Early fitting in cochlear implant surgery: preliminary results.

PURPOSE: Cochlear implants are usually activated 3-5 weeks after surgery; to date, no universal protocol exists regarding switch on and fitting of these devices. The aim of the study was to assess safety and functional results of activation and fitting of cochlear implant within 24 h following surgery. METHODS: In this retrospective case-control study, 15 adult patients who underwent cochlear implant surgery, for a total of 20 cochlear implant procedures were analyzed. In particular, clinical safety and feasibility were investigated by examinating patients at activation and at each follow-up. Values of electrodes' impedance and most comfortable loudness (MCL) were analyzed from the time of surgery to 12 months after activation. Free-field pure tone average (PTA) was also recorded. RESULTS: No major or minor complications were reported and all patients could perform the early fitting. Activation modality influenced impedance values only in the short term but the differences were not statistically significant (p > 0.05). Mean MCL values in the early fitting group were lower than MCL of the late fitting in all follow-up sessions, and the difference was statistically significant (p < 0.05). The mean PTA was lower in the early fitting group but the difference was not statistically significant (p < 0.05). CONCLUSIONS: Early fitting of cochlear implants is safe, allows for an early rehabilitation and can have possible beneficial effects on stimulation levels and dynamic range.

Noonan syndrome: cochlear implantation in the setting of cochlear nerve deficiency.

BACKGROUND AND AIM: Noonan syndrome (NS) is a congenital disorder characterized by a wide heterogeneity in clinical and genetic features. Hearing loss can frequently occur in NS, although not always mentioned in its diagnostic criteria. We are reporting on a child with an established NS who underwent bilateral cochlear implantation (CI) in the setting of cochlear nerve deficiency. CASE PRESENTATION: We present the case of a child-girl affected by NS. Newborn hearing screening and audiological evaluations reveled an asymmetric sensorineural hearing loss (SNHL), profound at left ear and severe at right ear. Hearing aids were fitted at the age of six months. Brain magnetic resonance imaging showed hypoplastic cochlear nerves. Due to progressive worsening of the hearing thresholds and inappropriate speech development, at the age of 2 years she underwent a left-sided cochlear implantation. Four years later, right ear was also implanted. Six years after the first surgery, a partial extrusion of the electrode array was noticed. Explantation and reimplantation of a new device was performed, adopting a subtotal petrosectomy approach. The patient reached a score of 95% in open-set speech perception tests. CONCLUSIONS: Hearing loss is a frequent finding in patients with NS; however, its nature and severity are very heterogenous. In consideration of the possible progression of SNHL, audiological follow-up in NS patients must be carefully and periodically performed so as to early detect worsening of hearing threshold. If indicated, cochlear implantation should be considered, taking account of audiological and systemic features of this syndrome.

Study on transfer learning capabilities for pneumonia classification in chest-x-rays images.

BACKGROUND: over the last year, the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and its variants have highlighted the importance of screening tools with high diagnostic accuracy for new illnesses such as COVID-19. In that regard, deep learning approaches have proven as effective solutions for pneumonia classification, especially when considering chest-x-rays images. However, this lung infection can also be caused by other viral, bacterial or fungi pathogens. Consequently, efforts are being poured toward distinguishing the infection source to help clinicians to diagnose the correct disease origin. Following this tendency, this study further explores the effectiveness of established neural network architectures on the pneumonia classification task through the transfer learning paradigm. METHODOLOGY: to present a comprehensive comparison, 12 well-known ImageNet pre-trained models were fine-tuned and used to discriminate among chest-x-rays of healthy people, and those showing pneumonia symptoms derived from either a viral (i.e., generic or SARS-CoV-2) or bacterial source. Furthermore, since a common public collection distinguishing between such categories is currently not available, two distinct datasets of chest-x-rays images, describing the aforementioned sources, were combined and employed to evaluate the various architectures. RESULTS: the experiments were performed using a total of 6330 images split between train, validation, and test sets. For all models, standard classification metrics were computed (e.g., precision, f1-score), and most architectures obtained significant performances, reaching, among the others, up to 84.46% average f1-score when discriminating the four identified classes. Moreover, execution times, areas under the receiver operating characteristic (AUROC), confusion matrices, activation maps computed via the Grad-CAM algorithm, and additional experiments to assess the robustness of each model using only 50%, 20%, and 10% of the training set were also reported to present an informed discussion on the networks classifications. CONCLUSION: this paper examines the effectiveness of well-known architectures on a joint collection of chest-x-rays presenting pneumonia cases derived from either viral or bacterial sources, with particular attention to SARS-CoV-2 contagions for viral pathogens; demonstrating that existing architectures can effectively diagnose pneumonia sources and suggesting that the transfer learning paradigm could be a crucial asset in diagnosing future unknown illnesses.

