UNASSIGNED: Since 20% of the patients with sensorineural hearing loss have confirmed radiographically inner ear malformation, this study aimed to compare the incidence rate of intra and postoperative complications of cochlear implantation amongst the patients with or without inner ear malformations.
UNASSIGNED: In this retrospective study, we evaluated the medical record of 954 patients who had undergone cochlear implantation. Seventeen patients had inner ear malformations and were selected as the case group, and 25 patients with normal inner ear were selected as the control group. Patient\'s information; including intraoperative complications, post-operative complications and neural response telemetry (NRT) immediately after the surgery were recorded. Finally, the collected data were analyzed, using the SPSS software, version 21.
UNASSIGNED: According to the CT-scan findings, the most observed internal ear abnormality was the temporal bone Mondini in 8 patients (47%), and the next inline was the common cavity at a frequency of (23.52%). Cerebrospinal fluid (CSF) gusher was detected in 11 patients (64.7%) of the case group, which was significantly higher than the other group. This includes mild CSF gusher in 7 cases (41.17%) and severe CSF gusher in 5 cases (29.41%). Interestingly, no significant post-operative complications were observed in either group, minor side effects were limited and not significantly different.
UNASSIGNED: In conclusion, despite the limitations of cochlear implantation surgery amongst patients with inner ear malformation with potential risk of early or late complications, it can still be recommended as an appropriate procedure to acquire hearing as well as auditory and speech perception.

Introduction: Cochlear implants (CI) is considered a safe procedure with overall complication rate of 12.5% amongst adult and pediatric population. Cerebrospinal fluid (CSF) gusher is regarded as a common CI intraoperative complication.Objective: In this study, we determined the association between probable associated factors and occurrence of intraoperative CSF gusher.Method: In a retrospective survey, 394 patients with severe to profound sensorineural hearing loss who underwent primary cochlear implantation surgery were evaluated. Patients with incomplete electronic or manual file and those with revision, explantation or reimplantation surgery were excluded.Result: three hundred and ninety-four CI patients with a mean age of 8.74 ± 12.21 years were reviewed 49.62% of them were female. CSF gusher developed in 22 patients (5.58%). Patients with CSF gusher had more structural abnormalities in their CT scans\' report. Common cavity malformation and Mondini dysplasia were the most common abnormal reports amongst those with intra-operative CSF gusher.Conclusion: In conclusion, cochlea structural abnormalities affect the incidence of CSF gusher in CI recipients. Common cavity malformation and Mondini dysplasia were associated with an increased incidence of CSF gusher. We insist on more precise pre-operative imaging of those with abnormal cochlea structures to provide the needed management.

OBJECTIVE: The aim of this study is to perform a systematic literature review on the occurrence of gusher during stapes surgery, to understand its surgical management and outcomes.
METHODS: The PRISMA standard was applied to identify English, Italian or French-language studies, related to stapes surgery and mentioning gusher or perilymphatic leak. Full-texts lacking information on the management of gusher and/or the post-operative hearing outcome were excluded.
RESULTS: Twenty-four articles were eventually included. Seventy-six patients were involved in the qualitative synthesis. The management of gusher mostly consisted in covering the oval window and/or filling the tympanic cavity, with absorbable and autologous graft materials. Packing of the external auditory canal was reported in 51 patients (67%). Gusher was related to complete/profound loss of hearing in 25% of the cases and to a worsening of hearing function in 31% of patients. In 19% of patients an improvement in hearing tests was reported; in 28% the hearing function was unchanged. Post-operative vestibular symptoms were reported in 7 patients, and were mainly mild and transient. The absence of vestibular symptoms was underlined in 9 cases, while in 79% of the patients the authors did not provide information.
CONCLUSIONS: The unexpected occurrence of gusher during stapes surgery represents a relevant issue for the otologic surgeon. Its management most commonly consists in plugging the oval window and the tympanic cavity. In most of the cases, a stapes prosthesis could be positioned. The results on hearing and vestibular functions are widely variable.

