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Objective: To make evidence-based recommendations for the treatment of Single-Sided Deafness (SSD) in children and adults., Methods: Task force members were instructed on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on SSD were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions., Results: The topics were divided into 3 parts: (1) Impact of SSD in children; (2) Impact of SSD in adults; and (3) SSD in patients with temporal bone tumors., Conclusions: Decision-making for patients with SSD is complex and multifactorial. The lack of consensus on the quality of outcomes and on which measurement tools to use hinders a proper comparison of different treatment options. Contralateral routing of signal hearing aids and bone conduction devices can alleviate the head shadow effect and improve sound awareness and signal-to-noise ratio in the affected ear. However, they cannot restore binaural hearing. Cochlear implants can restore binaural hearing, producing significant improvements in speech perception, spatial localization of sound, tinnitus control, and overall quality of life. However, cochlear implantation is not recommended in cases of cochlear nerve deficiency, a relatively common cause of congenital SSD., (Copyright © 2024. Published by Elsevier España S.L.U.)

Objective: To make evidence-based recommendations for the indications and complications of Cochlear Implant (CI) surgery in adults and children., Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on cochlear implantation were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions., Results: The topics were divided into 2 parts: (1) Evaluation of candidate patients and indications for CI surgery; (2) CI surgery - techniques and complications., Conclusions: CI is a safe device for auditory rehabilitation of patients with severe-to-profound hearing loss. In recent years, indications for unilateral hearing loss and vestibular schwannoma have been expanded, with encouraging results. However, for a successful surgery, commitment of family members and patients in the hearing rehabilitation process is essential., (Copyright © 2024 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier España S.L.U. All rights reserved.)

Purpose: While cochlea is adult size at birth, etiologies and bone density may differ between children and adults. Differences in neural response thresholds (tNRT) and the spread of excitation (SOE) width may impact the use of artificial intelligence algorithms in speech processor fitting., Aim: To identify whether neural response telemetry threshold and spread of excitation width are similar in adults and children., Methods: Retrospective cross-sectional study approved by the Ethical Board. Intraoperative tNRT and SOE recordings of consecutive cochlear implant surgeries in adults and children implanted with Cochlear devices (Cochlear™, Australia) were selected. SOE was recorded on electrode 11 (or adjacent, corresponding to the medial region of the cochlea) through the standard forward-masking technique in Custom Sound EP software, which provides SOE width in millimeters. Statistical comparison between adults and children was performed using the Mann-Whitney test (p ≤ 0.05)., Results: Of 1282 recordings of intraoperative evaluations, 414 measurements were selected from children and adults. Despite the tNRT being similar between adults and children, SOE width was significantly different, with lower values in children with perimodiolar arrays. Besides, it was observed that there is a difference in the electrode where the SOE function peak occurred, more frequently shifted to electrode 12 in adults implanted. In straight arrays, there was no difference in any of the parameters analyzed on electrode 11., Conclusion: Although eCAP thresholds are similar, SOE measurements differ between adults and children in perimodiolar electrodes., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction  Facial nerve stimulation (FNS) is a complication in cochlear implant (CI) when the electrical current escapes from the cochlea to the nearby facial nerve. Different management to reduce its effects are available, although changes might result in a less-than-ideal fitting for the CI user, eventually reducing speech perception. Objective  To verify the etiologies that cause FNS, to identify strategies in managing FNS, and to evaluate speech recognition in patients who present FNS. Methods  Retrospective study approved by the Ethical Board of the Institution. From the files of a CI group, patients who were identified with FNS either during surgery or at any time postoperatively were selected. Data collection included: CI manufacturer, electrode array type, age at implantation, etiology of hearing loss, FNS identification date, number of electrodes that generated FNS, FNS management actions, and speech recognition in quiet and in noise. Results  Data were collected from 7 children and 25 adults. Etiologies that cause FNS were cochlear malformation, head trauma, meningitis, and otosclerosis; the main actions included decrease in the stimulation levels followed by the deactivation of electrodes. Average speech recognition in quiet before FNS was 86% and 80% after in patients who were able to accomplish the test. However, there was great variability, ranging from 0% in quiet to 90% of speech recognition in noise. Conclusion  Etiologies that cause FNS are related to cochlear morphology alterations. Facial nerve stimulation can be solved using speech processor programming parameters; however, it is not possible to predict outcomes, since results depend on other variables., Competing Interests: Conflict of Interests The authors have no conflict of interests to declare., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ( https://creativecommons.org/licenses/by/4.0/ ).)

Introduction  Recently, there have been significant advancements in transcanal endoscopic ear surgery (TEES). The combination of rigid and thin otoendoscopes with high-definition cameras enabled a less invasive transcanal access to the middle ear and a clearer view of the surgical field. Several surgeons have recently published studies about cholesteatoma resection via transcanal endoscopic surgery, even in cases where the disease has extended to the mastoid, requiring transcanal endoscopic mastoidectomy. Objectives  To analyze the currently available literature on transcanal endoscopic inside-out mastoidectomy, and to determine its efficacy as a surgical technique by evaluating the disease's relapse/recurrence rate. Data Synthesis  Initially, the titles and abstracts of articles identified were analyzed. At this stage, 117 articles were analyzed, 97 of which were excluded for not meeting the inclusion criteria. The 20 remaining articles were further evaluated. The articles were classified on the basis of five levels of scientific evidence. Final Comments  The analysis of the studies showed that the transcanal endoscopic approach is effective in providing access to the attic or antrum, especially in cases of sclerotic mastoids. There was only one study with grade A recommendation, which showed the efficacy of endoscopic ear surgery in the treatment of cholesteatoma. Furthermore, there were three studies with grade B recommendation, showing less relapse/recurrence after TEES. More studies with grade A and B recommendations are needed to better evaluate the effectiveness of TEES, especially compared with that of traditional microscopic surgery., Competing Interests: Conflict of Interests The authors have no conflict of interests to declare., (Fundação Otorrinolaringologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