Subtotal petrosectomy and cochlear implantation.

OBJECTIVE: The objective of this study is to analyse surgical outcomes in a series of patients who underwent subtotal petrosectomy in combination with cochlear implantation. METHODS: Retrospective chart review. Thirty patients (32 ears) underwent subtotal petrosectomy and cochlear implantation in one stage. Indications for subtotal petrosectomy included the following: cholesteatoma, chronic otitis media, previous canal wall-down, osteoradionecrosis, revision surgery for clinical reasons, inner ear malformations, middle ear anatomical variations and severe cochlear ossification. RESULTS: Follow-up ranged from 2 to 54 months. Only 2 complications related to the subtotal petrosectomy (1 subcutaneous abdominal haematoma and 1 subcutaneous abdominal seroma) occurred in this series. Complete electrode insertion was achieved in all but 4 cases. CONCLUSIONS: Subtotal petrosectomy is a safe procedure and can offer technical advantages in some cases of complex cochlear implantation.

Real-time telemetry monitoring of oxygen in the central complex of freely-walking Gromphadorhina portentosa.

A new telemetric system for the electrochemical monitoring of dissolved oxygen is showed. The device, connected with two amperometric sensors, has been successfully applied to the wireless detection of the extracellular oxygen in the central complex of freely-walking Gromphadorhina portentosa. The unit was composed of a potentiostat, a two-channel sensor conditioning circuit, a microprocessor module, and a wireless serial transceiver. The amperometric signals were digitalized and sent to a notebook using a 2.4 GHz transceiver while a serial-to-USB converter was connected to a second transceiver for completing the communication bridge. The software, running on the laptop, allowed to save and graph the oxygen signals. The electronics showed excellent stability and the acquired data was linear in a range comprised between 0 and -165 nA, covering the entire range of oxygen concentrations. A series of experiments were performed to explore the dynamics of dissolved oxygen by exposing the animals to different gases (nitrogen, oxygen and carbon dioxide), to low temperature and anesthetic agents (chloroform and triethylamine). The resulting data are in agreement with previous O2 changes recorded in the brain of awake rats and mice. The proposed system, based on simple and inexpensive components, can constitute a new experimental model for the exploration of central complex neurochemistry and it can also work with oxidizing sensors and amperometric biosensors.

The Presence of Polysaccharides, Glycerol, and Polyethyleneimine in Hydrogel Enhances the Performance of the Glucose Biosensor.

The use of amperometric biosensors has attracted particular attention in recent years, both from researchers and from companies, as they have proven to be low-cost, reliable, and very sensitive devices, with a wide range of uses in different matrices. The continuous development of amperometric biosensors, since their use involves an enzyme, is specifically aimed at keeping and increasing the catalytic properties of the loaded protein, so as to be able to use the same device over time. The present study aimed to investigate the impact of glycerol and polysaccharides, in the presence of polycationic substances to constitute a hydrogel, in enhancing the enzymatic and analytic performance of a glucose biosensor. Initially, it was possible to verify how the deposition of the starch-based hydrogel, in addition to allowing the electropolymerization of the poly(p-phenylenediamine) polymer and the maintenance of its ability to shield the ascorbic acid, did not substantially limit the permeability towards hydrogen peroxide. Moreover, different biosensor designs, loading a mixture containing all the components (alone or in combination) and the enzyme, were tested in order to evaluate the changes of the apparent enzyme kinetic parameters, such as VMAX and KM, and analytical response in terms of Linear Region Slope, highlighting how the presence of all components (starch, glycerol, and polyethyleneimine) were able to substantially enhance the performance of the biosensors. The surface analysis of the biosensors was performed by scanning electron microscope (SEM). More, it was shown that the same performances were kept unchanged for seven days, proving the suitability of this biosensor design for short- and mid-term use.

Cochlear implantation after solid organ transplantation: long term results and review of the literature.

