Cogan's syndrome (CS) is a rare inflammatory disease characterized by interstitial keratitis or uveitis, vestibular impairment, and progressive hearing loss, commonly bilateral. Although glucocorticoids are fundamental treatment options, in most cases, hearing loss gradually worsens. Herein we report 2 pediatric cases of CS who were treated with corticosteroids and methotrexate. One patient had a cochlear implant, and the hearing of the other patient improved with treatment. Also, a systematic literature review was conducted for articles including pediatric CS patients. In the literature, 34 articles describing 44 pediatric patients with CS were identified. Sudden hearing loss (95.3%) and ocular symptoms (92.5%) were the most common manifestations in these patients. Also, aortic involvement was present in 19.5% of patients in the literature. Otorhinolaryngologists, ophthalmologists, and pediatricians should collaborate to diagnose and manage CS to prevent progressive hearing loss and eye involvement.

Abstract Objectives This study aimed to investigate automatic and voluntary motor control performances, which have an important function in maintaining balance, in children and adolescents with mucopolysaccharidosis (MPS). Methods The records of 70 patients were retrospectively analyzed. The results of Computerized Dynamic Posturography (CDP) performed according to the age and development of the individuals were examined. The results of 10 children and adolescents with MPS (mean age: 9.43 ranging from 6 to 14; four males and six females) who completed the sensory analysis, Weight-Bearing Squat Test, and Adaptation Test were retrieved from the database of the CDP. Nine healthy children and adolescents with typical development (mean age: 9.63 ranging from 6 to 14; four males and five females) were included as the control group. Results In the sensory analysis test, there was a statistically significant difference between the two groups in the visual ratio parameter. In the adaptation test, there was a statistically significant difference between the two groups in the toes up and toes down trials. There was no statistically significant difference between the groups in the Weight-Bearing Squat test at 0° knee extension and various knee flexions. Conclusions Children and adolescents with MPS should be directed to the appropriate exercise and therapy programs to develop postural and balance control, which have a significant effect on their quality of life and the ability to independently perform daily activities of living. In addition to routine hearing assessments for patients with MPS, other objective tests used in the differential diagnosis of balance and vestibular system should also be implemented.

Objective: Compared to adults, pediatric tracheotomy is associated with a higher incidence of complications. Various surgical techniques have been described to minimize complications.The aim of this study is to investigate the effects of three different tracheotomy techniques on tracheal complications and decannulation. Methods: Pediatric patients who underwent tracheotomy at Hacettepe University Department of Otorhinolaryngology were retrospectively analyzed. 62 patients were included in the study. Tracheotomy related complications and direct laryngoscopy images of all patients were examined in the postoperative period. The patients were divided into 3 groups according to the tracheotomy technique; vertical incision traction suture (VITS) technique (n: 24), vertical incision maturation suture (VIMS) technique (n: 19), inferior based flap (Bjork) technique (BT) (n: 19). Results: The rate of severe suprastomal pathology was detected as 29.2% in the VITS group, 15.8% in the VIMS group and 26.3% in the BT group (p = 0.656). The accidental decannulation rate was detected as 20.8% in the VITS group, 26.3% in the VIMS group and 10.5% BT group (p = 0.442). The rate of life-threatening occurrence until recannulated was detected as 60% in the VITS group, 20% in the VIMS group and 0% in the BT group (p = 0.394). The rate of tracheocutaneous fistula was detected as 13.3% in the VITS group, 40% in the VIMS group and 33.3% in the BT group (p = 0.337). Conclusions: There were no differences in complication rates based on techniques, and that stomal complications were common in small children requiring tracheotomies. (C) 2021 Japanese Society of Otorhinolaryngology-Head and Neck Surgery, Inc. Published by Elsevier B.V. All rights reserved.