Hearing loss is one of the most common sensory defects, affecting 5.5% of the worldwide population and significantly impacting health and social life. It is mainly attributed to genetic causes, but their relative contribution reflects the geographical region's socio-economic development. Extreme genetic heterogeneity with hundreds of deafness genes involved poses challenges for molecular diagnosis. Here we report the investigation of 542 hearing-impaired subjects from all Brazilian regions to search for genetic causes. Biallelic GJB2/GJB6 causative variants were identified in 12.9% (the lowest frequency was found in the Northern region, 7.7%), 0.4% carried GJB2 dominant variants, and 0.6% had the m.1555A > G variant (one aminoglycoside-related). In addition, other genetic screenings, employed in selected probands according to clinical presentation and presumptive inheritance patterns, identified causative variants in 2.4%. Ear malformations and auditory neuropathy were diagnosed in 10.8% and 3.5% of probands, respectively. In 3.8% of prelingual/perilingual cases, Waardenburg syndrome was clinically diagnosed, and in 71.4%, these diagnoses were confirmed with pathogenic variants revealed; seven out of them were novel, including one CNV. All these genetic screening strategies revealed causative variants in 16.2% of the cases. Based on causative variants in the molecular diagnosis and genealogy analyses, a probable genetic etiology was found in ~ 50% of the cases. The present study highlights the relevance of GJB2/GJB6 as a cause of hearing loss in all Brazilian regions and the importance of screening unselected samples for estimating frequencies. Moreover, when a comprehensive screening is not available, molecular diagnosis can be enhanced by selecting probands for specific screenings., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The aim of this study was to evaluate the contribution of the CROS system on the head shadow effect in unilateral implant users., Methods: Prospective cross-sectional study, approved by the ethics committee under protocol 2.128.869. Eleven adults with post-lingual deafness users of unilateral Advanced Bionics CI were selected. Speech recognition was evaluated with recorded words presented at 65dBA at 0o azimuth and at 90o on the side contralateral to the CI, with noise at 55dBA, using CI alone and CI + CROS system. The results were analyzed using paired t-test with a 0.05 alpha., Results: The mean speech recognition scores were significantly better with CI + CROS in relation to the condition of CI alone (p <0.05, p <0.005 and p <0.005 respectively). In the presentation at 0o azimuth, no significant differences were found., Conclusion: Users of unilateral CI without useful residual hearing for the use of hearing aids or unable to undergo bilateral surgery can benefit from the CROS device for speech recognition, especially when the speech is presented on the side contralateral to the CI.

Purpose: To assess whether there is an interference of the spread of excitation (SOE) on speech recognition., Methods: Retrospective cross-sectional study, approved by the institution's ethics committee (CAAE03409212.8.0000.0068). Adult patients with intraoperative neural response telemetry (NRT) performed on electrodes 6, 11 and 16 implanted with Cochlear Ltd (Sydney, Australia) devices were selected. Patients with partial array insertion, pre-lingual hearing loss, deafness etiology due to and CI experience less than 12 months were excluded. SOE was recorded at 10 current units above the NRT threshold (tNRT) and its width in millimeters was collected at point 0.75 of the function. Speech recognition test was 25-recorded monosyllables list, presented at 65 dBHL at 0° azimuth in a sound treated booth. The analysis was divided into groups by electrode array type, regarding the tNRT, SOE width, SOE's peak amplitude and electrode peak., Results: A 126 SOE measurements of the 3 tested electrodes were obtained from 43 patients. Patients with straight array had significantly wider SOE, greater peak amplitude at electrode 6 and higher tNRTs. In the perimodiolar array, there was a negative correlation between SOE and monosyllables recognition at electrodes 6 and 11, and in the combined average of the three electrodes, with a significant difference in electrode 11. Sixty-six percent of the SOE measurements had their peak shifted to adjacent electrodes., Conclusion: It was observed, in perimodiolar array, the greater the dispersion of electrical current, the worse the speech recognition, especially in the medial electrode.

Introduction: Cochlear implants (CI) programming is based on both the measurement of the minimum levels required to stimulate the auditory nerve and the maximum levels to generate loud, yet comfortable loudness. Seeking for guidance in the adequacy of this programming, the cortical auditory evoked potentials (CAEP) have been gaining space as an important tool in the evaluation of CI users, providing information on the central auditory system., Objective: To evaluate the influence of mishandling of electrical stimulation levels on speech processor programming on hearing thresholds, speech recognition and cortical auditory evoked potential in adult CI users., Material and Methods: This is a prospective cross-sectional study, with a sample of adult unilateral CI users of both sexes, aged at least 18 years, post-lingual deafness, with minimum experience of 12 months of device use. Selected subjects should have average free field hearing thresholds with cochlear implant equal to or better than 34 dBHL and monosyllable recognition different from 0%. Individuals who could not collaborate with the procedures or who had no CAEP recordings were excluded. Participants were routinely programmed, and the map was named MO (optimized original map). Then three experimentally wrong maps were made: optimized original map with 10 current units below the maximum comfort level (C), named MC- (map minus C); optimized original map with minus 10 current units at minimum threshold level (T), named MT- (map minus T) and optimized original map with 10 current units above minimum level (T), named MT + (map plus T). In all programs, participants underwent free-field auditory thresholds from 250Hz to 6000Hz, recorded sentences and monosyllabic recognition tests presented at 65dB SPL in quiet and in noise, and free field CAEP evaluation. All tests were performed in an acoustically treated booth, in a randomized order of map presentation. Data were compared by Wilcoxon test., Results: Thirty individuals were selected and signed an informed consent form. The MC- map provided worsening of all free field thresholds, quiet and noise speech recognition, and P1 wave latency delay with significant difference from the results with the MO map. The MT- map worsened the hearing thresholds and statistically significantly reduced the P2 wave latency; MT+ map improved free field thresholds except 6000Hz, worsening speech recognition, without statistical significance., Conclusions: The results suggest that maximum levels below the optimal thresholds lead to worse cochlear implant performance in both hearing thresholds and speech recognition tests in quiet and noise, increasing CAEP component P1 latency. On the other hand, the manipulation of minimum threshold levels showed alteration in audibility without significant impact on speech recognition., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Introduction  The use of cochlear implants and hearing aids (bimodal) has been growing with the expansion of the indication for them, and it is important to ensure protocols so that there is a balance of the loudness regarding the two devices. Objective  To evaluate if the limited complex sounds present in the frequency bands of the current devices enable the balance of the loudness in adult users of bimodal stimulation, and to analyze if speech recognition improves after balancing. Methods  A prospective cross-sectional study with convenience sampling. The sample was composed of 25 adults who had used either a cochlear implant for at least 6 months or a contralateral hearing aid, with a mean age of 46 years. The balancing of the loudness was performed in an acoustic room with the computer's sound box (0° azimuth at 70 dB SPL). The instrumental sounds were filtered through eight different frequency bands. The patients used both hearing devices and were asked if the sound was perceived to be louder in one of the ears or centrally. The speech test was evaluated with sentence silence (65 dB SPL) and/or noise signal ratio of 0 dB/+ 10 dB in free field at 0° azimuth, before and after balancing. Results : Out of the 25 patients, 5 failed to achieve balance at every tested frequency, and 3 achieved balance at almost every frequency, except 8 kHz. There was a significant difference between the speech recognition test only in silence before and after balancing. Conclusion : Most patients achieved sound equalization at all evaluated frequencies under the complex-sound protocol. Additionally, most patients experienced improved speech recognition after balancing., Competing Interests: Conflicts of Interest The authors have none to declare., (Fundação Otorrinolaringologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

Background: The spread of excitation (SOE) and auditory nerve recovery function (REC) are objective measures recorded by neural response telemetry and may interfere in cochlear implant (CI) stimulation., Objective: To analyze and correlate SOE with the refractory periods in subjects with pre- and postlingual deafness implanted with different electrode arrays., Methods: This was a retrospective study of 323 ears separated by perimodiolar or straight arrays and by pre- or postlingually deaf recipients. Measures were collected intraoperatively on electrode 11. The SOE width was measured in millimeters at the 0.75 point of the curve, and the relative (tau) and absolute (t0) refractory periods were measured in microseconds., Results: There was a statistical correlation between the SOE and the t0 in the patients with postlingual deafness implanted with the perimodiolar array. The SOE width was statistically different between the straight and perimodiolar arrays and between the pre- and postlingual groups in the perimodiolar array. Tau was statistically different between the pre- and postlingual groups with the straight array and the t0, between the pre- and postlingual groups with the perimodiolar array. Neural response threshold and amplitude of the neural response were not statistically different among groups., Conclusion: There was a correlation between SOE width and t0 only in patients with acquired deafness. The findings suggest that different factors influence SOE and REC, considering SOE is different according to the electrode array and REC being different according the onset of deafness., (© 2020 S. Karger AG, Basel.)

