the round window. All the surgeries were done by the first author. The surgeon always waits for several minutes until the gusher slows down considerably before attempting electrode insertion. In cases with CSF gusher, cochleostomies were done slightly larger (around 1.4 mm). In all patients, a watertight seal around the cochlear implant array was done with a customized technique using four to five pieces of fresh muscle. No other attempt was done. There was no case of persistent CSF leak or meningitis after the operations.
Discussion
The term ‘gusher’ is generally used in the literature to describe the egress of profuse clear fluid upon making
Figure 4
Cerebrospinal fluid gusher in cochlear implantation
Patient 6: Normal MRI.
an opening into the inner ear (Sennaroglu, 2010; Papsin, 2005). There are two different types of CSF outflow upon opening the cochlea. A gentle flow of clear fluid is called ‘oozing’ and a profuse flow is termed ‘gusher’ (Sennaroglu, 2010; Phelps et al., 1994). Oozing is an intermittent flow of CSF in small quantities which usually stops after a few minutes. The defect between the internal auditory canal and the malformed ear is small and the CSF outflow is easily controlled with soft tissue packing around the electrode. This type of CSF flow is more common in two types of inner ear anomalies; isolated large vestibular aqueduct and incomplete partition type II (Sennaroglu, 2010; Aschendorff et al., 1997). In cases of gusher, there is a wide communication between the subarachnoid space and the inner ear. In these patients, there is profuse CSF outflow upon opening the cochlea. It usually lasts between 10 to 20 minutes. This is the more serious type with more risk of causing post-operative meningitis
Patient 8: Normal imaging.
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Figure 6 Patient 1: Both sides show canal wall down mastoidectomy; right side: total ossification of the cochlea; left side: erosion of lateral semicircular canal.
(Sennaroglu, 2010). The incidence of CSF gusher in cochlear implantation is reported between 1 and 5% in large series (Wootten et al., 2006; Brito et al., 2012; Kempf et al., 1999; Ding et al., 2009; Kim et al., 2004). According to Papsin (2005), reports that include minor leaks may lead to an overestimate of the number of CSF gushers and may help explain the wide range in incidence of leaks in reports. In our series, out of 523 cochlear implantations including children and adults, 15 (2.87%) cases had CSF gusher. This is our incidence of true CSF gushers and cases with oozing or leak were not included in it. To our knowledge, 15 cases of CSF gusher are the largest reported series in the literature. This may be due to the large proportion of cochlear implantation in inner ear malformations in our center. Most of the literature addresses CSF gushers in the pediatric population with inner ear malformations (Wootten et al., 2006; Sennaroglu, 2010; Papsin, 2005). There are few reports of CSF gusher during cochlear implantation in adults (Wootten et al., 2006; Marks, 2004). Wootten et al. (2006) have reported six adults. One of them had radiographic features consistent with the X-linked progressive mixed deafness (‘gusher’) syndrome and the remaining five patients were pre-lingually deafened. Wootten did not describe any reason for CSF gusher occurrence in these five patients. Marks (2004) reported CSF gusher in one adult. His patient had progressive deafness over many years of unknown origin with normal anatomy of the cochlea in its pre-operative CT scan. Again, no reason for CSF gusher was mentioned. In our series, we had two post-lingually deafened adults. One of them ( patient 1) has had an episode of labyrinthitis due to cholesteatomatous erosion of his lateral semicircular canal (Fig. 5). Infective erosion of the cribriform plate causing abnormal communication between the cochlea and the subarachnoid
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space may be considered a reason but it still could be a point of discussion. Patient 6 had separate episodes of sudden sensorineural hearing loss in both the ears. Her imaging showed no abnormality. We do not have any explanation for its CSF gusher. According to Wootten et al. (2006), although rarer in normal ears, CSF leaks after inner ear opening are not limited to ears with obvious bony labyrinthine anomalies. They reported three cases with normal pre-operative imaging and an unremarkable history and physical examination. Marks (2004) reported three and Achiques et al. (2010) reported two patients. In an extensive review of the literature, we did not find any other clear reported case. So, it seems that it has a relatively uncommon occurrence in cochlear implant surgery. In this study, we reported three of these patients: one child with congenital hearing loss ( patient 8) and two adults with post-lingual hearing loss ( patients 1 and 6). Thus, cochlear implant surgeons