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Objective: The objectives were to determine the interrater agreement of the Skull AP X-ray in measuring angular insertion depth (AID), to provide descriptive information about the insertion depths of different electrodes used in inner ear malformations (IEMs), to investigate the effect of cochlear size and electrode length on AID, and to guide clinicians in electrode selection in IEMs., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: A total of 198 IEMs (n = 169 patients) and 60 cochleae with normal anatomy (n = 60 patients) were selected from patients with severe mixed or sensorineural hearing loss who presented to our clinic and underwent cochlear implantation (CI) between January 2010 and December 2022., Interventions: Three neurotologists independently measured AID on Skull AP X-rays. Basal turn length of the cochlea was measured in axial and coronal oblique reformatted sections on HRCT images., Main Outcome Measures: Interrater reliability (ICC) of the AID measurements on Skull AP X-ray, determining the impact of cochlea size and electrode length on AID measurements., Results: The interrater reliability (ICC) test showed a high level of consistency in measuring AID in the Skull AP X-ray (R = 0.906, p < 0.001). In the control group, a negative correlation was observed between the AID and the basal turn length of the cochlea, while a positive correlation was found between electrode length and AID (R = 0.947, p < 0.001)., Conclusions: The Skull AP X-ray appears to be a dependable tool for measuring AID. In cases of IEMs, it is important to select an electrode of appropriate length, considering the dimensions of the cochlea., Competing Interests: Conflict of interest: The authors have no relevant financial or nonfinancial interests to disclose., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Otology & Neurotology, Inc.)

Otitic Hydrocephalus (OH) is one of the most significant life-threatening complications of otological infections. Given their low prevalence and non-specific ear symptoms, this complication requires a high index of suspicion for diagnosis. In this case report, we aim to provide an analysis of OH and describe common clinical signs and symptoms, treatment options, morbidity and mortality., Competing Interests: Conflict of interestThe authors declare that they have no conflict of interest., (© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Objective: To analyze and compare the early and late post-operative results of glass ionomer bone cement (GIBC) used in ossiculoplasty., Methods: The pre-operative, early post-operative (3 months) and late post-operative (> 2 years) audiometric findings, namely the pure-tone average (PTA), bone conduction threshold (BCT) and air-bone gap (ABG) of 40 patients who underwent GIBC ossiculoplasty for different etiologies were analyzed. Early and late results were compared. Also, the patients were grouped in terms of prognostic factors and applied ossiculoplasty techniques, and the results were compared between the groups., Results: There were statistically significant improvements in the pure-tone average and air-bone gap of the patients in the early post-operative period (PTA from 59.60 ± 15.95 to 40.37 ± 17.83 and ABG from 37.12 ± 11.18 to 19.78 ± 10.41, p < 0.001 for both). There were no statistically significant changes in any of the audiometric parameters in the late post-operative period (PTA from 40.37 ± 17.83 to 39.79 ± 17.91, ABG from 19.78 ± 10.41 to 19.32 ± 9.60, BCT from 17.99 ± 12.71 to 18.31 ± 13.99, p > 0.05 for all). Presence of tympanosclerosis was found to be the only prognostic factor to affect the outcome., Conclusion: GIBC is a safe and reliable material for ossiculoplasty, which maintains its ability to conduct sound in the long-term follow-up., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: After auditory brainstem implant (ABI) surgery, stimulation of certain cranial nerves may result in a non-auditory response, and the electrodes that stimulate these nerves may be deactivated. The goals of this study are to compare the number of active electrodes in the initial activation and the last fitting, to investigate non-auditory response types and their frequency as a result of non-auditory stimulation, to compare the placements of deactivated electrodes as a result of non-auditory stimulation in the initial activation and the last fitting., Methods: The computer software system was used to perform a retrospective analysis of the fitting data of 69 ABI users who underwent auditory brainstem implant surgery between January 1997 and January 2019. The non-auditory response types, deactive electrodes, and the positioning of the deactive electrodes horizontally and vertically were recorded in these users during the initial activation and the last fitting., Results: There was no statistically significant difference between the number of active electrodes in the initial activation and the last fitting. The proportion of the users with deactive electrodes in the initial activation and the last fitting was not statistically significant different. In the horizontal and vertical placement classification, the placement of the deactive electrodes was not statistically different between initial activation and last fitting. The most common type of non-auditory response was facial nerve stimulation at the initial activation and no auditory perception at the last fitting. According to the difference between the number of active and deactive electrodes in the initial activation and the last fitting, as well as the auditory and non-auditory responses, it was found that the ABI users were statistically different between the initial activation and the last fitting., Conclusion: The results of this study show that not only auditory but also non-auditory responses occur in most ABI users. In addition, to the best of our knowledge, this study is the first to examine the frequencies of non-auditory response types, and the placement of the electrodes that cause these responses according to horizontal and vertical classifications., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users., Study Design: Retrospective cohort., Setting: Tertiary referral center., Patients: Five pediatric ABI users who had revision surgery for device malfunctions., Interventions: Revision surgery for ABI malfunctions., Main Outcome Measures: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared., Results: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed., Conclusion: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development., Competing Interests: The author discloses no funding and conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Background: This study was planned (1) to evaluate long-term outcome after cochlear implantation in patients with post-meningitic deafness and (2) to compare the outcome measures with patients implanted for deafness due to other causes., Methods: Records of 54 patients deafened as a sequel of bacterial meningitis and implanted at the largest university-based cochlear implant program in Turkey were retrospectively reviewed. Fifty-four age- and sex-matched patients with a similar interval of implant use were selected for controls. Surgical and long-term audiological outcome (in terms of categories of auditory performance-II scores) was assessed and compared., Results: Twenty-seven (52%) patients had some degree of labyrinthitis ossificans and 19 of them had full electrode insertion via basal turn cochleostomy. Patients with and without labyrinthitis ossificans in the post-meningitic group had no difference in final categories of auditory performance-II score (P=.559). Median categories of auditory performance-II scores were 6 for post-meningitic group and 7 for controls, with a significant statistical difference (P < .001). Partial or full insertions did not differ in outcome (P=.938). Mean time to implantation was not correlated with the final categories of auditory performance-II score for the post-meningitic group (P=.695)., Conclusion: Cochlear implant recipients deafened due to meningitis have a worse long-term hearing and speech performance as measured by categories of auditory performance-II than patients implanted for congenital deafness. The presence of labyrinthitis ossificans or the limited extent of electrode insertion produced overall results that were comparable with other cases.