Background. Choanal atresia is the most common congenital nasal anomaly, with an incidence of 1:5000-1:8000 live births. Atresia can be seen as membraneous, bony or mixed type. When it is bilateral, it is accepted lifethreatening, therefore bilateral atresia necessitates immediate intervention. Diagnosis is confirmed by endoscopic examination and computed tomography. The absolute treatment is surgical, and different approaches have been proposed. Methods. Herein, we describe our 15-year experience in the treatment of 58 patients of congenital choanal atresia with transnasal endoscopic approach, and we compare the efficacy of placement of an intranasal stent and applying mitomycin while endoscopic microsurgical repair. Results. The study included 41 female patients (71%) and 17 male patients (29%) with congenital CA. The mean age was 3 years ranging from 10 days to 16 years. The atretic plate was bilateral in 24 patients (41%) and unilateral in 34 (59%). The most common atresia type was the mixed type with 29 patients (50%). A total of 17 patients (29%) required postoperative revision(s). Postoperative revisions were more frequent among patients with bilateral CA (50%), and with mixed CA (31%). Stenting was used additionally by surgical correction for 10 patients. After stenting, fibrosis and restenosis was seen in 7 patients (79%). Mitomycin C was applied peroperatively in 8 patients. Restenosis after mitomycin application was seen in 4 patients (50%). Conclusions. By our experience, endoscopic microsurgical repair of atresia proved to be an effective and safe procedure, results compared with adjuvant treatment modalities like stent or mitomycin C use, was not better. Restenosis was the major problem seen after surgical correction.

Objective: This study is designed to determine the value and accuracy of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in the diagnosis of swallowing disorders as a diagnostic tool, in comparison with the widely accepted Videofluoroscopic Swallowing Study (VFSS) in pediatric patients.Methods: Cross-sectional study in tertiary referral center. Fifty one children with swallowing difficulty due to various diseases were prospectively evaluated using both VFSS and FEES. The variables, early pharyngeal spillover, pharyngeal residues, laryngeal sensitivity-silent aspiration, laryngeal penetration and laryngeal aspiration were evaluated in all patients. Results: Mean patient age was 29.8±17.8 (range 9-72) months. Six patients were younger than 13 months, 19 were between 13 and 24 months, and 26 patients were older than 2 years of age. There were 21 (42%) females and 30 (58%) males in the study group. Significant correlation in pharyngeal residues and laryngeal sensitivity-silent aspiration findings were found between FEES and VFSS data. No Significant dissimilarity in laryngeal aspiration and penetration evaluation was found. Conclusion: Swallowing evaluation in children is more challenging than adults. Study findings showed that FEES outcomes correlate with VFSS data, especially in the diagnosis of laryngeal aspiration and FESS is a valuable tool in identifying swallowing disorders in pediatric patients

INTRODUCTION:Mucopolysaccharidosis (MPS) IVA or Morquio A syndrome is an autosomal recessive lysosomal storage disorder (LSD) caused by deficiency of the N-acetylgalactosamine-6-sulfatase (GALNS) enzyme, which impairs lysosomal degradation of keratan sulphate and chondroitin-6-sulphate. The multiple clinical manifestations of MPS IVA present numerous challenges for management and necessitate the need for individualised treatment. Although treatment guidelines are available, the methodology used to develop this guidance has come under increased scrutiny. This programme was conducted to provide evidence-based, expert-agreed recommendations to optimise management of MPS IVA. METHODS:Twenty six international healthcare professionals across multiple disciplines, with expertise in managing MPS IVA, and three patient advocates formed the Steering Committee (SC) and contributed to the development of this guidance. Representatives from six Patient Advocacy Groups (PAGs) were interviewed to gain insights on patient perspectives. A modified-Delphi methodology was used to demonstrate consensus among a wider group of healthcare professionals with experience managing patients with MPS IVA and the manuscript was evaluated against the validated Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument by three independent reviewers. RESULTS:A total of 87 guidance statements were developed covering five domains: (1) general management principles; (2) recommended routine monitoring and assessments; (3) disease-modifying interventions (enzyme replacement therapy [ERT] and haematopoietic stem cell transplantation [HSCT]); (4) interventions to support respiratory and sleep disorders; (5) anaesthetics and surgical interventions (including spinal, limb, ophthalmic, cardio-thoracic and ear-nose-throat [ENT] surgeries). Consensus was reached on all statements after two rounds of voting. The overall guideline AGREE II assessment score obtained for the development of the guidance was 5.3/7 (where 1 represents the lowest quality and 7 represents the highest quality of guidance). CONCLUSION:This manuscript provides evidence- and consensus-based recommendations for the management of patients with MPS IVA and is for use by healthcare professionals that manage the holistic care of patients with the intention to improve clinical- and patient-reported outcomes and enhance patient quality of life. It is recognised that the guidance provided represents a point in time and further research is required to address current knowledge and evidence gaps.