should always be ready to manage this problem even in cases with unremarkable pre-operative imaging and history. The key point in the management of CSF gusher is to create a watertight seal around the entrance of cochlear implant array. Various types of materials for sealing are suggested in the literature. Achiques et al. (2010) use muscle. Loundon et al. (2008) use fragments of muscle with bioglue. Mylanus et al. (2004) use periosteum. Hoffman et al. (1997) use fascia. Wootten et al. (2006) use pieces of pressed fascia or periosteum. They believe that these are easier to manipulate in this small area and has less tendency to atrophy than muscle. Papsin (2005) packs the cochleostomy with four or five small pieces of temporalis fascia more tightly than usual and also considers bolstering his pack with Tisseel fibrin glue. Marks (2004) uses bone-waxed silk suture material. He cuts it into ∼1 cm segments. Then, using a Rosen needle,
Eftekharian and Amizadeh
the segments of waxed silk are carefully packed in a circular fashion around the electrode array in the cochleostomy. Sennaroglu (2010) although using pieces of the temporalis muscle, has also advocated a custom-made electrode array with a ‘cork’ stopper. We harvest a small round piece of fresh temporalis muscle, about 4–5 mm in diameter. The electrode is passed through a hole made in the center of the muscle block. When the electrode is inserted, first, we pack tightly the electrode array entrance (whether it is the round window or the cochleostomy) with three to four pieces of muscle then, the customshaped muscle is advanced to the level of the entrance around the electrode and is packed all around it. This completely surrounds the electrode at the level of the entrance. The cochleostomy size is another important issue. Weber et al. (1997) by advocating drilling of the smallest possible cochleostomy believe that this facilitates the closing of the fibrous tissue with fibrin glue. Loundon et al. (2005) also use the smallest possible cochleostomy. Conversely, most of the authors recommend a large cochleostomy, to make introduction and packing of soft tissue around the electrode easier (Sennaroglu, 2010; Papsin, 2005; Adunka et al., 2012). As stated before, in our department, the preferred technique of electrode insertion is the round window approach. Cochleostomy is chosen only when anatomically good view of the round window does not exist. We also believe that a relatively larger opening will make the introduction and packing of muscle around the electrode more precise. In round widow approach, the entrance diameter is already appropriate. In some cases, due to overhang of inferior lip of the round window, it may be necessary to remove a little bit of it (either by curette or drill) to improve inner ear visualization during electrode insertion. In cochleostomy approach, we make it a slightly larger (∼1.4 mm), and routinely wait several minutes (even up to 25 minutes) until the CSF gusher slows down considerably before attempting electrode insertion. Unlike Wootten et al. (2006), we do not place the patient in the reverse Trendelenburg position to slow the flow of CSF. We believe that this may make the CSF flow deceptively slow so, one cannot be assured that packing was effective or not. Additional measures are mentioned in the literature. These include, intraoperative lowering of the pCO2 and the administration of mannitol and post-operative oral acetazolamide and a head elevation (Loundon et al., 2005; Eisenman et al., 2001). For intractable cases, additional measures are also advocated by some authors. These include, temporary packing of the Eustachian tube with oxidized cellulose (Sennaroglu, 2010), packing the middle ear and the Eustachian tube with temporalis fascia or muscle
Cerebrospinal fluid gusher in cochlear implantation
with or without the use of fibrin glue (Wootten et al., 2006), and continuous lumbar CSF drain insertion for 4–5 days (Wootten et al., 2006; Sennaroglu, 2010; Papsin, 2005; Hoffman et al., 1997; Eisenman et al., 2001). It must be mentioned that it is difficult to maintain a lumbar CSF drain in a child so, unlike Syal et al. (2005) who advocated the insertion of an intraoperative continuous lumbar drain for all of the cases, we recommend it only when, in spite of tightly packing the electrode entrance, the CSF leak persists. In our series, all CSF gushers were controlled by our technique of the electrode entrance packing. In all patients, a complete insertion of all active electrodes was accomplished and subsequent functioning of the implants was satisfactory. No additional measures were needed and there have been no post-operative sequelae. We are agree with Adunka et al. (2012) that the management of the fluid gusher can be mainly performed intraoperatively during the initial surgery via tight packing and further non-surgical or surgical measures are rarely required to stop a persistent leak.