Objective: Some inner ear malformations may cause recurrent meningitis, which may be fatal. The etiology is usually a stapes footplate fistula which enables microorganisms to pass into the inner ear containing cerebrospinal fluid (CSF), causing repeated attacks of meningitis. Radiological signs of the fistula are not obvious and are not reported in detail in the literature. The aim of the study is to investigate the radiological features of stapes footplate fistula in inner ear malformations., Methods: Radiological findings were analyzed for seventeen patients with inner ear malformations (IEMs) operated on because of recurrent meningitis. Using this information, images of 1,010 patients with IEMs were retrospectively reviewed to investigate the radiological findings of stapes footplate fistula and their relationship to IEMs. They were classified according to the Sennaroglu classification system, and according to different stages of stapes footplate fistula., Results: In the case of a stapes footplate cyst, computerized tomography shows an opacity at the oval window. On magnetic resonance imaging, a fluid filled cystic structure continuous with and having similar signal characteristics to the CSF in the inner ear is a pathognomonic finding of a stapes footplate cyst. It is most commonly found in common cavity anomaly (18.2%); the second most frequent finding is incomplete partition type I (15%). And it can even be seen in cases of cochlear aplasia where only the vestibule is present., Conclusion: If the history reveals recurrent meningitis, particular attention should be given to the oval window area, where an opacity, cyst or a leaking lesion should be looked for on the imaging. It is the responsibility of the otolaryngologist to notice these findings, and to operate on the patient to prevent further attacks of meningitis., (©Copyright 2021 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.)