Objective: The aim of this study is to characterize the development of auditory and language skills in children during the first 3 years of auditory brainstem implant (ABI) use., Method: It is a retrospective longitudinal analysis of auditory and language skills in 12 children and pre-adolescents with pre-lingual deafness following ABI surgery (mean age at surgery: 4 years; age range: 2-11 years). Responses were analyzed aboutInfant Toddler Meaningful Auditory Integration Scale (IT-MAIS), MAIS, and Meaningful Use of Speech Scale (MUSS) at 1, 3, 6, 12, 18, 24, and 36 months after ABI activation., Results: Maximum IT-MAIS/MAIS and MUSS scores after 3 years of ABI use were 45.35% and 35.28%, respectively., Conclusion: Pediatric patients exhibit slow progressive development of auditory and language skills following ABI activation., Competing Interests: Declaration of competing interest There are no relevant conflict of interest for both authors for this study., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Objective: To verify changes in the perceptual and acoustic vocal parameters in prelingual hearing-impaired adults with cochlear implants after vocal rehabilitation., Hypothesis: Auditory feedback restoration alone after cochlear implant is not enough for vocal adjustments. A targeted and specific voice therapy intervention is required., Study Design: Prospective and pre-post repeated measures design., Methods: Twenty literate adults with severe to profound prelingual bilateral sensorineural hearing loss participated in the study; individuals were implanted late and were fluent users of oral language. Ages ranged from 17 to 48 years. All individuals presented normal results in laryngoscopy, and hearing thresholds with the cochlear implant were over 40 dB HL. Individuals were randomly distributed into two groups: Group 1 (treatment group) and Group 2 (control group), both with ten patients each, five men and five women, matching mean age and hearing deprivation time before the cochlear implantation. Patients from Group 1 underwent a protocol of vocal therapy including 12 individual sessions with the same clinician. Group 2 only underwent vocal recordings. The vocal recordings occurred before and after the participation in the therapy protocol for Group 1 and after the same period, 3 months later, without any intervention, for Group 2. The recording sessions used the Consensus Auditory-Perceptual Evaluation of Voice protocol sentence reading and emission of sustained vowel /a/. Auditory-perceptual evaluation of voices was performed by three judges, and the acoustical analysis used the Praat program., Results: Statistically significant reductions in the overall vocal degree, vocal instability, and degree of resonance change were observed after vocal rehabilitation in Group 1. Statistically, individuals from Group 1 did not differ in regard to the modification of acoustic parameters. Group 2 did not present significant changes in any of the analyzed parameters., Conclusions: The cochlear implanted adults submitted to vocal rehabilitation presented changes in the auditory-perceptual parameters, with reduction of the overall voice severity, vocal instability, and degree of resonance after vocal intervention. There were no changes in the acoustic parameters in the implanted prelingual hearing-impaired adult subjects., (Copyright © 2018 The Voice Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Currently, cochlear implant procedures are becoming increasingly broad and have greatly expanded. Bilateral cochlear implants and cochlear implants are more frequently applied in children. Our hypothesis is that the video head impulse test may be more sensitive than the caloric test in detecting abnormal vestibular function before cochlear implant surgery. The objective of this study was to compare the video head impulse test and caloric test results of patients selected for cochlear implant procedures before surgery., Methods: The patients selected for cochlear implant surgery were submitted to a bithermal caloric test and video head impulse test., Results: By comparing angular slow phase velocity values below 5° in the bithermal caloric test (hypofunction) and video head impulse test with a gain lower than 0.8, we identified 37 (64.9%) patients with vestibular hypofunction or canal paresis and 21 (36.8%) patients with abnormal video head impulse test gain before the cochlear implant procedure. Of the 37 patients with caloric test vestibular hypofunction, 20 (54%) patients exhibited an abnormal gain in the video head impulse test., Conclusion: The caloric test is more sensitive than the video head impulse test (Fisher's exact test, p=0.0002) in detecting the impaired ear before cochlear implant delivery. The proportion of caloric test/video head impulse test positive identification of abnormal vestibular function or caloric test/video head impulse test sensitivity was 1.8:1.

Background: Cochlear implantation (CI) is the gold standard therapy for profound or severe sensorineural hearing loss. It is a safe surgical procedure but, because of the proximity of the cochlea and vestibule, postoperative vestibular disorder may occur. Our hypothesis is that the video head impulse test (vHIT) may be a good tool to achieve a topographic diagnosis of dizziness in the early postoperative period after CI., Aims/objectives: To evaluate patients with instability, imbalance and vertigo between 7 and 14 days after CI procedure., Material and Methods: A total of 31patients scheduled for unilateral CI were included in this study. vHIT for horizontal semicircular canal was performed before CI and between days 7 to 14 after the surgery., Results: Six subjects had dizziness complaints after CI: instability (N = 2), imbalance (N = 2) and vertigo (N = 2). The postoperative vHIT test turned abnormal only in subjects with vertigo as compared to the preoperative vHIT test results., Conclusion and Significance: vHIT is a good vestibular function test during the first 2 weeks after CI surgery when vertigo is the main complaint.

Introduction  The cochlear implants centers in the world are studying the cochlear implantation in children with multiple handicaps. Objective  To develop a questionnaire to analyze the subjective benefits of the cochlear implantation in multiple handicapped children according to their parent's perspective. Methods  A questionnaire was applied to 14 families of multiple handicapped children, aging from 2-12 years old and having from 11 months to 11 years of implant use. Results  The social-emotional abilities were improved because of many factors, such as: auditory exposure, which happened in 84% of the children; recognition of their own names, which increased in 56%; and development of eye contact, in 28% of the subjects. Other benefits appeared to be: music appreciation and more attention and adherence to other therapies and school activities. Besides, some children became interested in objects, playing with other children, and more adapted to daily routines. Thirty-five percent of the children acquired oral language, mainly the bilaterally implanted, while 14% of them were engaged in sign language. Although all of the children showed a significant improvement in communication, the emotional issues of some families and the severity of the handicaps negatively impacted the outcomes. In spite of the families' acknowledgement of some benefits, the diagnosis of autism spectrum disorder caused frustration, requiring a readjustment of the expectations. Conclusion  The questionnaire turned out to be an adequate tool to reveal the social-emotional benefits of cochlear implantation. Although oral language was not the major outcome in these cases, the cochlear implant benefits involved the whole family. All of the families recommended the implant to other children in a similar situation.