Objectives: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It usually has a childhood onset and is characterized with recurrent attacks with irregular intervals. Few studies have been performed to investigate hearing in FMF patients ran with various tests and showed different results. In this study, first time in the literature, we aimed to evaluate and compare auditory functions in pediatric FMF patients during the attack periods and attack-free periods to see the possible effects of auto inflammation that was caused by FMF attacks. Methods: 40 pediatric FMF patients (80 ears) enrolled in the study as study group and 21 matching (42 ears) healthy children as a control group. Blood samples were taken from patients who presented clinical attack symptoms. Control group and FMF patients that were in attack period underwent audiometric evaluation including pure tone audiometry (0.25, 0.5, 1, 2, 4, 8 kHz) and Distortion product otoaoustic emission (DPOAE) (1, 1.4, 2, 2.8, 4 kHz) test. The tests were repeated in attackfree period. Correlations between hearing results and C-reactive protein, erythrocyte sedimentation rate levels, duration of disease, age at disease onset, colchicine usage time, cumulative dosage of colchicine and the number of attacks in the last six months were studied. Results: Hearing thresholds of FMF patients, compared to control group, were found to be increased at most frequencies. Lower signal/noise ratios (SNR) were detected at most frequencies in the DPAOE test. In the attack period compared to attack-free period, hearing thresholds in the audiometry were found to be increased at some frequencies (p < 0.05). However, this was not supported by the DPOAE test. In correlation analysis, increased colchicine usage time and colchicine cumulative dosage were associated with decreased thresholds in audiogram and increased SNR values in DPOAE. Detailed statistical analyses of all parameters were included in the study. Conclusion: Results demonstrated cochlear involvement in FMF patients. Acute changes in hearing thresholds in the attack period may be suggesting the effect of acute inflammation on cochlea. The difference between the audiometry and otoacoustic emission test results of FMF patients and healthy controls suggests the cumulative effect of recurrent inflammation attacks on cochlea. Positive affect of treatment of the disease with long term colchicine in hearing were also demonstrated.

Background : Cricotracheal stenosis has become recognised as a pathological condition and remains a challenging problem for otorhinolaryngology. The aim of this work is to stress importance of single stage surgery for cricotracheal stenosis. Methods: The patients with cricotracheal stenosis who were treated with single stage surgery with cricotracheal or tracheal resection between 2009 and 2015 at Hacettepe University Hospitals Department of Otorhinolaryngology Head and Neck Surgery were evaluated retrospectively. Postoperative restenosis rate and additional intervention requirement for decannulation were compared with stenosis grade and comorbidity. Results: This study included 24 males and 8 females aged between 18 and 76 years with a mean age of 38.8 years. Twenty seven (84.3 %) patients had a pre-existing tracheostomy at the time of surgery while in five (16.6%) patients surgery was performed without a preoperative tracheostomy. One patient had grade 2 (3.1%) , 25 Patients had grade 3 (78.1% ) and 6 patients had grade 4 (18.8%) stenosis according to Cotton-Myer grading system. All patients underwent single stage surgery and followed at ICU as an intubated. Overall decannulation rate was 93.7%. Conclusions: Cricotracheal reconstruction techniques pose many challenging obstacle. Single stage surgery introduce best treatment option and is especially required multidisciplinary approach which is essential to manage the cricotracheal stenosis with high decannulation rates. Keywords: Cricotracheal stenosis, cricotracheal resection, single stage surgery.

