Cochlear implantation outcomes in patients with far advanced otosclerosis,,,,

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1 Available online at American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) Cochlear implantation outcomes in patients with far advanced otosclerosis,,,, Maroun T. Semaan, MD a,, Neal C. Gehani, MD a, Neelima Tummala, MS b, Carolyn Coughlan, MS b, Souha A. Fares, PhD c, Daniel P. Hsu, MD d, Gail S. Murray, PhD a, William H. Lippy, MD e, Cliff A. Megerian, MD, FACS a a Department of Otolaryngology-Head and Neck Surgery, University Hospitals Case, Medical Center, Cleveland, OH, USA b Case Western Reserve University School of Medicine, Cleveland, OH, USA c Department of Statistics, Case Western Reserve University, Cleveland, OH, USA d Department of Radiology, University Hospitals Case Medical Center, Cleveland, OH, USA e The Lippy Group for Ear, Nose and Throat, Warren, OH, USA Received 5 May 2012 Abstract Objectives: To compare hearing outcomes in patients with far advanced otosclerosis (FAO) undergoing cochlear implantation to an age-matched group of controls, to describe the effects of cochlear ossification on hearing, and to review the adverse effects of implantation in patients with FAO. Hypothesis: Hearing performance in patients with FAO after cochlear implantation is comparable to similarly treated postlingually deafened adults without FAO. Ossification or retrofenestral otosclerosis does not predict poor hearing outcomes. Modiolar-hugging technology reduces postoperative facial nerve stimulation. Study Design: Retrospective chart review. Setting: Academic neurotologic tertiary referral center. Patients: Thirty patients with FAO, who metaudiological criteria for cochlear implantation, were compared to 30 age-matched controls, postlingually deafened by non-otosclerotic causes. Main Outcome Measures: Audiometric pre- and postoperative speech reception threshold, word, and sentence scores were analyzed. The presence of retrofenestral findings on computed tomography or intraoperative cochlear ossification were noted. Results: In the FAO group, radiographic abnormalities were noted in 26.4% of patients. Intraoperative ossification requiring drillout was seen in 29.4% of patients. None developed postoperative facial nerve stimulation. There was no difference between the FAO and control groups in the mean short-term and long-term postoperative speech reception threshold, word, and sentence scores (P =.77). The presence of radiographic abnormalities did not predict hearing outcome. Intraoperative cochlear ossification was not associated with worse short-term word and sentence scores (P =.58 and 0.79, respectively), and for the long-term hearing outcome (P =.24). Conclusions: In patients with FAO, effective and safe hearing rehabilitation can be accomplished with cochlear implantation Elsevier Inc. All rights reserved. Accepted for oral presentation at the 115th Annual Meeting at COSM, April 2012, in San Diego, CA. Each of the authors has contributed to, read and approved this manuscript. None of the authors has any conflict of interest, financial or otherwise. This manuscript is original and it, or any part of it, has not been previously published; nor is it under consideration for publication elsewhere. A copyright transfer letter signed by each author is provided. Corresponding author. Department of Otolaryngology-Head and Neck Surgery, University Hospitals Case Medical Center, Cleveland, OH, USA. address: maroun.semaan@uhhospitals.org (M.T. Semaan) /$ see front matter 2012 Elsevier Inc. All rights reserved. doi: /j.amjoto

2 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) Introduction Otosclerosis is a disease that affects the endochondral bone of the otic capsule. An otosclerotic lesion is characterized by disordered resorption and deposition of bone. Histologically, it consists of areas of bone resorption, new bone formation, vascular proliferation, and a connective tissue stroma. Otosclerosis occurs at sites of predilection within the otic capsule. The most common site is the fissula ante fenestram located anterior to oval window [1]. Clinical otosclerosis occurs when a lesion involves the stapes or stapediovestibular joint, resulting in conductive hearing loss. The metabolic condition may eventually progress, resulting in sensorineural hearing loss (SNHL) or cochlear otosclerosis. House and Sheehy first described far advanced otosclerosis (FAO) in 1961 as clinical otosclerosis with air conduction thresholds over 85 db and immeasurable bone conduction thresholds due to the limits of audiometers at the time [2]. In 1992, Iurato et al described very far advanced otosclerosis (VFAO) as patients with otosclerosis with undetectable