Introduction: An auditory brainstem implant (ABI) is an option for auditory rehabilitation in patients with totally ossified cochleae who cannot receive a conventional cochlear implant., Objective: To evaluate the outcomes in audiometry and speech perception tests after the implantation of an ABI via the extended retrolabyrinthine approach in patients with postmeningitis hearing loss., Materials and Methods: Ten patients, including children and adults, with postmeningitis hearing loss and bilateral totally ossified cochleae received an ABI in a tertiary center from 2009 to 2015. The extended retrolabyrinthine approach was performed in all the patients by the same surgeons. A statistical analysis compared pure tonal averages and speech perception tests before and at least 12 months after the ABI activation., Results: Eight patients (80%) showed improvements in tonal audiometry and the word and vowel perception tests after an average follow-up of 3.3 years. Two patients recognized up to 40% of the closed-set sentences without lip-reading. Two patients had no auditory response., Conclusions: The ABI improved hearing performance in audiometry and speech perception tests in cases of postmeningitis hearing loss. The extended retrolabyrinthine approach is a safe surgical option for patients with postmeningitis hearing loss and bilateral totally ossified cochleae.

Introduction: Cochlear implants are undeniably an effective method for the recovery of hearing function in patients with hearing loss., Objective: To describe the preoperative vestibular assessment protocol in subjects who will be submitted to cochlear implants., Methods: Our institutional protocol provides the vestibular diagnosis through six simple tests: Romberg and Fukuda tests, assessment for spontaneous nystagmus, Head Impulse Test, evaluation for Head Shaking Nystagmus and caloric test., Results: 21 patients were evaluated with a mean age of 42.75±14.38 years. Only 28% of the sample had all normal test results. The presence of asymmetric vestibular information was documented through the caloric test in 32% of the sample and spontaneous nystagmus was an important clue for the diagnosis. Bilateral vestibular areflexia was present in four subjects, unilateral arreflexia in three and bilateral hyporeflexia in two. The Head Impulse Test was a significant indicator for the diagnosis of areflexia in the tested ear (p=0.0001). The sensitized Romberg test using a foam pad was able to diagnose severe vestibular function impairment (p=0.003)., Conclusion: The six clinical tests were able to identify the presence or absence of vestibular function and function asymmetry between the ears of the same individual., (Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.)

Lateral and sigmoid sinus malformations are uncommon and dangerous anatomical variations that surgeons may encounter when performing a retroauricular approach. We report three cases of rare temporal bone venous sinus anomalies seen in patients who underwent cochlear implant surgery. The first patient had a diagnosis of CHARGE syndrome and presented a bilateral persistent petrosquamosal sinus with sigmoid sinus agenesis, which made mastoidectomy for cochlear implantation difficult. The second patient presented an anomalous venous lake in the occipital region, which communicated the left dural venous sinuses with a conglomerate of pericranial vessels in the left nuchal region, also consistent with left sinus pericranii. The third patient presented with an extracranial sigmoid sinus that produced a troublesome bleeding immediately after the muscular-periosteal flap incision was performed., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Introduction  Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear implantation as a salvage for their new condition. Is a late implantation beneficial to these children? Objective  The objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age. Methods  This is a retrospective study of ten patients diagnosed with US1. We collected pure-tone thresholds and speech perception tests from pre and one-year post implant. Results  Average age at implantation was 18.9 years (5-49). Aided average thresholds were 103 dB HL and 35 dB HL pre and one-year post implant, respectively. Speech perception was only possible to be measured in four patients preoperatively, who scored 13.3; 26.67; 46% vowels and 56% 4-choice. All patients except one had some kind of communication. Two were bilingual. After one year of using the device, seven patients were able to perform the speech tests (from four-choice to close set sentences) and three patients abandoned the use of the implant. Conclusion  We observed that detection of sounds can be achieved with late implantation, but speech recognition is only possible in patients with previous hearing stimulation, since it depends on the development of hearing skills and the maturation of the auditory pathways.

Introduction  After mastoidectomy, patients usually complain of bone depressions in the retroauricular region in the surgical site, especially in procedures that require extensive cortical resections. This causes inconveniences such as difficulty wearing glasses, cleaning, and aesthetics complaints. Objective  This study aims to describe a vascularized flap surgical technique that uses the mastoid cortical bone adhered to the periosteum, which is pedicled on the anterior portion and repositioned at the end of the surgery. This ensures the coverage of the mastoid cavity generated by surgery and prevents ear retraction into the cavity. This preliminary report describes the technique and intraoperative and immediate postoperative complications. Methods  After retroauricular incision, periosteal exposure is performed. A U-shaped incision is required for the procedure and delimits a periosteum area appropriate to the size of the mastoidectomy. The cortical bone is opened using a 2.5 mm drill around the perimeter of the "U," at a 3 mm depth. A chisel is introduced through the surface cells of the mastoid, and a hammer evolves into the anterior direction. The flap is lifted, leaving the periosteum adhered to it and forming a cap. The flap is anteriorly fixed to not hinder the surgery, and repositioned at the end. The periosteum is then sutured to the adjacent periosteum. Results  The first 14 cases had no intraoperative complications and were firm and stable when digital pressure was applied during the intraoperative and immediate postoperative periods. Conclusion  The osteoplastic flap pedicle is a safe and simple procedure, with good results in the immediate postoperative period.

Central auditory pathway maturation in children depends on auditory sensory stimulation. The objective of the present study was to monitor the cortical maturation of children with cochlear implants using electrophysiological and auditory skills measurements. The study was longitudinal and consisted of 30 subjects, 15 (8 girls and 7 boys) of whom had a cochlear implant, with a mean age at activation time of 36.4 months (minimum, 17 months; maximum, 66 months), and 15 of whom were normal-hearing children who were matched based on gender and chronological age. The auditory and speech skills of the children with cochlear implants were evaluated using GASP, IT-MAIS and MUSS measures. Both groups underwent electrophysiological evaluation using long-latency auditory evoked potentials. Each child was evaluated at three and nine months after cochlear implant activation, with the same time interval adopted for the hearing children. The results showed improvements in auditory and speech skills as measured by IT-MAIS and MUSS. Similarly, the long-latency auditory evoked potential evaluation revealed a decrease in P1 component latency; however, the latency remained significantly longer than that of the hearing children, even after nine months of cochlear implant use. It was observed that a shorter P1 latency corresponded to more evident development of auditory skills. Regarding auditory behavior, it was observed that children who could master the auditory skill of discrimination showed better results in other evaluations, both behavioral and electrophysiological, than those who had mastered only the speech-detection skill. Therefore, cochlear implant auditory stimulation facilitated auditory pathway maturation, which decreased the latency of the P1 component and advanced the development of auditory and speech skills. The analysis of the long-latency auditory evoked potentials revealed that the P1 component was an important biomarker of auditory development during the rehabilitation process., Competing Interests: The authors have declared that no competing interests exist.

