Anatomic features of the fetal round and oval windows, and their relations with the tympanic nerve.

OBJECTIVE: The study aimed to examine morphometric properties of the round window (RW) and oval window (OW) and to show their relation with the tympanic nerve (the Jacobson's nerve, JN) in human fetuses from the otologic surgeon's perspective. METHODS: Thirty temporal bones of 15 fetal cadavers (8 males, 7 females) aged with 24.40 ± 3.71 weeks were included in the study. The height, width and surface area of the RW and OW and also distance from the JN to the OW and RW were measured. RESULTS: The height, width and surface area of the RW in this work were measured as 1.48 ± 0.25 mm, 1.57 ± 0.37 mm, and 2.05 ± 0.69 mm2, respectively. The RW was detected as round-shaped (8 cases, 26.7%), oval-shaped (15 cases, 50%), and dome-shaped (7 cases, 23.3%). The height, width and surface area of the OW were measured as 1.42 ± 0.26 mm, 2.90 ± 0.44 mm, and 3.63 ± 0.74 mm2, respectively. The OW was observed as oval-shaped (15 cases, 50%), kidney-shaped (10 cases, 33.3%), D-shaped (4 cases, 13.3%), and trapezoid-shaped (1 case, 3.3%). The JN was found 1.21 ± 0.60 and 1.18 ± 0.54 mm away from the RW and OW, respectively. CONCLUSION: This study containing morphological data about the shapes, diameters and area of the RW and OW may be useful to predict surgical difficulty, and to select implants of suitable size preoperatively for the windows. Knowing the relationship between the JN and the windows can be helpful to avoid iatrogenic injuries of the nerve.

CT and MRI for the diagnosis of perilymphatic fistula: a study of 17 surgically confirmed patients.

BACKGROUND: We evaluated the usefulness of CT and MRI for the diagnosis of perilymphatic fistula (PLF) of the round (RW) and/or oval (OW) windows, with surgery as gold standard. METHODS: We retrospectively enrolled 17 patients who presented a surgically confirmed PLF of the round (RW) or oval (OW) windows. All patients were imaged by CT + MRI (T2W SSFP without contrast) prior to surgery (= gold standard). Two radiologists, analyzed the RW and OW on the side of the clinical symptoms and sensitivity (Se) + Specificity (Sp) were calculated. RESULTS: Round window fistula was the most frequent (71%). The best sign of PLF on imaging was a fluid filling of the window niches, which had good Se (83-100% for RW, 66-83% for OW) and Sp (60% for RW, 91-100% for OW). Disorientation of the footplate and pneumolabyrinth were also only observed in 50% of OW PLF. CONCLUSION: The combination of CT and MRI is a reliable tool for a fast and accurate diagnosis of round and oval window perilymphatic fistula, with good sensitivity (> 80%). The most common sign of PLF on imaging is the presence of a fluid-filling in the RW (especially if > 2/3 of the RW niche) or in the OW niches on both CT and MRI. A disorientation of the footplate or the presence of a pneumolabyrinth are clearly in favor of an oval window perilymphatic fistula.

Anatomic variability of oval window as pertaining to stapes surgery.

PURPOSE: The purpose of the study is to study the details of dimensions and shape of oval window in different age groups, sides and genders and their clinical implications. The oval window is a key structure while performing surgeries in relation to stapes. An intricate knowledge of the shape and size of the oval window is important for the reconstruction and fitting of cartilage compatible with the native shape of the oval window. METHODS: Sixty normal wet cadaveric temporal bones of known age; gender and side were micro-dissected. The maximum height and width of the oval window was measured. The shape of the oval window was noticed. RESULTS: The mean value for maximum height and width of the oval window was 1.31 ± 0.28 mm and 2.67 ± 0.42 mm, respectively. The height and width of the oval window ranged between 1 mm and 1.5 mm and 2 mm and 3 mm in majority of the cases. he oval window was found to be oval shaped in 53.3% cases, other shapes such as kidney, D shape, rectangular or trapezoidal were also observed. CONCLUSIONS: The refined morphometric information of the oval window will help in preoperative assessment and surgical planning of various oval window-related surgical procedures. The knowledge may also help in designing and selecting proper cartilage shoe for the best outcome. Narrow oval window may cause procedural complications and surgeon discomfort in various stapes surgeries.