air and bone conduction levels with standard clinical audiometers, resulting in a blank audiogram [3]. It has been estimated that 1.6% of patients with otosclerosis will eventually develop profound SNHL [4]. Stapedectomy or stapedotomy with hearing aid amplification has been proposed as a first-line treatment for patients with FAO, as it has been shown to be effective in 60% to 80% of patients with FAO and in 42.3% of patients with VFAO [4-6]. Lippy et al showed that within 1 month of stapedectomy, word recognition score (WRS) improvements of 16.5% were observed for all patients [7]. Furthermore, improvements were shown to continue past the immediate postoperative period. After 2 years, WRS continued to improve by an additional 12% in 71% of patients. This was thought to be consistent with acclimatization or recovery from auditory deprivation after hearing restoration. Although stapes surgery provides a good initial treatment option for hearing restoration in patients with otosclerosis, several studies have demonstrated that cochlear implantation may be more effective for aural rehabilitation in patients with FAO and VFAO [5,8-11]. Cochlear implantation, regardless of the etiology of SNHL, has shown an improvement of WRS in the immediate postoperative period, which continued to improve in long-term follow-up [12,13]. It is well established that patients with FAO and VFAO undergoing cochlear implantation demonstrate significant improvement in the short term; however, the extent of hearing restoration in the long term has not been reported, and the likelihood for the need for drillout of basilar turn ossification has not been quantified. In addition, an elevated incidence of facial nerve stimulation after cochlear implantation has been reported in patients with otosclerosis [14]. The objective of this study is to evaluate the short- and long-term hearing outcomes of cochlear implantation in patients with FAO. In order to analyze the outcome, we compared a group of patients with FAO to a randomly selected group of adult controls, postlingually deafened by other etiologies. We hypothesized that there is no difference in long-term hearing outcome between the study groups. In addition, we analyzed the incidence of adverse effects, in particular, the incidence of facial nerve stimulation, and whether preoperative radiographic findings on high-resolution computed tomography (CT) of the temporal bone predicted postoperative hearing outcomes or complications. 2. Materials and methods 2.1. Subjects After departmental and institutional review board approval, the cochlear implant database at University Hospitals, Case Medical Center was reviewed (IRB ). Only patients who had undergone standard evaluation for cochlear implant candidacy were included in this database. Standard evaluation includes comprehensive audiologic testing, counseling, and preoperative CT or magnetic resonance imaging. This study is a retrospective chart review of patients from this database. Patients with the diagnosis of obliterative, nonobliterative, and cochlear otosclerosis (ICD , 387.1, and 387.2, respectively) that underwent cochlear implantation between January 2003 and March 2011 were included in this study. The diagnosis of FAO was made clinically on the basis of a history of slowly progressive unilateral or bilateral hearing impairment, normal otoscopic examination, previous stapedectomy, and an audiogram demonstrating mixed or profound SNHL according to Sheehy's audiometric description [2]. Thirty-two consecutive patients with FAO (36 ears) that underwent cochlear implantation were identified in the database. Two patients were excluded due to inadequate clinical or audiometric data. The remaining 30 patients (34 years) in the FAO group were compared to an agematched control group of 30 patients (32 ears) of postlingually deafened adults obtained from the same cochlear implant database. This cohort of patients was randomly selected from a patient database that was recorded during a previous analysis from the same department [15]. Patients were implanted for etiologies other than otosclerosis during the same period of time Surgical technique All surgeries were performed under general anesthesia. Facial nerve monitoring was used in all cases. A mastoidectomy with facial recess approach was performed. The internal receiver stimulator was placed in a subperiosteal pocket and the electrode array was introduced atraumatically through a cochleostomy into the scala tympani. If applicable, the ground electrode was placed in a pocket underneath the temporalis muscle. When ossification was encountered, a drill-out was performed until luminal patency was observed. All patients had a full insertion of the electrode array.