Conclusion CSF gusher may even occur in adults with post-lingual hearing loss and children with apparently unremarkable imaging and history. Thus, surgeons should always be ready to manage this problem. Management of CSF gusher should be mainly performed during the initial surgery by precise tight packing of the electrode entrance site. Furthermore, non-surgical or surgical measures are rarely required to stop a persistent leak. Our results show that our management technique may be recommended as well.
References Achiques M.T., Morant A., Muñoz N. 2010. Cochlear implant complications and failures. Acta Otorrinolaringológica Española, 61(6): 412–417. Adunka O.F., Teagle H.F.B., Zdanski C.J., Buchman C.A. 2012. Influence of an intraoperative perilymph gusher on cochlear implant performance in children with labyrinthine malformations. Otology & Neurotology, 33(9): 1489–1496. Aschendorff A., Marangos N., Laszig R. 1997. Large vestibular aqueduct syndrome and its implication for cochlear implant surgery. The American Journal of Otology, 18(6 Suppl.): pS57. Brito R., Monteiro T.A., Leal A.F., Tsuji R.K., Pinna M.H., Bento R.F. 2012. Surgical complications in 550 consecutive cochlear implantation. Brazilian Journal of Otorhinolaryngology, 78(3): 80–85. Ding X., Tian H., Wang W., Zhang D. 2009. Cochlear implantation in China: review of 1237 cases with an emphasis on complications. ORL (Journal for Oto-Rhino-Laryngology, Head and Neck Surgery), 71(4): 192–195. Eisenman D.J., Ashbaugh C., Zwolan T.A., Arts H.A., Telian S.A. 2001. Implantation of the malformed cochlea. Otology & Neurotology, 22(6): 834–841. Hoffman R.A., Downey L.L., Waltzman S.B., Cohen N.L. 1997. Cochlear implantation in children with cochlear malformations. The American Journal of Otology, 18(2): 184–187. Kempf H.G., Tempel S., Johann K., Lenarz T. 1999. Complications of cochlear implant surgery in children and adults. LaryngoRhino-Otology, 78(10): 529–537.
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Kim C.S., Ju Kwon B., Chang S.O., Oh S.A., Lee H.J., Choi B.Y., et al. 2004. CSF gusher in cochlear implantation. Cochlear Implants International, 5(Suppl. 1): 67–69. Loundon N., Rouillon I., Munier N., Marlin S., Roger G., Garabedian E.N. 2005. Cochlear implantation in children with internal ear malformations. Otology & Neurotology, 26(4): 668–673. Loundon N., Leboulanger N., Maillet J., Riggouzzo A., Richard P., Marlin S., et al. 2008. Cochlear implant and inner ear malformation. Proposal for an hyperosmolar therapy at surgery. International Journal of Pediatric Otorhinolaryngology, 72(4): 541–547. Marks H.W. 2004. Simple method to control a cerebrospinal fluid gusher during cochlear implant surgery. Otology & Neurotology, 25(4): 483–484. Mylanus E.A., Rotteveel L.J., Leeuw R.L. 2004. Congenital malformation of the inner ear and pediatric cochlear implantation. Otology & Neurotology, 25(3): 308–317.
