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INTRODUCTION AND OBJECTIVES: Even though the incidence has decreased in recent years, Down syndrome (DS) remains the most common chromosomal disorder today. Despite being a condition with multisystemic involvement, it often tends to affect the head and neck area, making it a frequent reason for consultation with pediatric otolaryngologists or otologists. The purpose of this work is to be one of the first in Spain to characterize and describe the pathology and therapeutic approach typically provided to these patients, analyzing the evolution from a clinical and auditory perspective. MATERIAL AND METHODS: We aim to analyze a sample of 16 pediatric patients recruited over the past 24 years, diagnosed with Down syndrome, and experiencing a wide range of diseases affecting the ear and its auditory function. RESULTS: 62.50% of the patients were women, whose main reason for seeking specialist care was acute and serous otitis media, accounting for 31.25%. These patients have an indication for treatment for various entities within the otological sphere that usually do not differ from those of a healthy child. However, the evolution and response to treatments can take on a torpid character due to the anatomical characteristics of the ears of these patients. CONCLUSIONS: Although the frequency of children with DS in the pediatric otolaryngologist's clinic is decreasing, these patients have a predisposition to ear diseases with auditory repercussions, with variable evolution depending on the disease and the child's intrinsic characteristics.
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Background: Cholesteatoma is a lesion capable of destroying surrounding tissues, which may result in significant complications. Surgical resection is the only effective treatment; however, the presence of cholesteatoma recidivism is common. This study evaluated the effectiveness of the Attic Exposure-Antrum Exclusion (AE-AE) surgical technique in treating cholesteatomas and identifying factors associated with recidivism. Additionally, the study aimed to assess the utility of non-echo-planar diffusion MRI (non-EPI-DWI MRI) in detecting cholesteatoma recidivism in patients undergoing AE-AE surgery. Methods: The study involved 63 patients who underwent AE-AE surgery for primary acquired cholesteatoma and were followed up clinically and radiologically for at least five years. The radiological follow-up included a non-EPI-DWI MRI. Results: Results showed that the AE-AE technique successfully treated cholesteatomas, with a recidivism rate of 5.2%. The study also found that non-EPI-DWI MRI was a useful diagnostic tool for detecting cholesteatoma recidivism, although false positives could occur due to the technique's high sensitivity. As Preoperative Pure-tone average (PTA) increases, there is a higher probability of cholesteatoma recidivism in imaging tests (p = 0.003). Conclusions: Overall, the study highlights the importance of the AE-AE surgical technique and non-EPI-DWI MRI in managing cholesteatoma recidivism in patients, providing valuable insights into associated risk factors and how to manage recidivism. Non-EPI-DWI MRI can assist in patient selection for revision surgery, reducing unnecessary interventions and associated risks while improving treatment outcomes and patient care.
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INTRODUCTION: We present a series of six cases whose clinical presentations exhibited audiovestibular manifestations of a third mobile window mechanism, bearing a reasonable resemblance to Ménière's disease and otosclerosis. The occurrence of these cases in such a short period has prompted a review of the underlying causes of its development. Understanding the pathophysiology of third mobile window syndrome and considering these entities in the differential diagnosis of conditions presenting with vertigo and hearing loss with slight air-bone gaps is essential for comprehending this group of pathologies. MATERIALS AND METHODS: A descriptive retrospective cohort study of six cases diagnosed at a tertiary center. All of them went through auditive and vestibular examinations before and after a therapeutic strategy was performed. RESULTS: Out of 84 cases of dehiscences described in our center during the period from 2014 to 2024, 78 belonged to superior semicircular canal dehiscence, while 6 were other otic capsule dehiscences. Among these six patients with a mean age of 47.17 years (range: 18-73), all had some form of otic capsule dehiscence with auditory and/or vestibular repercussions, measured through hearing and vestibular tests, with abnormalities in the results in five out of six patients. Two of them were diagnosed with Ménière's disease (MD). Another two had cochleo-vestibular hydrops without meeting the diagnostic criteria for MD. In two cases, the otic capsule dehiscence diagnosis resulted from an intraoperative complication due to a gusher phenomenon, while in one case, it was an accidental radiological finding. All responded well to the proposed treatment, whether medical or surgical, if needed. CONCLUSIONS: Otic capsule dehiscences are relatively new and unfamiliar entities that should be considered when faced with cases clinically suggestive of Ménière's disease, with discrepancies in complementary tests or a poor response to treatment. While high-sensitivity and specificity audiovestibular tests exist, completing the study with imaging, especially petrous bone CT scans, is necessary to locate and characterize the otic capsule defect responsible for the clinical presentation.
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INTRODUCTION: Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems where the implant directly generates and applies vibration to the bone. MATERIALS AND METHODS: Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. OUTCOMES: Fourteen patients were included, seven in each group (n=14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43±21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29±19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49±14.16 dB and 23.72±6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients' quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant(p<0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. CONCLUSIONS: While auditory improvement is observed post-implantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®.