Objectives: To introduce the concept of stapedotomy as a new treatment alternative in cochlear hypoplasia (CH) and propose a new guideline for its management., Methods: Forty-two primary cases out of 355 presented with congenital stapes fixation between January 2003 and September 2015 were included in the study. Computed tomography scans of all cases with congenital stapes fixation were reviewed, and cases with inner ear anomalies were taken into account. Eleven cases had various inner ear anomalies, and 9 cases had various types of CH. In the present paper, only the CH cases with stapes fixation, all of whom underwent stapedotomy, are reviewed regarding preoperative audiological and radiological characteristics as well as surgical findings and postoperative audiological results., Results: The patients were aged between 4 and 22. There were 2 males (3 ears) and 4 females (6 ears). Three cases had bilateral stapedotomy. The remaining 3 cases had unilateral surgery. The average preoperative air-bone gap (ABG) was 50.3 dB. Postoperative hearing: preoperative ABG was 50.3 dB. Postoperative ABG was calculated as 20.1 dB hearing., Conclusion: Hearing loss (HL) in hypoplastic cochlea demonstrates the full spectrum of HL types. CH is a unique inner ear anomaly that can be treated with all of the available rehabilitation modalities. As a result of current findings, a new treatment algorithm for CH is proposed.

We discuss a case of lower lip carcinoma which presented with atypical symptoms; facial paralysis, conductive type hearing loss, and ophthalmoplegia. Due to an earlier resection, no mass was evident on the primary examination. Diagnostic imaging revealed a mass originating from the lower lip, the perineural spread of the tumor along the left inferior alveolar nerve to the left infratemporal fossa and the left foramen ovale. Through a retrograde course from the foramen ovale, the tumor extended the ipsilateral cavernous sinus, Meckel's cave, and cisternal portion of the CN V. This atypical spread pattern of the tumor caused symptoms that may be attributed to a diagnosis related to the ear. The biopsy confirmed squamous cell carcinoma, and the patient was referred for chemotherapy and radiotherapy.

Objectives/hypothesis: The objective of this study was to classify anomalous facial nerve (FN) routes and to determine their association with inner ear malformations (IEMs)., Study Design: Retrospective cross sectional study., Methods: The computed tomography images of 519 patients (796 ears) with IEMs were retrospectively evaluated, and the abnormal routes of the FN were classified as: Meatal segment: type 1, normal internal auditory canal (IAC); type 2, narrow IAC; type 3, facial canal (FC) only; type 4: separate FC/duplicated IAC. Labyrinthine segment (LS): type 1, normal; type 2a/b/c, mild/moderate/severe anterior displacement; type 3, superior displacement; type 4: straight LS. Tympanic segment (TS): type 1, normal; type 2, superiorly displaced TS; type 3, TS at the oval window; type 4: TS inferior to the oval window; type 5: unclassified. Mastoid segment: type 1, normal facial recess (FR)/normal mastoid segment; type 2: narrow FR; type 3, unclassified., Results: In meatal segment classification, a narrow IAC was common in ears with cochlear hypoplasia (CH) (76.1%), and only FC was common in ears with severe IEMs (62.7%) such as Michel deformity, common cavity, and cochlear aplasia. Incomplete partition-III has its unique superiorly displaced LS (100%). CH-IV also has its unique mild anterosuperior displacement. Ears with a superiorly displaced TS usually (93.1%) had aplastic or hypoplastic semicircular canals. The FR is likely to be narrow in CH and severe IEMs., Conclusions: The FN route is affected in IEMs, which must be kept in mind when operating on ears with IEMs. Especially in CH cases, all segments of the FN can be abnormal., Level of Evidence: 4 Laryngoscope, 130:E696-E703, 2020., (© 2020 The American Laryngological, Rhinological and Otological Society, Inc.)

Objectives: To determine the temporal order, resolution, and perception of prosody skills in Single-Sided Deafness (SSD) compared to an age- and sex-matched normal hearing group's same side ear and both ears., Methods: This was a Case-Control study including 30 subjects with SSD, and age- and sex-matched 30 subjects with bilateral normal hearing (total of 60 subjects- mean age: 38.7 ± 11.6 years). The Montreal Cognitive Assessment (MoCA), Frequency Pattern Test (FPT), Duration Pattern Test (DPT), Random Gap Detection Test (RGDT), Evaluation of Motor Response Time and Emotional Prosody Assessment were performed on the clinically normal ear in the SSD group, the same side ear in the normal hearing group, and both ears of the normal hearing group (the SSD, MNH, and BNH groups, respectively)., Results: Individuals with SSD had worse results in DPT (p < .001), gap detection at 0.5 kHz (p < .001), gap detection at 4 kHz (p < .001), and composite score (p < .001) than the BNH group. For reaction time measurements, the SSD group had slower performance scores than the BNH group for DPT (p < .001) and FPT (p < .001)., Conclusions: Poor temporal processing ability and reduced reaction times may help explain the difficulties in those with SSD in performing daily living activities such as speech understanding in noise and requires more processing efforts. However, there were no significant differences between the groups in frequency pattern performance and emotional prosody skills, supporting the claim that fundamental frequency is one of the most important measures of perception in emotional prosody. We demonstrated that unilateral hearing is adequate to analyze frequency patterns to aid in prosody perception. Analysis of reaction times in temporal processing and emotional prosody could provide different perspectives of auditory processing. Slower reaction time of SSD should be considered for habilitation purposes., Competing Interests: Declaration of Competing Interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article. This study wasn't funded., (Copyright © 2020. Published by Elsevier B.V.)