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Papsin B.C. 2005. Cochlear implantation in children with anomalous cochleovestibular anatomy. The Laryngoscope, 115(Suppl. 106): 1–26. Phelps P.D., King A., Michaels L. 1994. Cochlear dysplasia and meningitis. The American Journal of Otology, 15(4): 551–557. Sennaroglu L. 2010. Cochlear implantation in inner ear malformations–a review article. Cochlear Implants International, 11(1): 4–41. Syal R., Tyagi I., Goyal A. 2005. Cerebrospinal fluid otorhinorrhea due to cochlear dysplasias. International Journal of Pediatric Otorhinolaryngology, 69(7): 983–988. Weber B.P., Lenarz T., Dillo W., Maneke I., Bertram B. 1997. Malformations in cochlear implant patients. The American Journal of Otology, 18 (6 Suppl.): S64–S65. Wootten C.T., Backous D.D., Haynes D.S. 2006. Management of cerebrospinal fluid leakage from cochleostomy during cochlear implant surgery. The Laryngoscope, 116(11): 2055–2059.
Cerebrospinal fluid gusher in cochlear implantation Ali Eftekharian, Maryam Amizadeh Department of Otolaryngology, Shahid-Beheshti University of Medical Sciences, Tehran, Iran Objectives: To share our experience of cerebrospinal fluid (CSF) gusher in cochlear implantation. Methods: Demographic, radiological, and surgical results of patients with CSF gusher in 523 consecutive cochlear implant recipients including children and adults as well as our management technique were evaluated and a review of the literature has been included. Results: Fifteen (2.87%) cases had CSF gusher. Two patients (13.3%) were adults with post-lingual hearing loss and the rest 12 (86.7%) were children with congenital hearing loss. Twelve patients (80%) had various types of inner ear malformation. Three patients (20%) had no predictable risk of CSF gusher from history or pre-operative imaging. In all patients, CSF gushers were controlled with our technique of packing the electrode entrance site with no additional measures. Conclusion: CSF gusher may occur with post-lingual hearing loss and in children with apparently unremarkable imaging and history. Thus, surgeons should always be ready to manage it. Management of CSF gusher can be mainly performed during the initial surgery by precise tight packing of the electrode entrance site. Furthermore, non-surgical or surgical measures are rarely required to stop a persistent leak. Our results show that our management technique may be recommended as well. Keywords: Cochlear implant, CSF gusher, Management, Inner ear malformation, Complication
Introduction
Materials and methods
The incidence of cerebrospinal fluid (CSF) gusher in cochlear implantation is reported between 1 and 5% in large series (Wootten et al., 2006; Brito et al., 2012; Kempf et al., 1999; Ding et al., 2009; Kim et al., 2004). Most of the literature addresses CSF gushers in the pediatric population during cochleostomy and there are few reports of its occurrence in adults (Wootten et al., 2006). Failure to create a watertight seal around the cochlear implant array would create a risk for future CSF leakage and produce a potential hazard for meningitis. Various techniques have been reported to seal a CSF leak around a cochlear implant array. These methods include packing muscle or connective tissue around the implant, sealing the muscle with fibrin glue, packing with several strips of bone-waxed silk suture material plus muscle (Marks, 2004), and the use of a specially designed electrode array (Sennaroglu, 2010). Here, we report our radiological, surgical, and management experience in CSF gusher at cochlear implantation.
Between July 2008 and May 2013, 523 cochlear implant procedures including children and adults have been done in our department. Their clinical records were reviewed and the data of patients who had CSF gusher were enrolled in this study. Only those who had profuse CSF outflow upon opening the cochlea which usually last several minutes were considered. Cases with pulsatile perilymph, oozing, or leaks that are sometimes found on opening the cochlea, for example, in those with an isolated large vestibular aqueduct, were not enrolled in this study. Demographic, radiological, and surgical results of these patients as well as our management technique are evaluated.