OBJECTIVE: To analyze rate and type of complications in cochlear implant (CI) recipients receiving immunosuppressive therapy following solid organ transplant (SOT). STUDY DESIGN: Retrospective case series. English language literature review. SETTING: Tertiary referral center. INTERVENTION: Cochlear implantation surgery following solid organ transplantation (SOT) and immunosuppressive therapy. METHODS: Data of patients who received CI after SOT and with at least one year of follow up were reviewed. Main outcome measures were the rate and type of complications, classified as major (requiring a second surgical procedure) and minor (requiring medical therapy). A search was performed in PubMed database on January 2019 using the keywords: organ transplant; cochlear implant, complications, deafness, solid organ transplant, immunosuppressive therapy. Only studies reporting on patients who have been implanted after the transplant procedure and with a follow up period of at least 1 year were considered. Final analysis was performed on pooled data. RESULTS: Four patients received CI surgery following SOT. Age at treatment ranged from 40 to 47 years (mean 44.25 years). Follow-up after implantation averaged 5.25 years (range 1-10 years), without complications. Review of the available literature on the subject yielded seven papers; a total of 26 procedures in 22 patients satisfied inclusion criteria. Pooled data from the present series and from the literature were analyzed; the global rate of complications was 16.6%, with 10% major (3 of 30 procedures) and 6.6% minor (2 of 30 procedures). The three reported cases of major complications appear unrelated to SOT. Major complications were found in one case over 16 procedures in pediatric patients (6.2%), while in adults the percentage raised to 14.3% (2/14 procedures). CONCLUSIONS: Cochlear implantation is a safe and effective intervention, even during immunosuppressive therapy after organ transplantation.

Low-Temperature Storage Improves the Over-Time Stability of Implantable Glucose and Lactate Biosensors.

Molecular biomarkers are very important in biology, biotechnology and even in medicine, but it is quite hard to convert biology-related signals into measurable data. For this purpose, amperometric biosensors have proven to be particularly suitable because of their specificity and sensitivity. The operation and shelf stability of the biosensor are quite important features, and storage procedures therefore play an important role in preserving the performance of the biosensors. In the present study two different designs for both glucose and lactate biosensor, differing only in regards to the containment net, represented by polyurethane or glutharaldehyde, were studied under different storage conditions (+4, -20 and -80 °C) and monitored over a period of 120 days, in order to evaluate the variations of kinetic parameters, as VMAX and KM, and LRS as the analytical parameter. Surprisingly, the storage at -80 °C yielded the best results because of an unexpected and, most of all, long-lasting increase of VMAX and LRS, denoting an interesting improvement in enzyme performances and stability over time. The present study aimed to also evaluate the impact of a short-period storage in dry ice on biosensor performances, in order to simulate a hypothetical preparation-conservation-shipment condition.

Audiologic Manifestations of Marshall Syndrome.

OBJECTIVE: Marshall syndrome is a genetic disorder caused by mutations in the COL11A1 gene. This syndrome is characterized by skeletal, ophthalmologic, craniofacial, and auditory abnormalities. Hearing loss is among the main manifestations reported in this disorder being observed in approximately 80% of affected individuals.The present study aims to describe the audiologic characteristics of three members of a family with Marshall syndrome and also serves as a review of the literature. STUDY DESIGN: Family study. SETTING: Tertiary care otology and skull base center. PATIENTS: We report the audiologic findings in a family with Marshall syndrome consisting of a mother and her son and daughter. INTERVENTION(S): The audiologic evaluation included tympanometry, acoustic reflexes testing, auditory brainstem response, transient otoacoustic emissions, pure-tone audiometry, speech audiometry in quiet, and conditioned play audiometry. These methods were applied according to the age of the patients. In addition, we provide a review of the English-language literature in an attempt to clarify the auditory phenotype of this syndrome. RESULTS: All 3 affected individuals had heterozygous c.3816+1G>A mutation in the splicing donor site of intron 50 of the COL11A1 gene. All three patients in our study had bilateral sensorineural hearing loss. Hearing impairment ranged from mild to moderate in the daughter, moderate in the son, and from mild to moderate in their mother. CONCLUSION: The majority of individuals with Marshall syndrome present early-onset bilateral sensorineural hearing loss. Hearing impairment is usually detected in early childhood, progresses gradually, and becomes stable in late adulthood, with a severity ranging from mild to severe.

Prevention and management of vascular complications in middle ear and cochlear implant surgery.