Intratracheal ectopic thyroid tissue is a rare abnormality that can cause air-way obstruction. Presence of ectopic tissue in the larynx is extremely rare. The symptoms can easily be confused with those of bronchial asthma. We describe the case of a 52 year old man with subglottic thyroid tissue and multinodular goiter who undergone total thyroidectomy of for multinoduler goitre in octo-ber 2009. After the correct diagnosis was established, the lesion was excised via an external approach. We also discuss the clinical features and management of intratracheal thyroid tissu Keywords: Intratracheal, subglottic mass, ectopic thyroid tissue

Objective: The objective of this study was to review the management of deep neck space infections in pediatric patients and to evaluate the efficacy of intravenous antibiotic treatment alone before surgical drainage, and also to point out the indications for the drainage. Methods: We reviewed sixty pediatric cases who were treated in our clinic because of deep neck space infections. The details of demographic data, medical history, initial complaints and physical examination, radiological examination, microbiology and laboratory results (C-reactive protein level and leukocyte count), treatment modality and follow-up findings were collected. The bacteriological results, management, complications, follow-up data and outcomes were also noted. A basic treatment algorithm for the management of the pediatric deep neck space infections was constituted. Results: In 47 (78.3%) of the children, infection did not require any surgical intervention or puncture - in other words, needle aspiration - and it was successfully treated with antibiotic therapy alone. Fifty-six patients (93%) were initially treated with sulbactam-ampicillin. Conclusion: We advise surgical drainage in cases of fluctuating large abscesses and infections without clinical improvement despite antibiotic treatment, and in complicated or life-threatening cases such as retropharyngeal abscess and mediastinitis. An otolaryngologist should be patient before any surgical intervention.

Spirometry is an easy method to measure lung function and to show pathophysiology. It assists not only to determine the severity of bronchial obstruction in asthma but also to differentiate the characteristics of the intrathoracic diseases narrowing the central airways. Different types of benign and malignant tumors of the trachea may cause emergence of symptoms of airway obstruction. Herein a patient who had been initially diagnosed with asthma but later on shown to have intratracheal myofibroblastic tumor is presented. The importance of flow-volume curve in both initial diagnosis of the mass and in the detection of recurrence is discussed.

The aim of this study is to evaluate parotid masses management in pediatric population. From June 2002 to December 2014, 13 patients under the age of 18 with parotid tumors were treated via surgery at Hacettepe University Department of Otorhinolaryngology. Information on patients' demographics, clinical and histopathologic tumor characteristics and treatment modalities with results was obtained from medical records, retrospectively. The age was ranging between 8-17 years. Ten (76.9%) patients had benign tumors and 8 (61.5%) of them were pleomorphic adenoma. One case had chronic sialadenitis with sialolithiasis and one patient had fluoride follicular hyperplasia. Three patients had malignant tumors; two of them were adenoid cystic carcinoma and one case of malignant melanoma metastasis. Twelve cases had undergone partial parotidectomy. Only one total parotidectomy was performed which was the patient with malignant melanoma. None of the patients had major complications or facial nerve palsy. During the 3 years follow up period, recurrence or Frey syndrome had not been detected but the case with malignant melanoma had distant metastasis during the follow up time. Although pediatric parotid masses are unusual, they can represent a variety of pathological diagnoses, including malignancy. Parotidectomy remains the mainstay of treatment and surgical experience is needed for low complication rates especially in children.

Objectives: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC.Methods: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured.Results: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively).Conclusion: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

Ganglioneuroma is a rare benign tumor that originates from neural crest. Tumor tends to be slow growing, asymptomatic but can cause symptoms because of pressure to neighboring structures. In the head and neck region they are relatively rarely seen. We hereby present a rare case of multiple ganglioneuromas that were located in parapharyngeal space, iliac bone and other bones in a 13-year-old girl. Patient underwent surgery for the excision of a large mass, extending from parapharyngeal space to neck, with transparotid and transcervical combined approach. After operation, MIBG (iodine-123-meta-iodobenzylguanidine) scintigraphy was performed and involvement of parietooccipital bone, lumbal vertebra, right iliac wing medial cortex and left humerus were detected. No adjuvant therapy was given to the patient. There is no evidence of recurrence in the head and neck region in the following 12 months. In conclusion, complete surgical excision of the tumor, if possible, is the treatment of choice with high success rate. Close clinical and radiological follow-up for these tumors after surgery should be made.