Correspondence to: Ali Eftekharian, Department of Otolaryngology, Loghman Hospital, Kamali Street, South Kargar Ave. 13336-35445, Tehran, Iran. E-mail: [email protected]
© W. S. Maney & Son Ltd 2014 DOI 10.1179/1754762814Y.0000000069
Results Out of 523 cochlear implantations including children and adults, 15 (2.87%) cases had CSF gusher who also met our inclusion and exclusion criteria. Their data are shown in Table 1. Two patients (13.3%) were adults with post-lingual hearing loss and the rest 12 (86.7%) were children with congenital hearing loss. Highresolution computed tomography (CT) scans in three patients (20%) did not show any obvious anomaly to predict CSF gusher occurrence in the operation. Twelve patients (80%) had various types of inner ear
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Table 1
Patient’s demographics
Patient
Sex
Age (year)
Type of deafness
Op side
1
M
42
Post
L
2 3 4 5 6 7 8 9 10 11 12 13 14 15
F M F F F F M M M F F M F M
8 9 9 5 53 14 2 5 6 6 2 4 2 2
Pre Pre Pre Pre Post Pre Pre Pre Pre Pre Pre Pre Pre Pre
R R R L R L R R L R L L R L
CT scan findings Right side: total ossification Left side: just erosion of lateral semicircular canal Incomplete partition type I Incomplete partition type III Incomplete partition type I Common cavity Normal Incomplete partition type I Normal Incomplete partition type I Incomplete partition type I Incomplete partition type I Mondini deformity Mondini deformity Incomplete partition type I Incomplete partition type I
Electrode entrance
Device
Follow-up (months)
R.W
HiRes90k1j
46
C R.W R.W C R.W R.W R.W C R.W R.W R.W R.W R.W R.W
CI24R(ST) CI24RE HiRes90k1j HiRes90k1j HiRes90k1j CI24RE CI24RE HiRes90k1j HiRes90k1j HiRes90k1j CI24RE HiRes90k1j HiRes90k1j CI24RE
42 33 19 19 18 18 15 9 8 6 5 3 3 2
M, male; F, female; Op side, operated side; L, left; R, right; Post, post-lingual; Pre, pre-lingual; R.W, round window; C, cochleostomy.
Figure 1 Patient 11: Incomplete partition type I.
malformation. Eight cases (66.7% of malformations) were incomplete partition type I. Fig. 1 shows the CT scan of one of them (patient 11). Two patients had a Mondini deformity (Fig. 2 shows the CT scan of one of them; patient 12). One child had common cavity deformity and one child had X-linked deafness or incomplete partition type III (patient 3, Fig. 3). One child with congenital deafness ( patient 8) had imaging that seems to be normal (Fig. 4). Patient 6 was a 53-year-old female. She suddenly lost her left-
Figure 2 Patient 12: Mondini deformity.
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side hearing 28 years ago during her second pregnancy without any clear reason. Seven years later, her rightside hearing was abruptly lost. She claimed that it occurred after her father’s funeral. There is also no obvious reason for this side deafness. Fig. 5 demonstrates one of her magnetic resonance imaging (MRI) images. Patient 1 was a 42-year-old man with bilateral profound hearing loss. His right side was operated on 30 years ago possibly for a cholesteatoma but there are no clear data about his hearing loss. His left side was operated on for an acquired cholesteatoma in another center 6 months before admission in our center. His clinical history revealed that his left hearing was abruptly deteriorated after its operation. On the right side, the cochlea was nearly completely ossified and on the left side, the only apparent defect was an erosion of the lateral semicircular canal (Fig. 6). In our department, the preferred technique of electrode insertion is the round window approach. Cochleostomy is chosen just when anatomically adequate view of the round window does not exist. In 12 patients (80%), round window was the route of
Eftekharian and Amizadeh
Figure 5
Figure 3
Patient 3: Incomplete partition type III.
electrode insertion. In three remaining ones, cochleostomy was used anteroinferior to the round window. All the surgeries were done by the first author. The surgeon always waits for several minutes until the gusher slows down considerably before attempting electrode insertion. In cases with CSF gusher, cochleostomies were done slightly larger (around 1.4 mm). In all patients, a watertight seal around the cochlear implant array was done with a customized technique using four to five pieces of fresh muscle. No other attempt was done. There was no case of persistent CSF leak or meningitis after the operations.