3 610 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) When sequential implantation was performed on the contralateral ear, the same technique was employed; however, bipolar cautery was used instead of monopolar cautery. Neural response telemetry was completed in all patients at the conclusion of the operation and a plain skull radiograph was obtained in the immediate recovery period to confirm appropriate positioning of the implant Outcome assessment Several parameters were surveyed in this study. Demographic data, including age at implantation (to the nearest year) and sex, were recorded. Clinical parameters obtained included duration of hearing deprivation and prior history of stapes surgery. Radiographic and surgical findings were recorded with emphasis on the presence of signs of retrofenestral otosclerosis, cochlear ossification, and number of electrodes inserted. Audiologic data were compiled for each patient. The preoperative and postoperative pure tone average in decibels at 0.5, 1, 2, and 4 khz and speech reception thresholds (SRT) in decibels, when available, were recorded for the best-aided conditions. When no response was measured at the maximal limits of the audiometer (anacusis), the threshold was set at 120 decibels. In addition, pre and postoperative open-set words score and sentence measures were obtained in all patients. Results were reported as short-term (ST) when obtained at or less than 12 months after implantation and long-term (LT) when obtained more than 12 months after implantation. The words scores were recorded according to the NU-6 chip (Northwestern University words score chip). All sentence scores were determined by hearing in noise testing. All immediate or long-term surgical complications after implantation were documented Data analysis Data from the chart review were collected and analyzed by a statistician. Individual values were entered into an Excel file (Excel software; Microsoft, Seattle, WA) and imported into a statistical program (R project for statistical computing, version ) for analysis. Data missing in the postoperative audiometric analysis were considered missing completely at random and were computed using the multiple imputation technique. Analysis of normality using the Shapiro-Wilk multivariate normality test revealed that both groups did not have a normal distribution (P b.0001 for both groups). The nonparametric Permutation Hotelling's T 2 test was used for the multivariate comparison of means between both groups. When a difference is found, univariate analysis using the 2-sided Wilcoxon rank sum test was used. Criterion for statistical significance was set at P =.05, 2-tailed. 3. Results Thirty patients with 34 implanted ears were identified in the FAO group. For patients with bilateral implantation, the surgeries occurred independently and each ear was analyzed separately (Tables 1 and 2). Thirty control patients with 32 implanted ears were selected at random from the database for comparison (Tables 1 and 2). The mean age of the FAO group was 72 ± 5 years, compared to 70 ± 4 years in the control group. There was no statistical difference between both groups (P =.63). In the FAO group, 47% were men and 53% were women. In the control group, 50% were men and 50% were women. A previous stapedectomy had been performed on 42.8% of the patients in the FAO group. All of these patients reported dissatisfaction with the results of their stapedectomy and desired cochlear implantation. Two patients (13.3% of the patients who failed stapedectomy) reported full hearing loss following stapedectomy. Patients in the control group underwent cochlear implantation for suspected AIED (n = 13), adult-onset slowly progressive bilateral hearing loss of unknown cause (n = 11), hearing loss Table 1 Demographic, radiographic, and surgical data for the FAO group N Age Sex Previous MRI/CT Cochlear Type Insertion at CI stapedectomy abnormalities ossification/ of CI fibrosis 1 78 F Y N N L/NF F 2 78 F Y N N R/C24 F 3 62 M N N N L/NF F 4 78 M N N N R/HiRes F 5 61 F N N Y L/N5 F 6 63 M Y N Y L/N5 F 7 79 F Y N N L/NF F 8 73 M Y N Y L/C24 F 9 60 F N N N R/NF F 10 a 40 F N N N R/C24 F 11 a 39 F N Y N L/C24 F M Y Y N R/NF F M N N N R/N5 F F N N N R/NF F F Y Y N L/N5 F F N N N R/C24 F F Y N N R/NF F F N N Y R/C24 F M Y Y Y R/NF F 20 a 52 F N N N R/NF F 21 a 52 F N N N L/C24 F F N N Y L/C24 F M N N N L/NF F 24 a 48 M N Y N L/C24 F 25 a 49 M N Y N R/N5 F M N N N R/C24 F M Y N Y R/NF F M N Y Y R/N5 F 29 a 63 F Y Y Y L/N5 F 30 a 62 F Y Y Y R/NF F F N N N R/C24 F F Y N N R/N5 F M Y N N L/HiRes F M Y N N R/N5 F NF, Cochlear Nucleus Freedom device; C24,Cochlear Nucleus 24 contour device; N5, Cochlear Nucleus 5 device; HiRes, Advanced Bionics HiRes device; F, full insertion; L, left; R, right. a Patients who received bilateral implants (sequential).