Purpose To evaluate satisfaction and quality of life of users of Auditory Implant Brainstem. Methods This is a cross-sectional and descriptive study conducted at Divisão de Clínica Otorrinolaringológica of Hospital das Clínicas of Faculdade de Medicina da Universidade de São Paulo, Brazil. For the research, 19 users of an Auditory Brainstem Implant answered the following questionnaires: KINDLR (Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents), for children and adolescents, their parents and/or caregivers; WHOQOL-BREF questionnaire, for adult participants; and the Satisfaction with Amplification in Daily Life (SADL) questionnaire culturally adapted to Brazilian Portuguese. Results The quality of life of children using Auditory Brainstem Implant from the perspective of their parents showed global results above average, as for most domains, except for the emotional well-being domain. Adults showed results above average for all domains. Regarding satisfaction with the device, the adult users of auditory brainstem implant were satisfied in general, except with regard to personal image. The parents of the children showed dissatisfaction in all subscales, except for the subscale of services and cost. Conclusion The results indicated that although patients are dissatisfied with the device in some aspects, overall the quality of life was rated as good for most of the aspects assessed.

Introduction: ASSR allow frequency-specific evaluation in intensities up to 120dB HL and detection of residual hearing in patients with severe-to-profound hearing loss., Aim: to compare ASSR thresholds and behavioral test results in children with suspected severe-to-profound hearing loss., Methods: Cross sectional study to compare ASSR and behavioral responses (VRA or audiometry) in 63 pediatric cochlear implant candidates (126 ears) aged between 6 and 72 months. We included children with normal otomicroscopy, absent responses to click-ABR and otoaccoustic emissions. We excluded children with inner ear malformations, auditory neuropathy spectrum disorder or who did not complete VRA or achieve EEG noise<30nV during the ASSR test. Air-conduction ASSR stimuli were continuous sinusoidal tones presented at 0.5, 1, 2 and 4kHz starting at 110dB HL. Behavioral thresholds were acquired with warble tones presented at 0.5, 1, 2 and 4kHz in each ear through insert or head phones at maximum presentation level of 120dB HL., Results: Behavioral thresholds were obtained in 36.7% (185/504) of all frequencies in all subjects, 9% in intensities >110dB HL. Among 504 ASSR measurements, 53 thresholds were obtained (10.5%). Overall 89.5% of the tested frequencies did not show any response at 110dB HL. Most responses were at 500Hz. Mean differences between behavioral and ASSR thresholds varied from 0.09 to 8.94dB. Twenty-seven comparisons of behavioral and ASSR thresholds were obtained: 12 at 0.5kHz, 9 at 1kHz, 5 at 2kHz and 1 at 4kHz. Absent responses were observed in both tests in 38.1% at 0.5kHz, 52.4% at 1kHz, 74.6% at 2kHz and 81.0% at 4kHz. Specificity was>90% at 1, 2 and 4kHz. In ears with no behavioral response at 120dB HL all ASSR thresholds were in the profound hearing loss range, 90% of them were ≥110dB HL., Conclusion: Among 63 pediatric CI candidates, absent responses to high-intensity ASSR was the major finding (specificity>90%) predicting behavioral thresholds in the profound hearing loss range. These findings can be helpful to confirm the decision for cochlear implantation., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Conclusions: Balance was improved at 365 days after CI in all patients. Caloric test findings were important determinants of balance outcomes over a year after CI. Pre-operative vestibular assessment findings should be documented because postural recovery over time depends on this information., Objective: To verify the importance of the caloric test as a pre-operative predictor tool of postural control after CI surgery., Methods: Prospective observational study made with 24 post-lingual deafness patients who underwent unilateral CI surgery. Vestibular assessments: questionnaire assessing vertigo, caloric tests, rotary chair (RC) testing, and computerized dynamic posturography (CDP), were sequentially performed for all patients before and 60, 120, 180, and 365 days after CI., Results: Thirteen patients (54.2%) reported dizziness before CI. At the end of the study, dizziness remained unchanged in one (7.7%) patient, ameliorated in 11 (84.6%), and worsened in one (7.7%). Baseline caloric tests identified 29.2% patients with normal reflexes, 33.3% with unilateral areflexia or hyporeflexia, 12.5% with bilateral hyporeflexia, and 25% with bilateral vestibular loss (BVL). Most patients exhibited objective improvements in postural stability. At 365 days, the CDP condition (particularly C5) and CS were higher for caloric tests responders at baseline than for those with BVL at baseline.

Introduction Difficulties with telephone use by adult users of cochlear implants (CIs) are reported as a limitation in daily life. Studies to improve the speech understanding of CI users on the telephone are scarce in the Brazilian scientific literature. Objective To develop and evaluate the effectiveness of a training program of auditory abilities on the telephone for an adult CI user. Resumed Report The subject was a 55-year-old woman with a degree in accounting who used a CI for 24 months. The program consisted of three stages: pretraining evaluation, eight sessions of advanced auditory abilities training, and post-training evaluation. Auditory abilities with CI were evaluated before and after training in three conditions: sound field, telephone with the speech processor in the microphone function, and telephone with the speech processor in the telecoil function. Speech recognition was assessed by three different lists: one with monosyllabic and dissyllabic words, another with nonsense syllables, and another one with sentences. The Client Oriented Scale of Improvement (COSI) was used to assess whether the needs established by the CI user in everyday telephone use situations improved after training. The auditory abilities training resulted in a relevant improvement in the percentage of correct answers in speech tests both in the telephone use conditions and in the sound field condition. Conclusion The results obtained with the COSI inventory indicated a performance improvement in all situations presented at the beginning of the program.

Conclusion: Topical dexamethasone associated with hyaluronic acid in cochlear implant surgery demonstrated a statistically significant difference in the preservation of low-frequency thresholds when compared with topical dexamethasone alone and a control group. Topical dexamethasone alone was not superior in hearing preservation when compared to the control group., Objective: To compare the effects of topical dexamethasone alone and associated with hyaluronic acid intraoperatively in hearing preservation in cochlear implantation., Methods: Eighteen severely to profoundly hearing-impaired adult patients with measurable hearing were divided into three groups preoperatively: cochlear implantation as a control group (group 1), cochlear implantation using topical dexamethasone intraoperatively (group 2), and cochlear implantation using topical dexamethasone associated with hyaluronic acid intraoperatively (group 3). Preimplant and postimplant low-frequency pure-tone averages (PTAs) were calculated from unaided audiograms at 125, 250, and 500 Hz., Results: The mean changes in the low-frequency PTA comparing postoperative against preoperative thresholds were 28.03 ± 6.77 dB in group 1, 30 ± 14.53 dB in group 2, and 7.23 ± 6.12 dB in group 3. There was statistical difference when comparing group 3 with groups 1 and 2 using one-way ANOVA (p = 0.002) followed by Scheffé post hoc test.