The anatomical cause of congenital sensorineural hearing loss can be atresia of the bony cochlear nerve canal (BCNC). It has been reported that the cochlear nerve (CN) can be either hypoplastic or aplastic when the BCNC width is <1.5 mm radioanatomically. It is difficult to estimate the auditory-verbal abilities after cochlear implantation (CI) in patients with a hypoplastic CN. In such cases, it is also challenging to decide on the best treatment modality: CI or auditory brainstem implantation. In this case report, we present a 4-year-old male patient with BCNC atresia and the successful use of a cochlear implant; we also discussed the importance of audiological evaluation. A detailed radiological evaluation must be performed in every case following electrophysiological studies prior to CI. To accurately diagnose the pathology and select the surgical side, both computed tomography and magnetic resonance imaging scans should be used as complementary imaging methods in all CI candidates.

Objective: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear., Intervention(s): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear., Main Outcome Measures: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed., Results: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome., Conclusions: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.

Objective: The deficits in the cochlea which is at the one end of the ear sound transfer system, may effect middle ear functions. Wideband typanometry (WBT) is frequently used to evaluate these transfer functions which play a crucial role in setting the impedance matching between the external ear and the cochlea. To this end, the aim of this study was to investigate the ear transfer functions in inner ear malformations via WBT, and to question whether these functions change depending on the types of inner ear malformation., Methods: This prospective case-control study was conducted in a university hospital. One hundered-fifty-seven ears (aged 3-37 years) under the groups of cochlear hypoplasia, incomplete partition I, incomplete partition II, cochlear aplasia and complete labyrinthine aplasia were evaluated. In the control group, 30 ears with normal hearing were enrolled and WBT was carried out. Tympanometric peak pressure, equivalent middle ear volume, static admittance, tympanogram width, resonance frequency, average wideband tympanometry and absorbance measurements were analyzed., Results: The inner ear malformation groups demonstrated statistically significant differences than the control group and from each other in terms of traditional tympanometric parameters and WBT test parameters (p<0.05). The most remarkable difference was between the group of complete labyrinthine aplasia and the control group, most probably because of complete labyrinthine aplasia's structural effects. However, on some parameters, incomplete partition II and the control group showed similarities. In absorbance measurements, there was significant difference between all patient groups and the control group, especially at high frequencies (p<0.05). The largest difference was between the control group and the group of complete labyrinthine aplasia which has revealed the lowest absorbance values (p<0.05). In averaged-wideband tympanogram analysis, all patient groups obtained a lower amplitude peak than the control group; complete labyrinthine aplasia group had the flattest peaked amplitude, while the incomplete partition II group had a near-normal curve., Conclusion: The results of the study revealed the distinctive effects of inner ear malformations in middle ear transfer functions. It is concluded that the absence of inner ear structures causes negative effects on energy absorbance and the other transfer functions of the middle ear. WBT may provide additional information on diagnosis of patients with inner ear malformations., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: The aim of this report is to share our experience and treatment outcomes with far-advanced otosclerosis (FAO) patients., Methods: Patients that underwent surgery from 2003 through 2014 at a tertiary referral center were retrospectively reviewed. Nineteen FAO patients were included in the study. Audiological results and the ability to communicate face to face and over telephone were considered as the main outcome measures., Results: Six FAO patients benefited well from stapedotomy with an average of 5.9-decibel (dB) air-bone gap and 86% median speech discrimination. Cochlear implantation (CI) was performed in 13 patients; two had disease progression after stapedotomy, five had failed stapes surgeries elsewhere, and six preferred CI as primary treatment. Median speech discrimination score of CI patients was 78.4%. Overall, all patients had satisfactory face-to-face communication and 90% could use telephone., Conclusion: Bilateral stapedotomy and wearing hearing aid is an effective and cost-effective solution for restoring natural binaural hearing and requires no specific training. Should stapedotomy fail, cochlear implantation is always a successful back-up option., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare., (© Copyright 2020 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.)

