Fibrous dysplasia is a slowly progressive benign fibro-osseous disorder that involves one or multiple bones with a unilateral distribution in most cases. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Temporal bone involvement is the least frequently reported type, especially in children. We reviewed available articles regarding fibrous dysplasia with temporal bone involvement in children and added four patients aged 7 to 17 years who were diagnosed and treated in our institution from 2006 to 2017. The patients’ clinical picture comprised head deformity, external canal stenosis, headache, progressive conductive and/or sensorineural hearing loss, tinnitus, and sudden deafness. Two patients had experienced severe episodic vertigo with nausea and vomiting. Two were referred to us with external canal obstruction and secondary cholesteatoma formation with broad middle ear destruction. One was diagnosed with acute mastoiditis and intracranial complications. Optimal management of fibrous dysplasia is unclear and can be challenging, especially in children. In our two patients with disease expansion and involvement of important structures, surgical treatment was abandoned and a “wait-and-scan” policy was applied. The other two were qualified for surgical treatment. One patient underwent two surgeries: modified lateral petrosectomy (canal left open) with pathological tissue removal, cavity obliteration, and subsequent tympanoplasty. Another patient with extensive destruction of the left temporal bone underwent canal wall down mastoidectomy with perisinus abscess drainage and revision 12 months later. Tympanoplasty was unsatisfactory in both patients because of slow progression of the middle ear pathology. None of our patients underwent pharmacological treatment. In younger patients, observation and a “wait-and-scan” protocol is relevant until significant function, or cosmetic deficits are obvious. Surgery is not preferred and should be delayed until puberty because fibrous dysplasia has a tendency to stabilize after adolescence. In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial.

Acute mastoiditis remains the most common complication of acute otitis media. It may rarely appear also in cochlear implant patients. However, the treatment recommendations for this disease are not precisely defined or employed, and in the current literature the differences regarding both the diagnosis and management are relatively substantial. The aim of this study was to determine a standard and safe procedure to be applied in case of pediatric acute mastoiditis. A retrospective chart review of 73 patients with 83 episodes of acute mastoiditis hospitalized at our tertiary-care center between 2001 and 2016 was conducted. Bacteriology, methods of treatment, hospital course, complications, and otologic history were analyzed. Based on our experience and literature data, a protocol was established in order to standardize management of pediatric acute mastoiditis. All the patients treated for acute mastoiditis were submitted to an intravenous antibiotic regimen. In the analyzed group pharmacological treatment only was applied in 11% of children, in 12% myringotomy/tympanostomy was added, and in the vast majority of patients (77%) mastoidectomy was performed. In our study recurrent mastoiditis was noted in 8% of the patients. We also experienced acute mastoiditis in a cochlear implant child, and in this case, a minimal surgical procedure, in order to protect the device, was recommended. The main points of the management protocol are: initiate a broad-spectrum intravenous antibiotic treatment; mastoidectomy should be performed if the infection fails to be controlled after 48h of administering intravenous antibiotic therapy. We believe that early mastoidectomy prevents serious complications, and our initial observation is that by performing broad mastoidectomy with posterior attic and facial recess exposure, recurrence of acute mastoiditis can be prevented.

Introduction: Although it is recommended to perform cochlear implantation in both ears at the same time for management of profound hearing loss in children, many centers prefer to perform sequential implantation. There are many reasons as to why a simultaneous bilateral implantation is not commonly accepted and performed. The major risk is the possibility of bilateral vestibular organ impairment. However, it is beyond doubt that children who received the first implant should be given a chance for binaural hearing and associated benefits. In the literature, there are no homogenous criteria for bilateral implantation, and it is hard to find uniform and convincing algorithms for second cochlear implantation. The aim of this study is an attempt to identify a safe way of qualifying for second cochlear implantation in children. Material and methods: Forty children with one cochlear implant were qualified for the second implantation. During qualification, the following were taken into account: time of the first implantation, audiometry results, use of the hearing aid in the ear without an implant and benefit of the device, speech and hearing development, and vestibular organ function. R esults: Fifteen out of forty children (38%) were qualified for the second implantation. In 35% of children, the decision was delayed with possible second implantation in the future. Eleven children (27%) were disqualified from the second surgery. Discussion: During evaluation according to the protocol presented in our study, 38% of children with a single cochlear implant were qualified for the second implantation with a chance for an optimal development and effective use of the second cochlear implant. We are convinced that sequential implantation with a short interval between surgeries and with an examination of the vestibular organ, hearing and speech development as well as an assessment of potential benefits from the second implant (bimodal stimulation) before the second implantation is the safest and most beneficial solution for children with severe hearing loss.

Introduction: Transimpedance matrix measurement (TIM) is an electrophysiological measurement protocol of the impedance patterns of electrode contacts within the cochlea. Several studies have reported that TIM is an effective tool for the identification of abnormal electrode array placement. However, the normative values for properly inserted electrodes, as well as correlation of the TIM patterns with the electrode position, are not completely determined. Objectives: The first aim of this study is to establish normative values of TIM measurements obtained in children with proper electrode array insertion and tip fold-over, with proper inner ear anatomy and in congenital anomalies. The second aim of this study is to compare TIM measurements in Slim Modiolar (SM) and in Contour Advance (CA) electrodes, as their position is different according to the modiolus proximity. Methods: A total of 55 pediatric patients were included in the study and underwent cochlear implantation. 62 intraoperative measurements were conducted in this group—50 in children with normal inner ear anatomy and 12 in children with inner ear malformations. After each implantation, a plain x-ray was obtained. Results: There were clear statistically significant differences in TIM patterns in patients where electrode fold-over was confirmed and between SM and CA electrodes. Conclusions: TIM is a promising technique for intraoperative analysis of electrode placement. TIM patterns differ and correlate consistently with the different models of array implanted. This study is the first to report TIM patterns observed in children with normal inner ear anatomy and in inner ear malformations.