The objective of this study is to illustrate prevention strategies and management of vascular complications from the jugular bulb (JB) and internal carotid artery (ICA) during middle ear surgery or cochlear implantation. The study design is retrospective case series. The setting is tertiary referral university hospital. Patients were included if presented pre- or intraoperative evidence of high-risk anatomical anomalies of ICA or JB during middle ear or cochlear implant surgery, intraoperative vascular injury, or revision surgery after the previous iatrogenic vascular lesions. The main outcome measures are surgical outcomes and complications rate. Ten subjects were identified: three underwent cochlear implant surgery and seven underwent middle ear surgery. Among the cochlear implant patients, two presented with anomalies of the JB impeding access to the cochlear lumen and one underwent revision surgery for incorrect positioning of the array in the carotid canal. Subtotal petrosectomy was performed in all cases. Anomalies of the JB were preoperatively identified in two patients with attic and external auditory canal cholesteatoma, respectively. In a patient, a high and dehiscent JB was found during myringoplasty, while another underwent revision surgery after iatrogenic injury of the JB. A dehiscent ICA complicated middle ear effusion in one case, while in another case, a carotid aneurysm determined a cholesterol granuloma. Rupture of a pseudoaneurysm of the ICA occurred in a child during second-stage surgery and required permanent balloon occlusion without neurological complications. Knowledge of normal anatomy and its variants and preoperative imaging are the basis for prevention of vascular complications during middle ear or cochlear implant surgery.

Clinical findings and surgical results of middle ear cholesteatoma behind an intact tympanic membrane in adults.

BACKGROUND AND AIM: Cholesteatoma usually arises in the middle ear by the formation of a retraction pocket or a tympanic membrane perforation. In some cases, cholesteatoma presents behind an intact tympanic membrane (ITMC) and the underlying mechanism of its development is controversial. The aim of this study was to describe clinical features, pathogenesis and surgical results in a series of adult patients affected by ITMC. METHODS: We analyzed retrospectively 27 adult patients (age > 18 years) diagnosed with ITMC who underwent surgery between 1994 and 2013. We investigated the demographic data, presenting symptoms, otoscopic findings, disease location, surgical technique, postoperative complications and outcomes. RESULTS: Diagnosis was made on the basis of a white mass seen through the tympanic membrane in 24 cases and on explorative surgery in the remaining 3 patients. In 16 cases cholesteatoma was related to an acquired cause, while in 11 ears a congenital origin was supposed. Hearing loss was the chief complaint and it was present in 19 (70.3%) subjects. Cholesteatoma was managed by purely endaural or retroauricular transcanal tympanoplasty in 12 cases, by planned staged canal wall up mastoidectomy in 10 cases (37%), by canal wall down mastoidectomy in 3 cases, and by modified Bondy technique in 2 cases. A recurrent cholesteatoma was observed in one ear; one patient experienced a postoperative profound sensorineural hearing loss. CONCLUSIONS: ITMC in adults may have both congenital and acquired origin. It may grow silently over many years and develops into a massive size before being detected. Each patient's management should be tailored to clinical findings.

Long-term clinical outcomes of cochlear implantation in children with symptomatic epilepsy.

OBJECTIVES: To describe long-term clinical outcomes of cochlear implantation in deaf children with symptomatic epilepsy. MATERIALS AND METHODS: A retrospective data analysis review of patients implanted at the Cochlear Implant Center of the University of Parma, Italy, was performed, searching for implanted children with a confirmed diagnosis of symptomatic epilepsy. Clinical data, imaging findings, pre- and post-operative epilepsy pattern and EEG traces were analyzed; communicative skills were assessed using the Profile of Actual Linguistic Skills. RESULTS: Search retrieved two patients affected by profound bilateral sensorineural hearing loss and symptomatic epilepsy (associated respectively with methylmalonic acidemia and cerebral palsy). After careful parental counselling both patients were offered and underwent cochlear implantation. Activation and use of cochlear implant did not determine substantial changes of pre-existing seizure pattern and EEG traces. Both patients showed substantial development of their communicative abilities. CONCLUSIONS: Cochlear implantation in children with symptomatic epilepsy did not determine variations in seizure pattern or EEG traces. Both patients experienced substantial benefit from cochlear implantation.

Management of the cervico-petrous internal carotid artery in class C tympanojugular paragangliomas.