Objective: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility., Study Design: Retrospective single cohort design., Setting: Tertiary referral center., Patients: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ± 6.5) months in the early group, 45.53 (ranged ± 7.9) months in the late group., Intervention(s): Retrosigmoid craniotomy and ABI placement., Main Outcome Measure(s): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room., Results: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps., Conclusions: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

Objectives: The aim of the present study was to investigate the effect of the national pneumococcal vaccination program on postmeningitis sensorineural hearing loss (SNHL)., Materials and Methods: Overall, 2751 patients (2615 cochlear implantation and 136 auditory brainstem implantation) who underwent cochlear implantation (CI) and auditory brainstem implantation (ABI) at a tertiary referral hospital otolaryngology clinic were retrospectively analyzed. One hundred sixteen patients with a history of meningitis were included in the study. Patients were evaluated for their age at the time of surgery, gender, computerized tomography (CT) and magnetic resonance imaging (MRI) findings, implant type, side, and incidence before and after the vaccination program., Results: When patients with cochlear implants or ABI were examined, the incidence of meningitis-induced hearing loss was 6.2% in the pre-vaccination period and 0.6% in the post-vaccination period. There is a significant difference between them when compared by chi-square test (p<0.001)., Conclusion: The most important finding of the present study is the dramatic decrease in the number of CI and ABI surgeries performed in patients with SNHL due to meningitis. This shows the effectivity of pneumococcal vaccination in this special group of patients. If total ossification is detected on CT of patients with postmeningitis, ABI should be preferred to CI.

We present a rare case of traumatic facial and vestibulocochlear nerve injury in the internal acoustic canal in the absence of a temporal bone fracture. A 2.5-year-old female presented with sudden-onset left-sided facial paralysis and ipsilateral total hearing loss after being hit by a falling television. High-resolution computed tomography revealed an occipital fracture line that spared the temporal bone and otic capsule. Diagnostic auditory brainstem response testing showed that wave V at 90-db normal hearing level was absent in the left ear. Needle electromyography revealed severe axonal injury. Facial paralysis regressed to House-Brackmann grade IV 9 months after the trauma, and no surgical intervention was scheduled. Traumatic facial and vestibulocochlear nerve injury can occur in the absence of a temporal bone fracture. Thus, careful evaluation of the internal acoustic canal is mandatory if concurrent 7th and 8th cranial nerve paralyses exist with no visible fracture line.

Objective: Modiolus and modiolar base abnormalities in patients with incomplete partition-II anomaly (IP-II) increase the risk of intraoperative cerebrospinal fluid (CSF) leakage. This study aimed to classify modiolar abnormalities and define objective radiological measures for preoperatively evaluating intraoperative CSF leakage risk., Study Design: Retrospective case series., Setting: Tertiary referral center., Patients: The study included 73 patients with IP-II that underwent cochlear implant surgery between 2002 and 2017., Intervention: Analysis of preoperative temporal bone computed tomography (CT) scans and surgical records., Main Outcome Measures: Preoperative CT modiolar anomalies and intraoperative CSF leakage status., Results: Among the 73 patients (41 men and 32 women), mean age at cochlear implant surgery was 11.4 (range, 0-42) years. Preoperative CT-based modiolar base anomaly classification was as follows: grade 1 (complete modiolus, n = 1), grade 2 (thin plate of bone in the modiolar base + partial modiolus, n = 14); grade 3 (thin plate of bone in the modiolar base, n = 53); grade 4 (total modiolar base defect, n = 5). The gusher rate was 8%. All patients with grade 4 anomaly had an intraoperative gusher. Patients with grade 3 anomaly accounted for 86% of oozing cases. Oozing, pulsation, and no CSF leakage rates were similar in those with grade 2 anomaly., Conclusions: The modiolus and modiolar base must be carefully evaluated in patients with IP-II. Gushers primarily occur in IP-II patients with grade 4 anomaly. A thin plate of bone in the modiolar base most commonly prevents gushers.