Discussion
The term ‘gusher’ is generally used in the literature to describe the egress of profuse clear fluid upon making
Figure 4
Cerebrospinal fluid gusher in cochlear implantation
Patient 6: Normal MRI.
an opening into the inner ear (Sennaroglu, 2010; Papsin, 2005). There are two different types of CSF outflow upon opening the cochlea. A gentle flow of clear fluid is called ‘oozing’ and a profuse flow is termed ‘gusher’ (Sennaroglu, 2010; Phelps et al., 1994). Oozing is an intermittent flow of CSF in small quantities which usually stops after a few minutes. The defect between the internal auditory canal and the malformed ear is small and the CSF outflow is easily controlled with soft tissue packing around the electrode. This type of CSF flow is more common in two types of inner ear anomalies; isolated large vestibular aqueduct and incomplete partition type II (Sennaroglu, 2010; Aschendorff et al., 1997). In cases of gusher, there is a wide communication between the subarachnoid space and the inner ear. In these patients, there is profuse CSF outflow upon opening the cochlea. It usually lasts between 10 to 20 minutes. This is the more serious type with more risk of causing post-operative meningitis
Patient 8: Normal imaging.
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Figure 6 Patient 1: Both sides show canal wall down mastoidectomy; right side: total ossification of the cochlea; left side: erosion of lateral semicircular canal.
(Sennaroglu, 2010). The incidence of CSF gusher in cochlear implantation is reported between 1 and 5% in large series (Wootten et al., 2006; Brito et al., 2012; Kempf et al., 1999; Ding et al., 2009; Kim et al., 2004). According to Papsin (2005), reports that include minor leaks may lead to an overestimate of the number of CSF gushers and may help explain the wide range in incidence of leaks in reports. In our series, out of 523 cochlear implantations including children and adults, 15 (2.87%) cases had CSF gusher. This is our incidence of true CSF gushers and cases with oozing or leak were not included in it. To our knowledge, 15 cases of CSF gusher are the largest reported series in the literature. This may be due to the large proportion of cochlear implantation in inner ear malformations in our center. Most of the literature addresses CSF gushers in the pediatric population with inner ear malformations (Wootten et al., 2006; Sennaroglu, 2010; Papsin, 2005). There are few reports of CSF gusher during cochlear implantation in adults (Wootten et al., 2006; Marks, 2004). Wootten et al. (2006) have reported six adults. One of them had radiographic features consistent with the X-linked progressive mixed deafness (‘gusher’) syndrome and the remaining five patients were pre-lingually deafened. Wootten did not describe any reason for CSF gusher occurrence in these five patients. Marks (2004) reported CSF gusher in one adult. His patient had progressive deafness over many years of unknown origin with normal anatomy of the cochlea in its pre-operative CT scan. Again, no reason for CSF gusher was mentioned. In our series, we had two post-lingually deafened adults. One of them ( patient 1) has had an episode of labyrinthitis due to cholesteatomatous erosion of his lateral semicircular canal (Fig. 5). Infective erosion of the cribriform plate causing abnormal communication between the cochlea and the subarachnoid
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space may be considered a reason but it still could be a point of discussion. Patient 6 had separate episodes of sudden sensorineural hearing loss in both the ears. Her imaging showed no abnormality. We do not have any explanation for its CSF gusher. According to Wootten et al. (2006), although rarer in normal ears, CSF leaks after inner ear opening are not limited to ears with obvious bony labyrinthine anomalies. They reported three cases with normal pre-operative imaging and an unremarkable history and physical examination. Marks (2004) reported three and Achiques et al. (2010) reported two patients. In an extensive review of the literature, we did not find any other clear reported case. So, it seems that it has a relatively uncommon occurrence in cochlear implant surgery. In this study, we reported three of these patients: one child with congenital hearing loss ( patient 8) and two adults with post-lingual hearing loss ( patients 1 and 6). Thus, cochlear implant surgeons should always be ready to manage this problem even in cases with unremarkable pre-operative imaging and history. The key point in the management of CSF gusher is to create a watertight seal around the entrance of cochlear implant array. Various types of materials for sealing are suggested in the literature. Achiques et al. (2010) use muscle. Loundon et al. (2008) use fragments of muscle with bioglue. Mylanus et al. (2004) use periosteum. Hoffman et al. (1997) use fascia. Wootten et al. (2006) use pieces of pressed fascia or periosteum. They believe that these are easier to manipulate in this small area and has less tendency to atrophy than muscle. Papsin (2005) packs the cochleostomy with four or five small pieces of temporalis fascia more tightly than usual and also considers bolstering his pack with Tisseel fibrin glue. Marks (2004) uses bone-waxed silk suture material. He cuts it into ∼1 cm segments. Then, using a Rosen needle,