BACKGROUND: Management of the cervical and petrous internal carotid artery (ICA) is frequently required in Fisch class C tympanojugular paragangliomas (TJPs). The purpose of this study was to discuss the perioperative intervention of the ICA in patients who underwent surgical resection of a TJP. METHODS: A retrospective study of 237 patients surgically treated for Fisch class C TJPs was done to identify cases that required ICA management. RESULTS: Management of the ICA was required in 176 patients (74.2%). Forty-two patients required just an ICA decompression, 88 underwent a subperiosteal dissection, 19 underwent subadventitial dissection without intraluminal stenting, 17 underwent subadventitial dissection with intraluminal stenting, and 12 underwent arterial resection after permanent balloon occlusion. There were no complications associated with the endovascular procedures. Gross total tumor resection was achieved in 91.5% of the cases. CONCLUSION: Preoperative endovascular intervention, in selected cases, facilitates gross total tumor removal and significantly reduces the risk of an intraoperative ICA injury. © 2015 Wiley Periodicals, Inc. Head Neck 38: 899-905, 2016.

FDG-PET/CT appearance of injected silicone particles (VOX Implants®) in head and neck tissues.

OBJETIVES: In head and neck surgery, Positron Emission Tomography/Computed Tomography imaging (FDG-PET/CT) is often used to identify primary tumor site in patients with unknown primary carcinoma, to predict response after chemoradiotherapy and in some cases, to detect recurrence. To rehabilitate swallowing after surgery in patients with persistent dysphagia, an injectable suspension of silicone (VOX® Implants) can be used to reduce the gaps in the neoglottis. The purpose of this report is to document the PET appearance of PDMS in a series of 3 patients who underwent partial laryngectomy with subsequent VOX® Implants injection. MATERIAL AND METHODS: a retrospective chart and imaging review was performed at our institution. Three patients were identified and included in the study. Appearance of PDMS at PET was described and discussed. RESULTS: An increased uptake of 2-fluoro-2-deoxy-d-glucose (FDG) was noticed at PET in all patients. CONCLUSIONS: the increased uptake was possibly due to active inflammatory reactions that are necessary for tissue integration of textured silicone particles.

Cochlear implantation in irradiated tympanojugular paraganglioma.

OBJECTIVE: Indications for cochlear implantation have extended progressively over the years both from an audiological and clinical point of view, including the introduction of cochlear implants (CI) for hearing rehabilitation following skull base surgeries and neurotological procedures. In the past, cochlear implantation has been used for hearing rehabilitation in radiation-induced sensorineural hearing loss in nasopharyngeal and tonsillar carcinoma with successful outcomes. Here we describe a similar outcome following total deafness after tympanojugular paraganglioma irradiation, which is also the first such report in literature. DESIGN: The irradiated ear of this patient was implanted with a CI through standard posterior tympanotomy. STUDY SAMPLE: A 26 year old male with multiple paragangliomas with bilateral deafness, secondary to surgery on one ear and radiotherapy for the opposite ear. RESULTS: After a follow-up of 48 months, no local complications have occurred and the hearing results have remained stable with 100% sentence recognition. CONCLUSIONS: Bilateral and multiple paragangliomas are rare tumors. Despite the modality of treatment, the hearing is almost always compromised. In these patients, cochlear implantation offers a new perspective for hearing restoration. This report demonstrates that cochlear implantation can be effectively performed after tympanojugular paraganglioma irradiation with long-lasting, satisfactory results, even in the presence of residual tumor.

Cochlear implantation in patients with Cogan syndrome: long-term results.

The objective of this study was to evaluate the long-term outcomes of patients with Cogan syndrome (CS) who have undergone cochlear implantation. Subjects consisted of 12 cochlear implant users with a typical form of CS. Measures included word and sentence recognition scores. The speech recognition performance was rated before cochlear implantation and at 1 and 5 years after implantation. The speech materials were presented in quiet only condition. The mean 12-month post-operative word and sentence recognition scores were 91.4 and 93.1%, respectively. Five years after implantation, the group means for word and sentence recognition tests were 94 and 96.3%, respectively. No patients in this series experienced flap complication or other local or systemic complications. This long-term study on 12 subjects with CS over 5 years of cochlear implant use reveals that cochlear implantation is safe in the long term and provides excellent and stable hearing results.

Successful cochlear implantation in two profoundly deafened patients with neurofibromatosis type 1: further evidence to support a cochlear site of lesion.