Objective: Presenting the clinical features and treatment options for incomplete partition type-III., Methods: Nine primary and 1 revision incomplete partition type-III cochlear implant cases treated between 2004 and 2015 in Hacettepe University Department of Otolaryngology were included in the study. Treatment options and particularly cochlear implantation tecnique were described., Results: Nine primary and 1 revison cases were all succesfully implanted. Eight cases were standart cases with no secondary intervention. Case #9 has to be revised intraoperatively and case #10 were operated four times in another center and revised in our department., Conclusion: Incomplete partition type-III is one of the rarest inner ear anomaly and the rarest among incomplete partition group. Treatment options may differ depending on the hearing loss level of the patient. Stapes surgery should be avoided because it will lead to gusher and further hearing loss. Preoperative imaging is mandatory in order to avoid unnecessary stapes surgery. Incochlear implantation surgery a gusher and misplacement into the IAC may complicate the surgery. Gusher should be controlled intraoperatively and the position of the electrode should be controlled via intraoperative imaging., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.

Objective: To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings., Materials and Methods: The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 patients with Type III;IP-III (8 ears). Remaining 11 ears were diagnosed with other inner ear malformations. Air and bone conduction thresholds were determined with pure tone audiometry, and the air bone gap was recorded in all patients with IPs. Magnetic resonance imaging studies and computerized tomography scans of the temporal bones were analyzed using the PACS system of our university., Results: It was found that all the ears with IP-I were diagnosed with severe to profound hearing loss. The degree of the hearing loss varied from mild to severe/profound in patients with IP-II. Severe to profound mixed hearing loss (MHL) was determined in all ears with IP-III. The air bone gap was larger in the lower frequencies in the IP-II cases diagnosed with MHL. There was not a significant difference between the air bone gap and the size of the vestibular aqueduct in ears with IP-II (p>0.05)., Conclusion: Each type of IP has different audiological findings. Depending on the type and degree of the hearing loss, it is possible to choose the appropriate audiological intervention. Patients with IP should be evaluated according to the type of malformation.

Purpose: We aimed to evaluate the diagnostic accuracy of turbo spin-echo diffusion-weighted imaging (TSE-DWI) at 3 T, for cholesteatoma (CS) diagnosis, using qualitative and quantitative methods with numerical assessment of signal intensity (SI), signal intensity ratios (SIR), and apparent diffusion coefficient (ADC) values., Methods: In this retrospective study, two blinded observers independently evaluated the preoperative TSE-DWI images of 57 patients who were imaged with a presumed diagnosis of CS. Qualitative assessment with respect to the SI of the adjacent cortex and quantitative measurements of SI, SIR, and ADC values were performed., Results: Surgery with histopathologic examination revealed 30 CS patients and 27 patients with non-cholesteatoma (NCS) lesions including chronic inflammation and cholesterol granuloma. On TSE-DWI, 96.7% of the CS lesions and none of the NCS lesions appeared hyperintense compared with the cortex. The mean SI and SIR indices of the CS group were significantly higher and the mean ADC values significantly lower compared with those of the NCS group (P < 0.001). Using specific cutoff values for SI (92.5) and SIR (0.9), CS could be diagnosed with 100% sensitivity and specificity. The use of quantitative imaging further increased the sensitivity of the TSE-DWI technique., Conclusion: The quantitative indices of SI, SIR, and ADC of TSE-DWI appear to be highly accurate parameters that can be used to confirm the diagnosis of CS.

Objective: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations., Study Design: Retrospective chart review., Setting: Tertiary referral otolaryngology clinic., Subjects and Methods: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined., Results: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI., Conclusion: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

Background: Neurobrucellosis is a disease consisting of a wide spectrum of complications such as peripheral neuropathy, cranial nerve involvement, ataxia, meningeal irritation, paraplegia, seizures, coma, and even death. The vestibulocochlear nerve seems to be the most commonly affected cranial nerve (10%). We present a patient with neurobrucellosis whose auditory perception and speech intelligibility skill performances improved after cochlear implantation., Case Report: A 35 year-old woman was admitted to another hospital 2 years ago with the symptoms of headache, nausea, and altered consciousness, who was finally diagnosed with neurobrucellosis. She developed bilateral profound sensorineural hearing loss during the following 6 months. There was no benefit of using hearing aids. After successful treatment of her illness, she was found to be suitable for cochlear implantation. After the operation, her auditory perception skills improved significantly with a Categories of Auditory Performance (CAP) score of 5. According to clinical observations and her family members' statements, her Speech Intelligibility Rating (SIR) score was 3. Her speech intelligibility skills are still improving., Conclusion: Our case report represents the second case of hearing rehabilitation with cochlear implantation after neurobrucellosis. Cochlear implantation is a cost-effective and time-proven successful intervention in post-lingual adult patients with sensorineural hearing loss. Early timing of the surgery after appropriate treatment of meningitis helps the patient to achieve better postoperative results.