The role of endoscopic stapes surgery in difficult oval window niche anatomy.

PURPOSE: The surgical treatment of otosclerosis can be challenging in case of anatomical abnormalities or variations of the oval window niche (OWN) area, as in very narrow OWN or in an overhanging facial nerve. The aim of the present study was to explore the role of endoscopic stapes surgery in cases with difficult OWN anatomy. METHODS: Patients undergoing endoscopic stapes surgery from 2008 to 2017, which fulfilled the CT scan criteria for a "difficult" anatomical condition, according to the measurements and cut-off values defined in the literature, were retrospectively selected. The intraoperative endoscopic view of the anatomical details and surgical difficulties were analysed through the review of the operative videos. Finally, a statistical analysis of the relationship between endoscopic visualization of anatomical details and radiological measurements was carried out. RESULTS: Eighteen out of 205 patients (8.7%) were included in the study. The 94.4% of patients obtained an optimal endoscopic exposure and visualization of all the anatomical details considered in the study, during each step of stapes surgery. The OWN measurements (width, depth and facial-promontory angle) did not affect significantly the endoscopic surgical exposure of the footplate or any of the other anatomical details. CONCLUSIONS: The anatomic features of the oval window area which reduce the visualization in microscopic surgery, did not affect the surgical exposure in endoscopic stapes surgery. Patients having a difficult anatomy of the OWN can be treated safely with the endoscopic approach. In the case of a predicted "difficult anatomy", the endoscopic approach can be considered a viable option.

Gusher in stapes surgery: a systematic review.

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. CONCLUSION: 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.

The Impact of a Cochlear Implant Electrode Array on the Middle Ear Transfer Function.

OBJECTIVES: As a treatment for partial deafness with residual hearing in the lower frequency range, the combined acoustic and electric stimulation of the cochlea has become widespread. Acoustic stimulation is provided by a hearing aid's airborne sound and the electric stimulation by a cochlear implant electrode array, which may be inserted through the round window or a cochleostomy. To take advantage of that concept, it is essential to preserve residual hearing after surgery. Therefore, the intracochlear electrode array should not compromise the middle ear vibration transmission. This study investigates the influence of different electrode types and insertion paths on the middle ear transfer function and the inner ear fluid dynamics. DESIGN: Sound-induced oval and round window net volume velocities were calculated from vibration measurements with laser vibrometers on six nonfixated human temporal bones. After baseline measurements in the "natural" condition, a cochleostomy was drilled and closed with connective tissue. Then, four different electrode arrays were inserted through the cochleostomy. Afterwards, they were inserted through the round window while the cochleostomy was patched again with connective tissue. RESULTS: After having drilled a cochleostomy and electrode insertion, no systematic trends in the changes of oval and round window volume velocities were observed. Nearly all changes of middle ear transfer functions, as well as oval and round window volume velocity ratios, were statistically insignificant. CONCLUSIONS: Intracochlear electrode arrays do not significantly increase cochlear input impedance immediately after insertion. Any changes that may occur seem to be independent of electrode array type and insertion path.

Case 226: Oval Window Atresia.