Purpose: The aim of the study is to compare long term otoscopic and audiological findings of cleft palate patients with or without early grommet insertion.Methods: Cleft palate patients followed-up in Hacettepe University between 2008 and 2013 were included in the study. Age, gender, cleft types and palate surgery data, grommet tube insertion history and otological audiological evaluations of the patients were recorded. Patients were evaluated in three groups according to grommet insertion history: A-early routine grommet insertion, B-grommet insertion during follow-up, C-no grommet insertion. Otological and audiological findings were compared.Results: There were 154 patients in the study, with a median age of 7.7 years. There were 67 patients in group A (43.5%), 22 patients in group B (14.3%) and 65 patients in group C (42.2%). OME was identified significantly higher in group A and normal otoscopic examination findings were higher in group C. Complications showed a higher rate than other otoscopic findings in group B patients. There was no significant difference for any frequencies in between the groups in terms of mean air-bone gap (ABG) values. There were 20 grade I, 25 grade II, 77 grade III and 32 grade IV patients in the study according to the Veau classification.Conclusion: Prophylactic grommet insertion may not be applied as some cleft palate patients with no OME. Wait and see protocol can be recommended for these patients, and they should be followed-up up closely to avoid complications. If the effusion does not recover or tympanic membrane changes occur in follow-up, grommet insertion should be considered. (C) 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Lower cranial nerve palsy, also known as Collet-Sicard syndrome, is a fairly rare pathology characterized by unilateral palsy of the IX-X-XI-XII cranial nerves. We report a multiple cranial nerve palsy developing after a head trauma that might have been considered negligible. A 16-year-old boy was admitted with swallowing and articulation problems and difficulty lifting one shoulder after a head trauma suffered during a football match. No pathology was revealed in the subsequent imaging. Cranial nerve palsies due to head trauma are very rare. Awareness of the possibility of such a condition, leading to early recognition and treatment, may result in significant functional recovery.

The aim of this work is to stress the importance of and discuss the timing and options for the treatment of congenital aural atresia (CAA), including non-surgical alternative treatment modalities and amplification, and to report the audiological and surgical results of a series of patients. Thirty-eight children with CAA were evaluated with regard to hearing and anatomical anomalies accompanying CAA: the state of the ossicles and the facial nerve, postoperative complications and audiological results. The ages of the patients ranged between 4 and 18 years, with a mean of 10 years. All underwent surgical treatment; 32 had unilateral atresia, while 6 had bilateral atresia. The mean follow-up duration was 7 months. The facial canal was dehiscent in 36.8% of cases. In 70.2% cases, the malleus and incus were present as an ossicular mass, fixed and attached to the atretic bone. The stapes was normal in 97.3% of the patients; in 2.7% the suprastructure was deformed. The success rate, defined as an air-bone gap of 20 dB or less, was 63.1% in this series of patients. If atresia is bilateral, very early hearing stimulation to prevent the maldevelopment of children's speech and cognitive skills is of the utmost importance. In unilateral cases, surgery may be postponed until early adulthood, when the patient is able to make his/her own decision and cooperate in the treatment and postoperative aspects.

Objectives: The scope of the study is to compare endolaryngeal dilatations (ED) with laryngotracheal reconstruction with cartilage grafting (LTRCG) in terms of restenosis.Methods: Pediatric subglottic stenosis patients treated in Hacettepe University, between 2002 and 2012 were retrospectively evaluated. Patients who had ED or LTRCG as primary management were included in the study. EDs were grouped into bronchoscopic dilatation (BD), laser incision and balloon dilatation (LBD) and cold knife incision and balloon dilatation (CKBD). The groups were evaluated in terms of restenosis and decannulation rates.Results: There were 35 patients (9 females, 26 males; mean age 4.42). LTRCG was performed in 16 patients (9 anterior and 7 anterior and posterior grafts). EDs were performed in 19 patients with 6 CKBDs, 7 LBDs and 6 BDs. There were 3 grade II, 13 grade III cases in the LTRCG group while 4 grade 1, 6 grade II, 8 grade 3 and 1 grade 4 in the ED group. Overall decannulation rate was 97% (34/35) in all patients. Restenosis was higher in the ED group (63.2%) than the LTRCG group (31.3%) with rates of CKBD 16.7% (1/6), LBD 71.4% (5/7) and BD 100% (6/6). Restenosis rates were found to be increasing with higher grades (grade I-25%, grade II-66%, grade III-85%).Conclusion: ED may need more repetitive interventions than LTRCG due to restenosis. Less restenosis might be observed when balloon is used for dilatation and cold knife for mucosal incisions. (C) 2014 Elsevier Ireland Ltd. All rights reserved.