4 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) Table 2 Demographic, radiographic, and surgical data for the control group N Age Sex Previous MRI/CT Cochlear Type Insertion at CI Stapedectomy abnormalities ossification/ of CI fibrosis 1 75 M N N N R/N5 F 2 64 M N N N L/N5 F 3 68 M N N N R/C24 F 4 70 M N N N L/C24 F 5 73 F N N N R/N5 F 6 a 41 F N N N L/MED F 7 a 47 F N N N R/MED F 8 58 F N N N R/N5 F 9 70 M N N N L/ C24 F F N N Y L/ C24 F F N N N R/ C24 F F N N Y R/ C24 F M N N N L/ C24 F M N N N R/ C24 F F N N N R/ C24 F F N N N R/ C24 F F N N N L/ C24 F M N N N L/NF F M N N N L/C24 F M N N N L/NF F M N N N L/N5 F 22 a 63 M N N N R/N5 F 23 a 62 M N Y N L/NF F F N N N R/NF F M N N N R/N5 F M N N N L/N5 F M N N N L/NF F F N Y N R/N5 F F N N N R/C24 F F N N Y L/C24 F F N N N L/C24 F F N N N L/C24 F NF, Cochlear Nucleus Freedom device; C24,Cochlear Nucleus 24 contour device; N5, Cochlear Nucleus 5 device; MED, MED-EL device; F, full insertion; L, left; R, right. a Patients that received bilateral implants (sequential). ears. Full electrode insertion was achieved in all patients, and no facial nerve stimulation was appreciated at the time of surgery or at follow-up. There were no intraoperative or postoperative complications noted in the control group. Two patients in the FAO group reported postoperative tinnitus and one patient suffered recurrent ear infections following cochlear implantation. Three patients in the FAO group and 8 patients in the control group experienced dizziness preoperatively, but symptoms resolved postoperatively. Six patients in the FAO group who did not experience dizziness preoperatively developed symptoms after cochlear implantation. None of the patients in the control group experienced postoperative dizziness. Intraoperative ossification of the cochlea was found in 29.4% of patients in the FAO group and 9.4% of patients in the control group. An experienced neuroradiologist (DPH), blinded to the hearing outcome observed following implantation, reviewed all images that were accessible through the computerized medical record. Computed tomographic findings were abnormal in 26.4% of patients with FAO as compared to 6.2% in the control group. In the FAO group, one patient's CT revealed narrowing of the anterior basal turn of the cochlea, whereas fenestral and retrofenestral otosclerosis was identified in 2 and 5 patients, respectively (Figs. 1 and 2). In one of these patients, both fenestral and retrofenestral otosclerosis were noted concomitantly. In the control group, 2 patients had findings of fenestral otosclerosis described in their report. These patients were not thought to have clinical otosclerosis based on the selection criteria used in this study. from childhood illness (n = 7), and chronic ear disease with presumed labyrinthitis (n = 1). Patients were implanted with related devices from the Cochlear Corporation company (Cochlear Americas, Ltd, Centennial, CO) except for 2 patients in the FAO group, who received HiRes devices (Advanced Bionics, LLC, Valencia, CA) and 1 patient (2 ears) in the control group who received bilateral sequential implantation using Med-El Combi 40 + device (MED-EL Corp, Dunham, NC). Four patients in the FAO group received bilateral sequential implants. Two additional patients had bilateral implants however both patients had 1 implant removed due to infection (15 years after implantation) in one case and device failure in the other. Since only one side was functional, these 2 patients were included as unilateral implantees. In the control group, 2 patients received bilateral implants. In the FAO group, the left side was implanted in 14 (41%) ears and the right side in 20 (59%) ears. In the control group, the left side was implanted in 17 (53%) ears and the right side in 15 (47%) Fig. 1. An axial view of a HRCT of the temporal bone demonstrating a narrow basal turn on the right (black arrow) and an abnormal round window niche on the left (black arrowhead). Note that a halo sign is evident on the right side (above black arrow).