A 6-year-old girl presented with bilateral hearing loss. Her otologic, birth, and family histories were limited, given that she was adopted, but her parents reported that she had had difficulty hearing and speaking ever since they adopted her at 2 years of age. Her parents denied a history of acute otitis media, otorrhea, otalgia, vertigo, autophony, or tinnitus since her adoption. At 2.5 years of age, a diagnosis of hearing loss was made, and she was given hearing aids. Her parents believed that she had been doing well with both receptive and expressive language since she had received the hearing aids. At examination, she had small bilateral preauricular skin tags and normal pinna. Her external auditory canals were of a normal caliber bilaterally, with no otorrhea or lesions. The tympanic membranes were translucent and mobile at pneumatic otoscopy. There was no evidence of a middle ear lesion, nor was there a Schwartz sign. She had no nystagmus or vertigo at pneumatic otoscopy. Audiometry was performed and revealed moderate to severe conductive hearing loss bilaterally, with a mixed component present at 2000 KHz. She had normal bilateral middle ear pressure at tympanometry. Thin-section computed tomography (CT) of the temporal bone was performed.

Congenital malformation of the oval window: experience of radiologic diagnosis and surgical technique.

The aim of this study was to introduce and discuss the method of preoperative radiological diagnosis to the congenital absence or atresia of the oval window (OW), and the method of surgical treatment. From July 2010 through August 2014, patients with normal external canal and tympanic membrane but conductive hearing loss underwent high resolution CT scan (HRCT). The multi-planar reformation (MPR), a post-processing protocol, was used. The patients with diagnosis of OW atresia and malformed stapes preoperatively underwent surgical treatment. The vestibular drilled-out and promontory drilled-out technique was used to reconstruct the ossicular chain. In the preoperative radiological diagnosis, six patients (ears) were noted to have congenital absence or atresia of the oval window with malformed facial nerve (class 4) and two patients (ears) were found to have footplate fixation (class 2). In the surgical treatment of eight ears, the malformed structure was identified and the ossicular chain reconstruction was made in six ears. The coronal HRCT CT imaging and the MPR post-processing technique can provide us practical and definite information for surgical treatment, especially in the discrimination of OW atresia and the fixed stapedal footplate. The promontory drill-out technique, fenestration in the bottom of the basal turn, provides us a new method in the hearing reconstruction when the area of OW was fully covered by malformed facial nerve. This technique was first reported in the literature.

Round and oval window reinforcement for the treatment of hyperacusis.

PURPOSE: To present the outcomes of two patients (three ears) with hyperacusis treated with round and oval window reinforcement. MATERIALS AND METHODS: Transcanal placement of temporalis fascia on the round window membrane and stapes footplate was performed. Loudness discomfort level testing was performed. Results of pre and post-operative hyperacusis questionnaires and audiometric testing were reviewed. RESULTS: Two patients (three ears) underwent surgery. Results from the hyperacusis questionnaire improved by 21 and 13 points, respectively. Except for a mild loss in the high frequencies, no change in hearing was noted post-operatively. Both patients reported no negative effects from surgery, marked improvement in ability to tolerate noise, and would recommend the procedure to others. There were no complications. CONCLUSIONS: Round and oval window reinforcement is a minimally invasive option for treating hyperacusis when usual medical therapies fail. Further studies are needed to evaluate the effectiveness of the procedure in reducing noise intolerance.

Perilymphatic fistulas: can we predict the diagnosis?

This study intends to identify factors that could better predict the diagnosis of perilymphatic fistula (PLF) since exploration surgery is currently the only confirmatory method. This retrospective chart review in a tertiary care center is based on all 71 available patient files operated for a suspicion of PLF between 1983 and 2012. History of predisposing factors, clinical findings and investigations were documented pre- and postoperatively. Patients were divided according to intraoperative findings into two groups: group I (fistula negative) and group II (fistula positive). In addition, group II was divided into two subgroups: patients with or without a history of stapedectomy. Both groups were demographically similar. With the exception of history of previous partial stapedectomy (p = 0.04), no statistical difference could be identified in predisposing factors and in clinical findings between the two groups. The evolution of symptoms showed an overall improvement of vestibular symptoms (91 %) and cochlear symptoms (53 %) postoperatively. Audiograms showed a significant improvement postoperatively in the pure tone audiometry and bone conduction threshold of group II while the air-bone gap and speech discrimination score did not improve. Group I did not show any significant improvement postoperatively in any audiogram parameter. This study failed to identify factors that could better predict the diagnosis of PLF. However, it shows that middle ear exploration with oval and round window obliteration is effective in PLF especially to decrease vestibular symptoms even when fistula is unidentified intraoperatively.