Background: Sudden sensorineural hearing loss (SSNHL) is still a complex and challenging process which requires clinical evidence regarding its etiology, treatment and prognostic factors. Therefore, determination of prognostic factors might aid in the selection of proper treatment modality., Aims: The aim of this study is to analyze whether there is correlation between SSNHL outcomes and (1) systemic steroid therapy, (2) time gap between onset of symptoms and initiation of therapy and (3) audiological pattern of hearing loss., Study Design: Retrospective chart review., Methods: Patients diagnosed at our clinic with SSNHL between May 2005 and December 2011 were reviewed. A detailed history of demographic features, side of hearing loss, previous SSNHL and/or ear surgery, recent upper respiratory tract infection, season of admission, duration of symptoms before admission and the presence of co-morbid diseases was obtained. Radiological and audiological evaluations were recorded and treatment protocol was assessed to determine whether systemic steroids were administered or not. Treatment started ≤5 days was regarded as "early" and >5 days as "delayed". Initial audiological configurations were grouped as "upward sloping", "downward sloping", "flat" and "profound" hearing loss. Significant recovery was defined as thresholds improved to the same level with the unaffected ear or improved ≥30 dB on average. Slight recovery was hearing improvement between 10-30dB on average. Hearing recovery less than 10 dB was accepted as unchanged., Results: Among the 181 patients who met the inclusion criteria, systemic steroid was administered to 122 patients (67.4%), whereas 59 (32.6%) patients did not have steroids. It was found that steroid administration did not have any statistically significant effect in either recovered or unchanged hearing groups. Early treatment was achieved in 105 patients (58%) and 76 patients (42%) had delayed treatment. Recovery rates were no different in these two groups; however, when unchanged hearing rates were compared, it was statistically significantly lower in the early treatment group (p<0.05). When hearing outcomes were compared according to initial audiological pattern, significant recovery and unchanged hearing rates did not differ between groups; however, slight recovery rate was highest in the "flat" type audiological configuration (p<0.05)., Conclusion: According to this patient series, oral steroid therapy does not have any influence on the outcomes of SSNHL. However, mid-frequency hearing loss of flat type and initiation of treatment earlier than 5 days from the onset of symptoms, seem to have positive prognostic effects. Further randomized controlled subject groups might contribute to determine prognostic factors of SSNHL.

Objective: The aim of this study is to describe the rehabilitative outcomes of pediatric auditory brainstem implant (ABI) users in the Department of Otolaryngology in the Hacettepe University. It was a retrospective study, and all patients' files were reviewed., Materials and Methods: The data was collected from 41 children who were fitted with ABI between 2005 and 2013. Inclusion criteria for children in our study are profound, congenital bilateral sensory-neural hearing loss with anomalies (such as cochlear, labyrinthine, and cochlear nerve aplasia) and more than one year of auditory experience with ABI. Post-meningitis patients and neurofibromatosis type 2 (NF2) patients were excluded. Auditory perception was evaluated using the Meaningful Auditory Integration Scale (MAIS), Functioning after Pediatric Cochlear Implantation (FAPCI) instrument, Categories of Auditory Performance (CAP), and Children's Auditory Perception Skills Test in Turkish (CIAT). Speech intelligibility was categorized with speech intelligibility rating (SIR), and language development was assessed using the Test of Early Language Development-Third Edition (TELD-3) and Manchester Spoken Language Development Scale (MSLD)., Results: All patients gained basic audiological functions and were able to recognize and discriminate sounds by the third month of ABI surgery. According to the duration of ABI use and learning skills, patients revealed development from word identification to sentence recognition level in a wide spectrum., Conclusion: Preliminary results indicate that all children have gained basic auditory perception skills. On the other hand, language and speech development data were varying among children. Additional handicaps seemed to slow down progression. Secondary improvement was seen at psychosocial areas with respect to behavioral and social adjustment as well as eagerness to start communication.