5 612 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) Of the patients with abnormal CT findings consistent with otosclerosis, 33% had cochlear ossification observed intraoperatively. Conversely, 60% of the patients in the FAO group with cochlear ossification found intraoperatively did not have CT findings consistent with otosclerosis Hearing threshold The mean preoperative SRT was 58 ± 14 db in the FAO group and 68 ± 27 db in the control group (Table 3). The mean postoperative ST SRT was 23 ± 7 and 24 ± 7 db in the FAO and control groups, respectively. The mean postoperative LT SRT was 22 ± 6 db in the FAO group and 22 ± 5 db in the control group. There was no statistically significant difference in the preoperative, postoperative ST, and postoperative LT SRT between both groups (P =.77) Words scores The preoperative words scores were 14.9% ± 16% in the control group and 13.8% ± 15% in the FAO group (Table 3). The ST and LT postoperative words scores in the control group were 65% ± 19% and 74% ± 16%, respectively. In the FAO group, the ST and LT words scores were 70% ± 20% and 68.88% ± 15.05%, respectively. No statistical difference was present between the preoperative, postoperative ST, and postoperative LT words scores in both groups (P =.77) Sentence scores Preoperative sentence scores were 17% ± 22% in the FAO group and 17% ± 20% in the control group (Table 3). The postoperative ST and LT sentence scores in the FAO group were 89% ± 13% and 92% ± 5%, respectively. In the control group, the postoperative ST and LT sentence scores were 85% ± 19% and 91% ± 7%. There was no statistical difference in the preoperative, ST, and LT postoperative sentence scores between both groups (P =.77) Hearing outcome prediction The presence of radiographic abnormalities did not predict ST or LT hearing outcome (P =.47 and 0.68, Table 3 Preoperative and postoperative words and sentences scores of both FAO and control groups Control group Otosclerosis group Preoperative word scores (%) Mean Median 12 8 SD ST postoperative word scores (%) Mean Median SD LT postoperative word scores (%) Mean Median SD Preoperative sentence scores (%) Mean Median 10 7 SD ST postoperative sentence scores (%) Mean Median SD LT postoperative sentence scores (%) Mean Median SD Preoperative speech reception thresholds (db) Mean Median SD ST speech reception thresholds (db) Mean Median SD LT speech reception thresholds (db) Mean Median SD respectively). Intraoperative cochlear ossification was associated with worse ST postoperative SRT (P =.008). However, no association was seen for the ST word and sentence scores (P =.58 and 0.79, respectively) and for the LT hearing outcome (P =.24). 4. Discussion Fig. 2. A coronal reconstruction of a HRCT of the temporal bone, demonstrating a narrowed and partially obliterated round window niche on the right (black arrow) and abnormal bone remodeling of the left round window niche (white arrowhead). Otosclerosis is most commonly associated with conductive hearing loss, but can also be associated with SNHL. Conductive hearing loss is usually due to fixation of the stapes footplate to the oval window of the cochlea or sclerosis of the round window. Both of these mechanisms would increase the impedance to the transmission of sound waves into the inner ear. The pathogenesis of SNHL is controversial, and numerous theories have emerged. One hypothesis postulates that proteolytic enzymes from otospongiotic foci leak into the perilymph. Subsequent diffusion into the endolymph can damage hair cells, leading to SNHL

6 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) [16,17]. Additionally, the pattern of degeneration of sensorineural elements has been reported to be very similar to that found in cases of age-related hearing loss, such as presbycusis[18]. Another factor possibly contributing to SNHL is hyalinization of the spiral ligament. In 1983, Parahy and Linthicum observed that patients with otosclerosis and SNHL had hyalinization of the spiral ligament and atrophy of the striavascularis on histologic analysis [19]. Further studies suggested a correlation between the degree of hyalinization and hearing thresholds [20]. High-resolution CT (HRCT) of the temporal bone has been used to assess the progression and prognosis of otosclerosis[21,22]. High-resolution CT can demonstrate hypodensity in the anterior part of the footplate as well as provide reliable information regarding the extent of disease to the pericochlear endosteum, cochlear endosteum, and the internal auditory canal [23]. Sclerosis of the cochlear endosteum can result in irregularity and narrowing of cochlear turns noted on HRCT [24]. A scan that fails to reveal these findings does not rule out otosclerosis. For example, in our FAO group, 73.6% of patients had no remarkable findings on imaging preoperatively. In addition, 2 patients in the control group had findings typical of otosclerosis on imaging. Of those with radiographic abnormalities, 33% were found intraoperatively to have cochlear ossification or fibrosis. A normal radiographic study does not exclude peri- or endocochlear pathologic changes. In our study, the incidence of cochlear ossification was 29.4% and 60% of patients with intraoperative evidence of cochlear ossification or fibrosis had a normal preoperative HRCT. In patients with FAO undergoing cochlear implantation, a surgeon must be prepared to perform a