Enhanced oval window and blocked round window passages for middle-inner ear transportation of gadolinium in guinea pigs with a perforated round window membrane.

To elucidate the communication between the middle and inner ear, and the fluid dynamics of the inner ear with the perilymphatic fistula (PLF) of the round window membrane (RWM). The PLF of the RWM was created in nine guinea pigs. Gadolinium diethylenetriamine pentaacetic acid bismethylamide (Gd-DTPA-BMA) was delivered into the middle ear and followed in the inner ear using a 4.7 Tesla MRI. Pressure was delivered to the external ear canal of PLF ear in an attempt to enhance the inner ear uptake of Gd-DTPA-BMA. The immediate loading of Gd-DTPA-BMA in the scala tympani of the basal turn was ablated by the outflow of perilymph through the leaking RWM while the oval window passage for Gd-DTPA-BMA was enhanced. There was more Gd-DTPA-BMA distribution in the scala tympani than in the scala vestibuli in the second turn of the PLF cochlea (within 20 min). Signal in the vestibulum and scala vestibuli of the basal turn and rest part of PLF cochlea was greater than that of the control cochlea with intact RWM within 30 min. Pressure applied to the external ear canal tended to enhance the loading of Gd-DTPA-BMA in the perilymphatic scalae of the PLF cochlea. The enhanced oval window passage of Gd-DTPA-BMA was proven by the distorted distribution in the inner ear with PLF. The radial communication of cochlear perilymph was supported by the Gd-DTPA-BMA gradient among the perilymphatic scalae. Applying positive pressure to the external ear canal caused backflow of perilymph into the cochlea which has a potential of transmitting microbes from the middle ear into the inner ear.

Oval Window Temperature Changes in an Endoscopic Stapedectomy.

OBJECTIVES: Following the initial use of endoscopes in otology, the pros and cons of these instruments have been questioned increasingly. These instruments cause an increase in temperature that needs to be investigated. In this study, the authors aimed to investigate the temperature increase caused by endoscopes and light sources in the perilymph by performing a stapedotomy in an animal model under anesthesia. STUDY DESIGN: The study was performed in a guinea pig model. METHODS: In the animal model, a simulated otologic stapes surgery was performed at room temperature. The body temperatures of the guinea pigs were monitored; the temperature increase caused by the 0-degree rigid endoscopes with diameters of 3 and 4  mm as well as the light sources, including halogen, light-emitting diode (LED), and xenon lamps, were monitored following the stapedotomy to measure and document the continuous temperature increase in the perilymph using sensors placed at the oval window. RESULTS: Rigid endoscopes cause a temperature increase in the tympanum regardless of their diameter when used with xenon and halogen light sources. The LED light caused a relatively small temperature increase. CONCLUSIONS: The endoscopic instruments used in the stapes operation caused a temperature increase in the oval window. The authors concluded that this heat could easily be transmitted to the cochlea by the perilymph, which has obstructed contact with the outer environment following stapedomy, resulting in neurosensorial damage.

Anatomic Landmarks for Localization of the Vertical Segment of Facial Nerve on Multislice CT Multiplanar Reconstruction Images.

The facial nerve decompression via mastoid is simple and easy to expose the vertical segment of the facial nerve (VFN). The objective of this study was to find out the relationship between the VFN and anatomic landmarks to facilitate prediction of the location of VFN during facial nerve decompression. The different landmarks were cochlear window (CW), oval window (OW), promontorium tympani (PT), and mastoid antrum (MA). Parameters of 140 patients (280 observations) with healthy middle ears were measured on high-resolution spiral multislice computed tomographic multiplanar reconstruction (MPR) images that were parallel to the base plane. The data gained were analyzed by statistical method and were also analyzed with respect to side and gender. The angle between orientation of VFN to the CW of the longest distance and horizontal axis was larger on the left side than that of the right (P < 0.05). Our results may provide more detailed information to predict the location of VFN during facial nerve decompression through mastoid.

