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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.)

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.)

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.

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.

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.