cochlear drill-out if faced with obliteration of the basal turn of the cochlea. In our series, the presence of preoperative radiographic findings did not appear to predict hearing outcome after implantation. The intraoperative presence of cochlear ossification was associated with worse ST SRT (P =.008). However, no significant difference was seen in the ST word and sentence scores and in the LT SRT, word, and sentence scores. This might be helpful in counseling patients with cochlear ossification that if poor outcome is observed in the initial postoperative period, long-term hearing outcome should improve with adequate rehabilitative therapy. One could argue that T2-weighted magnetic resonance imaging is superior to CT in the evaluation of luminal patency. Fibrous obliteration of the cochlear lumen is not evident on CT. Conversely, grade I ossification (commonly seen in FAO patients) can be missed on magnetic resonance imaging (MRI). With the exception of auditory neuropathy and labyrinthitis secondary to bacterial meningitis, the role of MRI in the preoperative evaluation of cochlear implant candidates remains controversial. Furthermore, many of our patients had been implanted with various types of stainless steel stapes prosthesis and MRI imaging was not obtained due to safety reasons. Therefore, in order to maintain conformity of analysis with our control group, CT scans of the temporal bone were obtained and analyzed. Previous studies have demonstrated that patients with FAO experience similar outcomes after cochlear implantation as compared to patients deafened by other etiologies [10,11,24-26]. Both our study groups were considered comparable, as there was no difference in their demographic characteristics (P =.63) and preoperative hearing (P =.77). Following implantation, we found no significant difference between the 2 groups in the ST and LT postoperative word scores, sentence scores, and SRT. In patients with FAO, it is rational to offer stapes surgery prior to consideration of cochlear implantation. Several authors have demonstrated adequate auditory restoration and rehabilitation with stapes surgery [4,7,22]. In the event that hearing loss continues to progress or the stapedectomy fails to improve hearing, cochlear implantation should be considered for hearing rehabilitation. A previous stapedectomy was performed in 42.8% of our patients, with 2 patients reporting loss of residual hearing following their stapes surgery. All of our patients were adequately rehabilitated with a cochlear implant. In our study, the postoperative outcome measures used have a known ceiling effect [27]. In other words, using more stringent criteria may have uncovered a difference not otherwise noted in our patient population. However, due to the retrospective design of the current study and the more recent introduction of these criteria, obtaining such stringent criteria may prove to be impossible. In addition, the goal of this study was merely to evaluate the superiority of speech perception performance of the group as a whole but rather to analyze whether both the control and study groups were comparable using known outcome criteria. Although the efficacy of cochlear implantation in FAO has been reported, studies have also demonstrated an increased rate of complications in these patients, specifically an increased rate of facial nerve stimulation of up to 78% [10,14,24,28]. This results in poorer hearing outcome due to the use of a limited number of electrode channels, as some electrodes were switched off to reduce facial nerve stimulation. The incidence was elevated when a straight electrode array was used [14]. In contrast, a 0% rate of facial nerve stimulation was reported when contour electrodes were utilized [25,29]. Our study showed similar results with no patients experiencing facial nerve stimulation during postoperative follow up. Conceivably, a modiolar-hugging technique avoids electrical stimulation of the lateral wall and decreases the incidence of facial nerve stimulation. Another known postoperative complication of implantation in patients with otosclerosis is tinnitus [10,28]. In our FAO group, 2 patients reported postoperative tinnitus that eventually subsided while our control group had no reported complications. Further studies are necessary to determine if patients with otosclerosis are at an increased risk of tinnitus after cochlear implantation. To our knowledge, this is the first review describing the incidence of retrofenestral radiographic findings and

7 614 M.T. Semaan et al. / American Journal of Otolaryngology Head and Neck Medicine and Surgery 33 (2012) intracochlear ossification requiring drillout and analyzing their postoperative hearing predictive value in patients with FAO. In addition, this study is among the first to describe the low incidence of postoperative facial nerve stimulation using modiolar-hugging technology. 5. Conclusion Cochlear implantation is a safe and effective option for patients with far advanced otosclerosis in whom stapedectomy and amplification was not beneficial. When ossification is encountered, a drill-out of the basal turn reestablishes luminal patency and allows full electrode insertion. Utilizing a modiolar-hugging technique with a contour electrode during insertion decreases the incidence of postoperative facial nerve stimulation. 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