[About the surgical treatment of vestibular window abnormalities].

The objective of the present study was to develop the sparing strategy for the surgical treatment of the patients presenting with malformations of the vestibular window based on the results of the evaluation of synopty of the main clues of the internal and middle ears. This article contains the analysis of the effectiveness of the surgical treatment of 17 patients with malformations of the vestibular window operated during the period from 2008 till 2013. It presents the results of investigations into the topographic relationships between the principal structures of the internal and middle ears of special importance for the assessment of the possibilities for the surgical treatment of such patients with the minimal risk of development of postoperative complications. The results of the surgical treatment were estimated based on the data of audiological studies in the early and late postoperative periods. It was shown that none of the patients developed the symptoms of dizziness, unstable gait or sensorineural impairment of hearing in the immediate postoperative period. Good functional results in the form of the reduced bone-air interval (15.2±1.0 dB) were achieved in 13 patients. Nevertheless, the considerable decrease in the sound conductivity in the late postoperative period observed in 64.7% of the patients related to the closure of the vestibular fistula dictates the necessity of developing the novel techniques for its prevention.

Abnormal CT findings are risk factors for otitis media-related sensorineural hearing loss.

OBJECTIVES: Inflammatory processes in chronic otitis media (COM) can damage the inner ear, resulting in sensorineural hearing loss (SNHL). The purpose of this study was to evaluate risk factors by computed tomography (CT) findings for COM-related SNHL in patients with unilateral COM. DESIGN: Records from January 2009 to December 2012 of 231 patients with unilateral COM and a normal contralateral ear were retrospectively evaluated. Bone conduction thresholds were calculated for each ear at 0.5, 1, 2, and 4 kHz. If bone conduction threshold averaged across the four frequencies of the COM ear was 10 dB greater than that of the contralateral ear, the patient was classified under the SNHL group. Temporal bone CT images were analyzed to determine the presence or absence of soft tissue density in the attic (medial/lateral), antrum, oval window niche, and round window niche. Multivariate analysis of sex, age (< 50 years; ≥ 50 years), disease duration (< 20 years; ≥ 20 years), perforation size (small; moderate; large) and the presence of soft tissue density in the attic (medial/lateral), antrum, oval window niche, and round window niche was used to determine the factors related to COM-related SNHL. RESULTS: Of the 231 patients, there were 51 patients (22.1%) in the SNHL group and 180 patients (77.9%) in the non-SNHL group. Multivariate analyses showed that the presence of soft tissue density in the antrum (odds ratio [OR] 3.80; 95% confidence interval [CI] 1.74-8.32; p = 0.001), age ≥ 50 years and more (OR 3.32; 95% CI 1.62-6.81; p = 0.001), disease duration ≥ 20 years (OR 2.80; 95% CI 1.31-6.02; p = 0.008), and the presence of soft tissue density in the round window niche (OR 2.42; 95% CI 1.12-5.21; p = 0.024) were independently related to COM-related SNHL. CONCLUSIONS: COM-related SNHL was present in 22% of ears with COM. The presence of soft tissue density in the antrum, determined from temporal bone CT, increased the OR of SNHL to 3.8. Age, disease duration, and the presence of soft tissue density in the round window niche were also independent predicting factors for SNHL. These findings may be used as informative references for patients with COM.

The windows of the inner ear.

The oval and round windows of the inner ear are important structures for the transmission of sound and may be affected by a variety of disease entities. The anatomy of this small area is one that often causes the radiology trainee some difficulty, but there are certain disease states that can be easily diagnosed when knowing where and how to look. As this area is very important to the otologist in a variety of preoperative settings, accurate assessment of the windows and recognition of important and potentially complex intra-operative anomalies, will greatly aid our surgical colleagues.