Hypothesis: This study aimed to evaluate whether there is a difference in the degree of intracochlear trauma when the cochlear implant electrode arrays is inserted through different quadrants of the round window membrane., Background: The benefits of residual hearing preservation in cochlear implant recipients have promoted the development of atraumatic surgeries. Minimal trauma during electrode insertion is crucial for residual hearing preservation., Methods: In total, 25 fresh human temporal bones were subjected to mastoidectomy and posterior tympanotomy. The cochlear implant electrode array was inserted through the anterosuperior quadrant of the round window membrane in 50% of the bones and through the anteroinferior quadrant in the remaining 50%. The temporal bones were dehydrated, embedded in epoxy, serially polished, stained, viewed through a stereomicroscope, and photographed with the electrode arrays in situ. The resulting images were analyzed for signs of intracochlear trauma., Results: Histological examinations revealed varying degrees of damage to the intracochlear structures, although the incidence and severity of intracochlear trauma were not influenced by the quadrant of insertion., Conclusions: The incidence and severity of intracochlear trauma were similar in all samples, irrespective of electrode array insertion through the anterosuperior or anteroinferior quadrant of the round window membrane.

The purpose of this study was to longitudinally assess the behavioral and electrophysiological hearing changes of a girl inserted in a CI program, who had bilateral profound sensorineural hearing loss and underwent surgery of cochlear implantation with electrode activation at 21 months of age. She was evaluated using the P1 component of Long Latency Auditory Evoked Potential (LLAEP); speech perception tests of the Glendonald Auditory Screening Procedure (GASP); Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS); and Meaningful Use of Speech Scales (MUSS). The study was conducted prior to activation and after three, nine, and 18 months of cochlear implant activation. The results of the LLAEP were compared with data from a hearing child matched by gender and chronological age. The results of the LLAEP of the child with cochlear implant showed gradual decrease in latency of the P1 component after auditory stimulation (172 ms-134 ms). In the GASP, IT-MAIS, and MUSS, gradual development of listening skills and oral language was observed. The values of the LLAEP of the hearing child were expected for chronological age (132 ms-128 ms). The use of different clinical instruments allow a better understanding of the auditory habilitation and rehabilitation process via CI.

Purpose: To verify the effectiveness of remote programming of cochlear implants by stimulation levels and results in the perception of speech and free-field audiometry tests., Methods: Twelve patients from both genders, aged between 18 and 59 years, users of internal cochlear implant and speech processor of the same model for at least 12 months, were selected. Both the remote programming (RP) and the live programming (LP) were performed on the same day, measuring the minimum (T) and maximum (C) stimulation levels of five electrodes with the interpolation of the remaining ones. Speech perception tests were applied using 65 dBSPL (recorded open context sentences and monosyllables). The patients were submitted to free-field audiometry at 250-8,000 Hz frequencies. The results for the RP and LP were compared., Results: Differences in mean of the T levels for three electrodes and the C levels for one electrode were found. No difference between the results was obtained in the speech perception tests and audiometric thresholds in the RP and LP., Conclusion: The RP is a simple and effective procedure for programming cochlear implant devices and, although there were differences in the stimulation levels of some electrodes, it did not interfere in the speech perception outcomes.

Objective: To review the outcomes of the fully implantable middle ear devices Carina and Esteem regarding the treatment of hearing loss., Data Sources: PubMed, Embase, Scielo, and Cochrane Library databases were searched., Study Selection: Abstracts of 77 citations were screened, and 43 articles were selected for full review. From those, 22 studies and two literature reviews in English directly demonstrating the results of Carina and Esteem were included., Data Extraction: There were a total of 244 patients ranging from 18 to 88 years. One hundred and 10 patients were implanted with Carina and with 134 Esteem. There were registered 92 males and 67 females. Five studies provided no information about patients' age or gender. From the data available, the follow-up ranged from 2 to 29.4 months., Data Synthesis: The comparison of the results about word recognition is difficult as there was no standardization of measurement. The results were obtained from various sound intensities and different frequencies. The outcomes comparing to conventional HAs were conflicting. Nevertheless, all results comparing to unaided condition showed improvement and showed a subjective improvement of quality of life., Conclusion: There are still some problems to be solved, mainly related to device functioning and price. Due to the relatively few publications available and small sample sizes, we must be careful in extrapolating these results to a broader population. Additionally, none of all these studies represented level high levels of evidence (i.e. randomized controlled trials).

Introduction Currently the cochlear implant allows access to sounds in individuals with profound hearing loss. The objective methods used to verify the integrity of the cochlear device and the electrophysiologic response of users have noted these improvements. Objective To establish whether the evoked compound action potential of the auditory nerve can appear after electrical stimulation when it is absent intraoperatively. Methods The clinical records of children implanted with the Nucleus Freedom (Cochlear Ltd., Australia) (CI24RE) cochlear implant between January 2009 and January 2010 with at least 6 months of use were evaluated. The neural response telemetry (NRT) thresholds of electrodes 1, 6, 11, 16, and 22 during surgery and after at least 3 months of implant use were analyzed and correlated with etiology, length of auditory deprivation, and chronological age. These data were compared between a group of children exhibiting responses in all of the tested electrodes and a group of children who had at least one absent response. Results The sample was composed of clinical records of 51 children. From these, 21% (11) showed no NRT in at least one of the tested electrodes. After an average of 4.9 months of stimulation, the number of individuals exhibiting absent responses decreased from 21 to 11% (n = 6). Conclusion It is feasible that absent responses present after a period of electrical stimulation. In our sample, 45% (n = 5) of the patients with intraoperative absence exhibited a positive response after an average of 4.9 months of continued electrical stimulation.

Introduction The complaints associated with the use of conventional amplifying hearing aids prompted research at several centers worldwide that ultimately led to the development of implantable devices for aural rehabilitation. Objectives To review the history, indications, and surgical aspects of the implantable middle ear hearing devices. Data Synthesis Implantable hearing aids, such as the Vibrant Soundbridge system (Med-El Corporation, Innsbruck, Austria), the Maxum system (Ototronix LLC, Houston, Texas, United States), the fourth-generation of Carina prosthesis (Otologics LLC, Boulder, Colorado, United States), and the Esteem device (Envoy Medical Corporation - Minnesota, United States), have their own peculiarities on candidacy and surgical procedure. Conclusion Implantable hearing aids, which are currently in the early stages of development, will unquestionably be the major drivers of advancement in otologic practice in the 21st century, improving the quality of life of an increasingly aged population, which will consequently require increased levels of hearing support.

Introduction: Evaluation of cortical auditory evoked potentials in children with cochlear implants has been proven to be an effective method for assessing cortical maturation after electrical stimulation., Objective: To analyze the changes in latency values of cortical auditory evoked potentials before and three months after cochlear implant use., Material and Methods: This was a case-control study with a group of five children using cochlear implant awaiting activation of the electrodes, and a control group composed of five normal-hearing children. Auditory electrophysiological assessment was performed by the testing of the cortical auditory evoked potentials at two different periods: prior to cochlear implant activation and after three months of cochlear implant use., Results: A significant decrease in the latency time of the P1 component was observed after three months of stimulation via cochlear implant, whose values were higher than those from the control group. The younger the child was at electrode activation, the greater the reduction in latency of the P1 component., Conclusion: Changes in the characteristics of cortical auditory evoked potentials can be observed in children who receive cochlear implants; these changes are related to the age of intervention, suggesting a rapid maturation of the auditory pathways after electrical stimulation.