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the segments of waxed silk are carefully packed in a circular fashion around the electrode array in the cochleostomy. Sennaroglu (2010) although using pieces of the temporalis muscle, has also advocated a custom-made electrode array with a ‘cork’ stopper. We harvest a small round piece of fresh temporalis muscle, about 4–5 mm in diameter. The electrode is passed through a hole made in the center of the muscle block. When the electrode is inserted, first, we pack tightly the electrode array entrance (whether it is the round window or the cochleostomy) with three to four pieces of muscle then, the customshaped muscle is advanced to the level of the entrance around the electrode and is packed all around it. This completely surrounds the electrode at the level of the entrance. The cochleostomy size is another important issue. Weber et al. (1997) by advocating drilling of the smallest possible cochleostomy believe that this facilitates the closing of the fibrous tissue with fibrin glue. Loundon et al. (2005) also use the smallest possible cochleostomy. Conversely, most of the authors recommend a large cochleostomy, to make introduction and packing of soft tissue around the electrode easier (Sennaroglu, 2010; Papsin, 2005; Adunka et al., 2012). As stated before, in our department, the preferred technique of electrode insertion is the round window approach. Cochleostomy is chosen only when anatomically good view of the round window does not exist. We also believe that a relatively larger opening will make the introduction and packing of muscle around the electrode more precise. In round widow approach, the entrance diameter is already appropriate. In some cases, due to overhang of inferior lip of the round window, it may be necessary to remove a little bit of it (either by curette or drill) to improve inner ear visualization during electrode insertion. In cochleostomy approach, we make it a slightly larger (∼1.4 mm), and routinely wait several minutes (even up to 25 minutes) until the CSF gusher slows down considerably before attempting electrode insertion. Unlike Wootten et al. (2006), we do not place the patient in the reverse Trendelenburg position to slow the flow of CSF. We believe that this may make the CSF flow deceptively slow so, one cannot be assured that packing was effective or not. Additional measures are mentioned in the literature. These include, intraoperative lowering of the pCO2 and the administration of mannitol and post-operative oral acetazolamide and a head elevation (Loundon et al., 2005; Eisenman et al., 2001). For intractable cases, additional measures are also advocated by some authors. These include, temporary packing of the Eustachian tube with oxidized cellulose (Sennaroglu, 2010), packing the middle ear and the Eustachian tube with temporalis fascia or muscle
Cerebrospinal fluid gusher in cochlear implantation
with or without the use of fibrin glue (Wootten et al., 2006), and continuous lumbar CSF drain insertion for 4–5 days (Wootten et al., 2006; Sennaroglu, 2010; Papsin, 2005; Hoffman et al., 1997; Eisenman et al., 2001). It must be mentioned that it is difficult to maintain a lumbar CSF drain in a child so, unlike Syal et al. (2005) who advocated the insertion of an intraoperative continuous lumbar drain for all of the cases, we recommend it only when, in spite of tightly packing the electrode entrance, the CSF leak persists. In our series, all CSF gushers were controlled by our technique of the electrode entrance packing. In all patients, a complete insertion of all active electrodes was accomplished and subsequent functioning of the implants was satisfactory. No additional measures were needed and there have been no post-operative sequelae. We are agree with Adunka et al. (2012) that the management of the fluid gusher can be mainly performed intraoperatively during the initial surgery via tight packing and further non-surgical or surgical measures are rarely required to stop a persistent leak.
Conclusion CSF gusher may even occur in adults with post-lingual hearing loss and children with apparently unremarkable imaging and history. Thus, surgeons should always be ready to manage this problem. Management of CSF gusher should be mainly performed during the initial surgery by precise tight packing of the electrode entrance site. Furthermore, non-surgical or surgical measures are rarely required to stop a persistent leak. Our results show that our management technique may be recommended as well.
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