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.

BACKGROUND: To report the presentation, diagnostic process, management and results of cochlear implantation of patients diagnosed with Bjornstad syndrome with profound sensorineural hearing loss (SNHL).
UNASSIGNED: A retrospective report of two siblings with Bjornstad syndrome suffering profound SNHL unresponsive to conventional hearing aids treated with bilateral simultaneous cochlear implantation.
METHODS: Tertiary-referral center.
RESULTS: Cochlear implant surgeries of two siblings (four ears) with profound SNHL and bilateral inner ear anomaly (incomplete partition type 1) were performed without complications. Postoperative audiometric measurements showed a significiant improvement in pure-tone threshold and a word recognition score. In the literature review, no previous case of Bjornstad syndrome treated with cochlear implantation has been reported.
CONCLUSIONS: Cochlear implantation is an effective, safe, and ultimate treatment option for Bjornstad syndrome with profound SNHL not responding to hearing aids.

BACKGROUND: There are particular challenges in the implantation of malformed cochleae, such as in cases of facial nerve anomalies, cerebrospinal fluid (CSF) leaks, erroneous electrode insertion, or facial stimulation, and the outcomes may differ depending on the severity of the malformation. The aim of this study was to assess the impact of inner ear malformations (IEMs) on surgical complications and outcomes of cochlear implantation.
METHODS: In order to assess the impact of IEMs on cochlear implant (CI) outcomes, 2 groups of patients with similar epidemiological parameters were selected from among 863 patients. Both the study group (patients with an IEM) and control group (patients with a normal inner ear) included 25 patients who received a CI and completed at least 1 year of follow-up. Auditory performance, receptive and expressive language skills, and production and use of speech were evaluated preoperatively and at least 1 year after implantation. Types of surgical complications and rates of revision surgeries were determined in each group.
RESULTS: In the study group, the most common malformation was an isolated enlarged vestibular aqueduct (EVA) (44.8%). Overall, the patients with IEMs showed significant improvement in auditory-verbal skills. In general, the patients who had normal cochleae scored significantly better compared to patients with IEMs (p < 0.05). The complication rate was significantly lower in the control group compared to the study group (p = 0.001), but the rate of revision surgeries did not differ significantly (p = 0.637).
CONCLUSIONS: It is possible to improve communication skills with CIs in patients with IEMs despite the variations in postoperative performances. Patients with EVA, incomplete partition type 2, and cochlear hypoplasia type 2 were the best performers in terms of auditory-verbal skills. Patients with IEMs scored poorly compared to patients with normal cochleae. CSF leak (gusher or oozing) was the most common complication during surgery, which is highly likely in cases of incomplete partition type 3.

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.

UNASSIGNED: To present a report on the incidence and management protocol of cerebrospinal fluid gusher in cochlear implantation in the national institute of respiratory diseases.
UNASSIGNED: We conducted a clinical, longitudinal, retrospective investigation of all the medical charts from implanted patients between October 1999 and December 2016 in the national institute of respiratory diseases to evaluate the incidence of gusher, management protocol and evolution after surgery. Statistical analysis was conducted using Fishers test to find out if there was a relationship between the number of inner ear malformations and gusher severity and between the surgical access to the inner ear (cochleostomy or round window) and gusher severity.
UNASSIGNED: 276 patients were evaluated, 12 (4.3%) presented gusher during cochlear implantation. Seven had minimal gusher that stopped completely with a fascia seal, three had moderate gusher that required fascia and cartilage and two had severe gusher that required middle ear packing and obliteration of the eustachian tube. There was no statistically significant relationship between the number of inner ear malformations or the surgical access to the inner ear with gusher severity.
UNASSIGNED: A thorough evaluation of the imaging study should always be conducted, it should include specific measurements and an individualized management in accordance with gusher severity should always be performed.
UNASSIGNED: Presentar un reporte de la incidencia y del protocolo de manejo del gusher del líquido cefalorraquídeo durante la implantación coclear en el Instituto Nacional de Enfermedades Respiratorias.
UNASSIGNED: Se realizó una investigación clínica, longitudinal y retrospectiva de los expedientes clínicos de todos los pacientes con implantación coclear entre octubre de 1999 y diciembre de 2016, para valorar la incidencia de Gusher, el protocolo de manejo y la evolución posterior. Se realizó el análisis estadístico utilizando la prueba exacta de Fisher para buscar si había relación entre el número de malformaciones de oído interno y el grado de Gusher, y entre la vía de abordaje al oído interno (cocleostomía o ventana redonda) y el grado de Gusher.
UNASSIGNED: Se valoraron 276 pacientes, de los cuales 12 (4.3%) presentaron Gusher durante la implantación coclear. De estos, siete fueron Gusher mínimo y cedió totalmente con el sellado con fascia, tres fueron Gusher moderado que ameritó el sellado con fascia y cartílago, y dos fueron Gusher grave que ameritó empaquetamiento de oído medio y obliteración de la trompa de Eustaquio. No se encontró una relación estadísticamente significativa entre el número de malformaciones del oído interno o la vía de abordaje al oído interno con el grado de Gusher.
UNASSIGNED: Debe realizarse una minuciosa evaluación del estudio de imagen prequirúrgico, que incluya la toma de mediciones específicas, y brindar un manejo individualizado según la gravedad del Gusher.

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.