Conclusion: The technique proposed is simple, reliable, and provides sufficient exposure of the basal portion of the cochlea while avoiding disabling complications. It enables visualization of the cochlear basal turn and the osseous spiral lamina, facilitating the insertion of the cochlear implant array through the scala tympani., Objectives: To describe a novel approach for exposing the cochlear basal turn for cochlear implantation through the middle cranial fossa., Methods: Fifty temporal bones were dissected and a cochleostomy was performed via a middle fossa approach on the most superficial part of the cochlear basal turn, using the superior petrosal sinus, the skeletonized petrous apex, the lateral surface of the meatal plane trailed on the petrous apex from its most proximal portion, and the great superficial petrosal nerve as landmarks. The distance between the landmarks and the distance between the cochleostomy and the round window were measured., Results: In all temporal bones, only the top portion of the cochlear basal turn was uncovered. The cochleostomy allowed both the scala tympani and the vestibule to be exposed. A computed tomography scan of the temporal bones was performed to document the electrode insertion from the cochlear basal turn until its apex. The mean ± SD minor and major distances between the cochleostomy and the meatal plane were estimated to be 2.48 ± 0.88 mm and 3.11 ± 0.86 mm, respectively. The mean distance from the cochleostomy to the round window was 8.38 ± 1.96 mm, and that to the superior petrosal sinus was 9.19 ± 1.59 mm. The mean minor and major distances between the cochleostomy and the long axis of the meatal plane from its most proximal portion were estimated to be 6.63 ± 1.38 mm and 8.29 ± 1.43 mm, respectively.

Objective: To identify the technological contributions of the newer version of speech processor to the first generation of multichannel cochlear implant and the satisfaction of users of the new technology. Among the new features available, we focused on the effect of the frequency allocation table, the T-SPL and C-SPL, and the preprocessing gain adjustments (adaptive dynamic range optimization)., Study Design: Prospective exploratory study., Setting: Cochlear implant center at hospital., Patients: Cochlear implant users of the Spectra processor with speech recognition in closed set. Seventeen patients were selected between the ages of 15 and 82 and deployed for more than 8 years., Interventions: The technology update of the speech processor for the Nucleus 22., Main Outcome Measures: To determine Freedom's contribution, thresholds and speech perception tests were performed with the last map used with the Spectra and the maps created for Freedom. To identify the effect of the frequency allocation table, both upgraded and converted maps were programmed. One map was programmed with 25 dB T-SPL and 65 dB C-SPL and the other map with adaptive dynamic range optimization. To assess satisfaction, SADL and APHAB were used., Results: All speech perception tests and all sound field thresholds were statistically better with the new speech processor; 64.7% of patients preferred maintaining the same frequency table that was suggested for the older processor. The sound field threshold was statistically significant at 500, 1,000, 1,500, and 2,000 Hz with 25 dB T-SPL/65 dB C-SPL. Regarding patient's satisfaction, there was a statistically significant improvement, only in the subscale of speech in noise abilities and phone use., Conclusion: The new technology improved the performance of patients with the first generation of multichannel cochlear implant.

Introduction:  The most relevant clinical symptom in Waardenburg syndrome is profound bilateral sensorioneural hearing loss., Aim:  To characterize and describe hearing outcomes after cochlear implantation in patients with Waardenburg syndrome to improve preoperative expectations., Method:  This was an observational and retrospective study of a series of cases. Children who were diagnosed with Waardenburg syndrome and who received a multichannel cochlear implant between March 1999 and July 2012 were included in the study. Intraoperative neural response telemetry, hearing evaluation, speech perception, and speech production data before and after surgery were assessed., Results:  During this period, 806 patients received a cochlear implant and 10 of these (1.2%) were diagnosed with Waardenburg syndrome. Eight of the children received a Nucleus 24(®) implant and 1 child and 1 adult received a DigiSonic SP implant. The mean age at implantation was 44 months among the children. The average duration of use of a cochlear implant at the time of the study was 43 months. Intraoperative neural responses were present in all cases. Patients who could use the speech processor effectively had a pure tone average of 31 dB in free-field conditions. In addition, the MUSS and MAIS questionnaires revealed improvements in speech perception and production. Four patients did not have a good outcome, which might have been associated with ineffective use of the speech processor., Conclusion:  Despite the heterogeneity of the group, patients with Waardenburg syndrome who received cochlear implants were found to have hearing thresholds that allowed access to speech sounds. However, patients who received early intervention and rehabilitation showed better evolution of auditory perception.

Introduction:  Adenomas with neuroendocrine differentiation are defined as neuroendocrine neoplasms, and they are rarely found in the head and neck., Objective:  To describe two cases of a middle ear adenoma with neuroendocrine differentiation, with a literature review., Case Report:  Patient 1 was a 41-year-old woman who presented with a 3-year history of left aural fullness associated with ipsilateral "hammer beating" tinnitus. Patient 2 was a 41-year-old male who presented with unilateral conductive hearing loss., Conclusion:  Adenoma with neuroendocrine differentiation of the middle ear is a rare entity, but it should be considered in patients with tinnitus, aural fullness, and a retrotympanic mass and remembered as a diferential diagnosis of tympanic paraganglioma.

Unlabelled: The classic approach for cochlear implant surgery includes mastoidectomy and posterior tympanotomy. The middle cranial fossa approach is a proven alternative, but it has been used only sporadically and inconsistently in cochlear implantation., Objective: To describe a new approach to expose the basal turn of the cochlea in cochlear implant surgery through the middle cranial fossa., Method: Fifty temporal bones were dissected in this anatomic study of the temporal bone. Cochleostomies were performed through the middle cranial fossa approach in the most superficial portion of the basal turn of the cochlea, using the meatal plane and the superior petrous sinus as landmarks. The lateral wall of the internal acoustic canal was dissected after the petrous apex had been drilled and stripped. The dissected wall of the inner acoustic canal was followed longitudinally to the cochleostomy., Results: Only the superficial portion of the basal turn of the cochlea was opened in the fifty temporal bones included in this study. The exposure of the basal turn of the cochlea allowed the visualization of the scala tympani and the scala vestibuli, which enabled the array to be easily inserted through the scala tympani., Conclusion: The proposed approach is simple to use and provides sufficient exposure of the basal turn of the cochlea.

Objective: The present investigation evaluated the speech perception performance of patients with ossified cochlea implanted with the 24M Double Array cochlear implant, using standard and duplicated maps in each of the arrays., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Sixteen subjects received a Double Array cochlear implant. Among these, 9 fulfilled the following inclusion criteria: bilateral severe-to-profound postlingual deafness; bilateral obliterated cochlea, as shown by a computed tomographic scan; and a minimum age of 14 years to ensure reliable responses in the behavioral tests with the 3 tested maps., Intervention: Rehabilitative., Main Outcome Measures: The speech perception performance with the 2 arrays was compared with that with a basal array duplicated map and an apical array duplicated map. Three maps were fitted: the default map with both arrays activated, a double channel map using only the electrodes of the basal array, and a double channel map programmed only with the electrodes of the apical array. The test battery was composed of a vowel test, a 4-choice word test, and sentence recognition in quiet., Results: Statistical significance was reached in comparison the all tests in all programming conditions. Speech recognition in the standard map with both electrode arrays activated showed the highest scores., Conclusion: Performance with the 2 split electrode arrays was superior to those with the single arrays, regardless of the duplication of channels., ((C) 2013 Otology & Neurotology, Inc.)

