No abstract

Inflammation of the paranasal sinuses is a common condition that affects the upper respiratory tract. The pathomechanism and course of sinusitis are multifaceted, depending on the etiological factors, duration of the disease, anatomical abnormalities, and additional conditions exacerbating the inflammation of the nasal mucosa and paranasal sinuses. The gold standard of diagnostic imaging is computed tomography (CT), performed in particular cases. An auxiliary examination is a magnetic resonance imaging (MRI) for soft tissue imaging when there is a suspicion of a neoplastic process. The treatment of patients with rhinosinusitis is very complex and long-lasting, associated with the use of nasal or systemic corticosteroids, irrigation with physiological saline, as well as antibiotic therapy, antihistamines or herbal supplements. The treatment is selected individually for the patient's condition or the sinus phenotype, and in exceptional cases, surgical intervention is undertaken. Work is continuing on genetic, molecular and immunological research to search for new and effective methods of treatment of rhinosinusitis.

Objective: The aim of the study was to analyze the long-term outcomes after cochlear implantation in deaf children with Down syndrome (DS) regarding age at the first implantation and refer the results to preoperative radiological findings as well as postoperative auditory and speech performance. Additionally, the influence of the age at implantation and duration of CI use on postoperative hearing and language skills were closely analyzed in children with DS. Study Design: Retrospective analysis. Setting: Referral center (Cochlear Implant Center). Materials and Methods: Nine children with Down syndrome were compared with 220 pediatric patients without additional mental disorders or genetic mutations. Patients were divided into four categories depending on the age of the first implantation: CAT1 (0–3 yr), CAT2 (4–5 yr), CAT3 (6–7 yr), and CAT4 (8–17 yr). The auditory performance was assessed with the meaningful auditory integration scales (MAIS) and categories of auditory performance (CAP) scales. The speech and language development were further evaluated with meaningful use of speech scale (MUSS) and speech intelligibility rating (SIR). The postoperative speech skills were analyzed and compared between the study group and the reference group by using nonparametric statistical tests. Anatomic abnormalities of the inner ear were examined using magnetic resonance imaging (MRI) and high-resolution computed tomography of the temporal bones (HRCT). Results: The mean follow-up time was 14.9 years (range, 13.1–18.3 yr). Patients with DS received a multichannel implant at a mean age of 75.3 months (SD 27.9; ranging from 21 to 127 mo) and 220 non-syndromic children from reference group at a mean age of 51.4 months (SD 34.2; ranging from 9 to 167 mo). The intraoperative neural response was present in all cases. The auditory and speech performance improved in each DS child. The postoperative mean CAP and SIR scores were 4.4 (SD 0.8) and 3.2 (SD 0.6), respectively. The average of scores in MUSS and MAIS/IT-MAIS scales was 59.8% (SD 0.1) and 76.9% (SD 0.1), respectively. Gathered data indicates that children with DS implanted with CI at a younger age (<6 years of age) benefited from the CI more than children implanted later in life, similarly in a control group. There were additional anomalies of the temporal bone, external, middle, or inner ear observed in 90% of DS children, basing on MRI or HRCT. Conclusions: The early cochlear implantation in children with DS is a similarly useful method in treating severe to profound sensorineural hearing loss (SNHL) as in non-syndromic patients, although the development of speech skills present differently. Due to a higher prevalence of ear and temporal bone malformations, detailed diagnostic imaging should be taken into account before the CI qualification. Better postoperative outcomes may be achieved through comprehensive care from parents/guardians and speech therapists thanks to intensive and systematic rehabilitation.

Branchial cleft anomalies constitute 32% to 45% of all neck pathologies in the pediatric population. These disorders may be a part of a branchio-oto-renal syndrome (Melnick-Fraser syndrome), characterized by branchial arch abnormalities, preauricular pits, hearing impairment, and various types of renal anomalies. Usually, the treatment of a branchial fistula does not necessarily require extensive diagnostics. However, in patients with a congenital branchial cleft fistula associated with ear anomalies recognized during a physical examination, and history of hearing loss or similar findings in other relatives, the additional analysis should be carried out to eliminate the possibility of BOR syndrome. The aim of this study is to present a rare case of a male patient presenting complete second branchial cleft fistula, diagnosed as having BOR syndrome.

Introduction: Primary tumors arising from the peripheral nerves are a rare occurrence. In 90% they are benign, although in a small percentage there is a risk of neoplastic transformation. Schwannomas and neurofibromas are the leading etiologies. They are commonly found in the neck and large nerves of limbs and arms, with a preference for regions of the elbow, wrist, and knee. The most vulnerable group of patients are people aged 20 to 50 years. The occurrence of this type of tumors in children is sporadic, especially if the tumor includes the brachial plexus. Materials and Methods: The clinical case of a neurinoma in a supraclavicular region, affecting a 12-year-old girl. After a detailed diagnosis, the tumor finally came to be a schwannoma of a brachial plexus. Discussion: Brachial plexus schwannoma is a rare find, although it should be considered in the differential diagnosis. A detailed search for signs of neurofibromatosis (von Recklinghausen's disease) should also be performed. Initial diagnostic imaging of cervical mass includes mainly MRI, thanks to which the surgeon can suspend the tumor of nervous origin, assess the vicinity of important nerves, vessels, and cervical spinal cord, as well as qualify for the surgery. Surgical treatment should be carried out after assessing the benefits and risks associated with the procedure, including irreversible damage to the brachial plexus. To maintain safety during the operation, the use of microsurgical instruments, microscopes or endoscopes should be considered by an experienced surgeon. Preserving nerve continuity is the essential goal of the treatment, especially in young, and active patients. Finally, the long-term observation is necessary for the assessment of potential tumor neoplastic transformation, nerve function of the brachial plexus and possible symptoms of neurofibromatosis. Keywords: Brachial plexus tumor; Schwannoma in early adolescence; Neurofibromatosis; Supraclavicular swelling