OBJECTIVE: To share our experience in cochlear implanted patients with incomplete partition type I, to compare it with the literature results and to disclose difficulties facing cochlear implant teams dealing with these patients.
METHODS: Clinical records of 1089 cochlear implant procedures in a cochlear implant center were reviewed and data of patients who had incomplete partition type I were enrolled in this study. Their auditory and speech performances were evaluated 3 years after the implantation.
RESULTS: Eighteen cases (1.65%) had incomplete partition type I. Cerebrospinal fluid gusher was encountered during opening the cochlea in 15 patients (83.3%). There were no cases of persistent CSF leak or postoperative meningitis. In 61.1% of patients, some additional anomalies were found during the operation. Although in 55.6% of cases no electrically evoked compound action potential was detected even in long-term follow-up, all patients had satisfactory auditory and speech outcome.
CONCLUSIONS: Cochlear implantation is a relatively safe and effective treatment for patients who have incomplete partition type I, even if the procedure may be somehow challenging.

Patients with X-linked deafness carry mutations in the POU3F4 gene and have pathognomonic inner ear malformations characterised by symmetrical incomplete partition type 3 (absent modiolus and lamina spiralis but preserved interscalar septum in a normal-sized cochlea) and large internal auditory meatus (IAM) with an increased risk of gusher during stapes surgery. We describe a range of fairly characteristic malformations in the hypothalamus of some patients with this rare condition, ranging from subtle asymmetric appearance and thickening of the tuber cinereum to more marked hypothalamic enlargement. We discuss the role of POU3F4 in the normal development of both the inner ear and hypothalamus and the proposed pathophysiology of incomplete partition type 3.

To investigate and compare residual hearing preservation between patients based on the presence of intraoperative gusher.
We retrospectively compared 2 cohorts of cochlear implant recipients significantly distinguished by whether or not they experienced gusher intraoperatively. Patients underwent cochlear implantation using 24-mm lateral wall electrode arrays as well pharmacologic steroid protection. All patients were assessed by a hearing implant MDT. Hearing preservation rates and speech perception outcomes were assessed at 1, 6, 12, 24, 36, 48, and 60 months.
The patients with no gusher demonstrated complete hearing preservation. The patients with gusher demonstrated significant postoperative reduction of hearing thresholds, which declined at a significantly higher pace during follow-up. All patients demonstrated significantly better speech performance after cochlear implantation.
The present study suggests that intraoperative gusher is associated with a significant drop in residual hearing, both immediately and over time, which may be related to the large change in intracochlear pressure intraoperatively.

OBJECTIVE: To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA).
BACKGROUND: Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications.
METHODS: Retrospective review of patients\' medical records and CT/MRI.
METHODS: Tertiary referral center.
METHODS: The medical records and CT/MRI images of 55 PS/NSEVA patients receiving 80 cochlear implantations from 1982-2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved.
RESULTS: Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo.
CONCLUSIONS: Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.

OBJECTIVE: Gusher in inner ear malformations is common in patients with incomplete partition type I and type III. It is also common in less severe form as oozing in incomplete partition type II and large vestibular aqueduct. It is important to prevent cerebrospinal fluid (CSF) escape around the electrode to prevent meningitis.
METHODS: The custom-made device was produced by Med-El Company. It has a \"cork\"-like stopper instead of the usual silicon ring to prevent gusher. There are two types of electrodes of different lengths. The standard one is 25mm (contact space 1.7mm) and the short one is 20mm (contact space 1.3mm). It was used in 50 patients with different inner ear malformations.
RESULTS: Thirteen patients had gusher, and 11 patients oozing during cochleostomy. One patient with initial prototype of the cork electrode had to be revised because of persistent oozing around the electrode. Another patient had slow extrusion of the electrode most probably due to CSF pulsation and had to be revised. Both patients had no more CSF fistula.
CONCLUSIONS: CSF fistula in inner ear malformations is a serious situation which may lead to recurrent meningitis. The new electrode with \"cork\" stopper looks promising in preventing the postoperative CSF leak around the electrode.