Introduction:  Cochlear implants may guarantee sound perception and the ability to detect speech at a close-to-normal hearing intensity; however, differences have been observed among implantees in terms of performance on discrimination tests and speech recognition., Objective:  To identify whether patients with post-meningitis deafness perform similarly to patients with hearing loss due to other causes., Method:  A retrospective clinical study involving post-lingual patients who had been using Nucleus-22 or Nucleus-24 cochlear implants for at least 1 year. These patients were matched with respect to age (± 2 years), time since the onset of deafness (± 1 year), and the duration of implant use with implant users who had hearing loss due to other causes. Speech perception was assessed using the Portuguese version of the Latin-American Protocol for the Evaluation of Cochlear Implants., Results: The sample consisted of 52 individuals (26 in each of the 2 groups). The post-meningitic group had a median of 18.5 active electrodes. The group with hearing loss due to other causes had a median of 21, but no significant statistical difference was observed (p = 0.07). The results of closed- and open-set speech recognition tests showed great variability in speech recognition between the studied groups. These differences were more pronounced for the most difficult listening tasks, such as the medial consonant task (in the vowel-consonant-vowel format)., Conclusion:  Cochlear implant recipients with hearing loss due to bacterial meningitis, who had been using the device for 1 year performed more poorly on closed- and open-set speech recognition tests than did implant recipients with hearing loss due to other causes.

Unlabelled: Neurofibromatosis 2 (NF2) is an autosomal dominant disease in which hearing loss is predominant. Auditory restoration is possible using cochlear implants (CI) or auditory brainstem implant (ABI)., Objective: To assess the auditory results of CI and ABI in NF2 patients and review the literature., Methods: Four NF2 patients were prospectively evaluated. They were submitted to tumor resection followed by ipsilateral CI or ABI depending on cochlear nerve preservation. Long term auditory results were described for CI (12 months) and ABI (48 months)., Results: All patients achieved auditory perception improvements in their hearing thresholds. The CI patient does not recognize vowels or sentences. The 3 ABI patients discriminate 70% of vowels and 86% in the 4-choice test. One of them does not recognize sentences. The other two recognize 100% of closed sentences and 10% and 20% of open sentences., Conclusion: The choice of implant type to restore hearing to NF2 patients will relay on anatomical and functional cochlear nerve preservation during tumor resection surgery. Although our experience was different, the literature shows that if this condition is achieved, CI will offer better auditory results. If not, ABI is recommended.

Objectives: To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites., Case Report: A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine., Conclusion: This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.

Introduction:  The majority of patients with hearing loss, including those with severe hearing loss, benefits from the use of hearing aids. The cochlear implant is believed to achieve better results in a child with hearing loss in cases where the severity of disability renders hearing aids incapable of providing adequate sound information, as they require sufficient cochlear reserve so that acoustic detention occurs., Objective:  To assess if cochlear implants provide more benefit than conventional hearing aids in prelingually deaf patients., Summary of the Findings:  The study was a systematic review of scientific papers selected by a search of the SciELO, Cochrane, MEDLINE, and LILACS-BIREME databases. Among the 2169 articles found, 12 studies proved relevant to the issue and presented an evidence strength rating of B. No publications rated evidence strength A. Seven of the studies analyzed were prospective cohorts and 5 were cross-sectional studies., Conclusion: Based on several studies, cochlear implants were demonstrated to be the best current alternative for bilateral severe or profound hearing loss, achieving better results in speech perception and development in prelingual children when compared to conventional hearing aids.

Unlabelled: Cochlear implants are the best treatment for congenital profound deafness. Pediatric candidates to implantation are seen as vulnerable citizens, and the decision of implanting cochlear devices is ultimately in the hands of their parents/guardians. The Brazilian Penal Code dictates that deaf people may enjoy diminished criminal capacity. Many are the bioethical controversies around cochlear implants, as representatives from the deaf community have seen in them a means of decimating their culture and intrinsic values., Objective: This paper aims to discuss, in bioethical terms, the validity of implanting cochlear hearing aids in children by analyzing their vulnerability and the social/cultural implications of the procedure itself, aside from looking into the medical/legal aspects connected to their criminal capacity., Materials and Methods: The topic was searched on databases Medline and Lilacs; ethical analysis was done based on principialist bioethics., Results: Cochlear implants are the best therapeutic option for people with profound deafness and are morally justified. The level of criminal capacity attributed to deaf people requires careful analysis of the subject's degree of understanding and determination when carrying out the acts for which he/she has been charged., Conclusion: Cochlear implants are morally valid. Implantations must be analyzed on an each case basis. ENT physicians bear the ethical responsibility for indicating cochlear implants and must properly inform the child's parents/guardians and get their written consent before performing the procedure.

Unlabelled: Cochlear implantation is a safe and reliable method for auditory restoration in patients with severe to profound hearing loss., Objective: To describe the surgical complications of cochlear implantation., Materials and Methods: Information from 591 consecutive multichannel cochlear implant surgeries were retrospectively analyzed. All patients were followed-up for at least one year. Forty-one patients were excluded because of missing data, follow-up loss or middle fossa approach., Results: Of 550 cochlear implantation analyzed, 341 were performed in children or adolescents, and 209 in adults. The mean hearing loss time was 6.3 ± 6.7 years for prelingual loss and 12.1 ± 11.6 years for postlingual. Mean follow-up was 3.9 ± 2.8 years. Major complications occurred in 8.9% and minor in 7.8%. Problems during electrode insertion (3.8%) were the most frequent major complication followed by flap dehiscence (1.4%). Temporary facial palsy (2.2%), canal-wall lesion (2.2%) and tympanic membrane lesion (1.8%) were the more frequent minor complications. No death occurred., Conclusion: There was a low rate of surgical complications, most of them been successfully managed. These results confirm that cochlear implant is a safe surgery and most surgical complications can be managed with conservative measures or minimal intervention.

Objective: To report the audiological outcomes of cochlear implantation in two patients with severe to profound sensorineural hearing loss secondary to superficial siderosis of the CNS and discuss some programming peculiarities that were found in these cases., Method: Retrospective review. Data concerning clinical presentation, diagnosis and audiological assessment pre- and post-implantation were collected of two patients with superficial siderosis of the CNS., Results: Both patients showed good hearing thresholds but variable speech perception outcomes. One patient did not achieve open-set speech recognition, but the other achieved 70% speech recognition in quiet. Electrical compound action potentials could not be elicited in either patient. Map parameters showed the need for increased charge. Electrode impedances showed high longitudinal variability., Conclusion: The implants were fairly beneficial in restoring hearing and improving communication abilities although many reprogramming sessions have been required. The hurdle in programming was the need of frequent adjustments due to the physiologic variations in electrical discharges and neural conduction, besides the changes in the impedances. Patients diagnosed with superficial siderosis may achieve limited results in speech perception scores due to both cochlear and retrocochlear reasons. Careful counseling about the results must be given to the patients and their families before the cochlear implantation indication.