Vibrant SoundBridge application to middle ear windows versus conventional hearing aids: a comparative study based on international outcome inventory for hearing aids.

In this study, we aimed to compare the outcomes of satisfaction of the patients who used hearing aids preceding the vibrant sound bridge (VSB) application on middle ear windows (14 oval window and 5 round window). Nineteen adult patients with conductive or mixed hearing loss were included in the study. All patients used behind the ear hearing aids on the site which was selected for VSB application. The patients used hearing aids for at least 3 months before the VSB operation. The floating mass transducer (FMT) was placed on one of the middle ear windows (oval or round) in VSB operation. The patients were evaluated with International Outcome Inventory for Hearing Aids (IOI-HA) preoperatively after at least 3 months trial of conventional hearing aid and postoperatively after 3 months use of VSB. No perioperative problem was encountered. The total score of IOI-HA was significantly higher with VSB compared with conventional hearing aids (p < 0.05). No statistically significant difference was found between the daily use, residual activity limitations, satisfaction, impact on others, quality of life between middle ear implant and hearing aid (p > 0.05). The IOI-HA scores were significantly higher with the middle ear implant than the conventional hearing aid regarding benefit and residual participation restrictions (p < 0.05). Although the scores for quality of life assessment was similar between VSB and hearing aid use, there was a superiority of VSB in terms of benefit and residual participation restrictions as well as overall IOI-HA scores as the FMT was placed on one of the middle ear windows.

Oval and round window vibroplasty: a comparison of hearing results, risks and failures.

The aim of this study was to compare oval and round window vibroplasty. Eighteen (18) patients implanted with Vibrant Soundbridge (VSB) were enrolled. Two groups were formed depending on FMT placement: on round window in ten cases (RW group) and on oval window in eight (OW group). Pre and postoperative audiological tests were performed both under headphones and free-field settings, VSB on and off. One (1) RW patient experienced sudden hearing loss at the operated side after 4 months from surgery and was excluded from the analysis. Both groups showed good hearing results. Significant differences were measured at free-field pure-tone test with VSB on at 0.5 kHz (RW better than OW, p = 0.026) and 4 kHz (OW better than RW, p = 0.043). Both techniques share similar good results and are considered safe. However, we had one failure with deep and sudden hearing threshold worsening after some months of good results. From a surgical point of view OW vibroplasty is easier and safer to perform, when the stapes suprastructure is absent, as it does not require any drilling and should be preferred in such cases. More reports are needed to explain if RW vibroplasty is risky in a mid to long term.

The location of the mastoid portion of the facial nerve in patients with congenital aural atresia.

In order to investigate the location of the mastoid portion of the facial nerve in patients with congenital aural atresia and to assess its effect on the round window middle ear implant (MEI) transducer implantation approach, 70 patients with unilateral congenital aural atresia were examined by computer tomography (CT). The patients were divided into two groups based on their ages: 44 patients in Group A (2-12 years) and 26 patients in Group B (13-29 years). CT scans were reviewed for each patient. Based on the CT findings, the mastoid portion of the facial nerve's spatial configuration with respect to the oval and round windows was qualitatively recorded. Additionally, the exact location of the facial nerve was measured quantitatively. The results suggested that of the 70 deformed ears, 57 had facial nerves located at the round window, six at the oval window, and seven at the normal site. Of the 70 normal opposite ears, 63 had facial nerves located at the normal site, and the other seven had facial nerves located at the round window. Based on the quantitative measurements, the mastoid portion of the facial nerve was more anteriorly positioned in the deformed ears: 3.44-6.09 mm more anteriorly located in Group A and 4.35-7.41 mm more anteriorly located in Group B. In conclusion, in patients with congenital aural atresia, the dislocation of the facial nerve could have significant effects on the surgical approach to round window MEI transducer implantation.

Cochlear implantation in children with "CHARGE syndrome": surgical options and outcomes.

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with "CHARGE syndrome" submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy-posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid's audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.