OBJECTIVE: To investigate the usefulness of cochlear implantation and to examine the evidence for a cochlear or retrocochlear site of lesion for deafness in Neurofibromatosis Type 1 (NF1). PATIENTS: Two profoundly deafened NF1 patients who underwent cochlear implantation. INTERVENTIONS: Preoperative electrophysiologic and audiologic evaluation including pure-tone audiometry, auditory brainstem response, speech audiometry, otoacoustic emission, and promontory stimulation test. Intraoperative electrophysiologic tests were also performed. MAIN OUTCOME MEASURES: Results of preoperative diagnostic workup. Cochlear implant performances were evaluated in the auditory-only condition in both closed-set and open-set formats. RESULTS: Preoperative diagnostic assessment suggested cochlear impairment with preserved auditory pathway at the basis of the sensorineural hearing loss in both patients. Intraoperative electrically evoked auditory nerve and brainstem responses confirmed the integrity of the neural population all along the length of the cochlea. To date, with a follow-up of 2 and 10 years, both patients gained excellent open-set, auditory-only, speech understanding and were able to converse on the telephone without a code. CONCLUSION: Cochlear impairment with preserved auditory pathways can be responsible for sensorineural hearing loss in NF1 patients. When their hearing deficit meets criteria for cochlear implantation, this type of auditory rehabilitation can restore social hearing in these patients while dramatically improving their quality of life.

Lower cranial nerves function after surgical treatment of Fisch Class C and D tympanojugular paragangliomas.

The aim of this study was to report the postoperative lower cranial nerves (LCNs) function in patients undergoing surgery for tympanojugular paraganglioma (TJP) and to evaluate risk factors for postoperative LCN dysfunction. A retrospective case review of 122 patients having Fisch class C or D TJP, surgically treated from 1988 to 2012, was performed. The follow-up of the series ranged from 12 to 156 months (mean, 39.4 ± 32.6 months). The infratemporal type A approach was the most common surgical procedure. Gross total tumor removal was achieved in 86% of cases. Seventy-two percent of the 54 patients with preoperative LCN deficit had intracranial tumor extension. Intraoperatively, LCNs had to be sacrificed in 63 cases (51.6%) due to tumor infiltration. Sixty-six patients (54.09%) developed a new deficit of one or more of the LCNs. Of those patients who developed new LCN deficits, 23 of them had intradural extension. Postoperative follow-up of at least 1 year showed that the LCN most commonly affected was the CN IX (50%). Logistic regression analysis showed that intracranial transdural tumor extension was correlated with the higher risk of LCN sacrifice (p < 0.05). Despite the advances in skull base surgery, new postoperative LCN deficits still represent a challenge. The morbidity associated with resection of the LCNs is dependent on the tumor's size and intradural tumor extension. Though no recovery of LCN deficits may be expected, on long-term follow-up, patients usually compensate well for their LCNs loss.

Open vs closed type congenital cholesteatoma of the middle ear: two distinct entities or two aspects of the same phenomenon?

OBJECTIVE: The clinical features and surgical results of "closed type" versus "open type" congenital cholesteatoma were compared in order to analyse the differences between the two forms; whether the morphology of the disease may have a role in the staging systems has been also evaluated. PATIENTS AND METHODS: We reviewed retrospectively 95 patients (96 ears) who underwent surgery for congenital cholesteatoma over a 15-year period focusing on the clinical differences between open and closed type congenital cholesteatoma. RESULTS: Seventy-one patients (74%) had a closed-type and 25 (26%) an open type congenital cholesteatoma. Our study confirmed the higher prevalence of the closed type, as well as, a younger age at initial diagnosis compared with the open type congenital cholesteatoma. Other differences between the two forms were: modality of diagnosis (pathognomonic otoscopy in 100% of the closed type and in 40% of the open type), positive history for otitis media with effusion (51.4% in closed type vs 20% in open type), involvement of the tympanic membrane quadrants (anterior quadrants were more frequently involved in the closed forms, whereas posterior quadrants were more frequently involved in the open forms), disease extension and aggressiveness. A residual cholesteatoma was found in 6 out of the 71 patients (8.4%) with a closed type congenital cholesteatoma and in 10 out of the 25 patients (40%) with an open type congenital cholesteatoma. After adjusting for potential confounders, open-type congenital cholesteatoma was significantly associated with residual cholesteatoma compared to the closed-type (odds ratio [OR] 7.39, 95% confidence interval [CI] 1.10-49.77, p=0.03). CONCLUSION: This study confirmed that the open congenital cholesteatoma has global clinical features that are uniquely different from the classical closed form. These differences could reflect a distinct pathogenesis, but there is no proof of this to date. The classification of the congenital cholesteatoma could be further refined by adding the morphologic type of the disease.