Georgios Kontorinis

Pseudohypacusis: the most frequent etiology of sudden hearing loss in children

Sudden hearing loss is a rare pathology in children. Several factors may be responsible for it although the exact etiology remains frequently undiagnosed. Among them, pseudohypacusis has been reported. However, the extent to which this pathology contributes to sudden hearing loss in children is unknown. This study evaluates the incidence of pseudohypacusis in children presented with sudden hearing loss. The medical records of 48 children presented to our department because of sudden hearing loss from 2002 to 2007 were reviewed. Diagnostic process included both subjective and objective audiological tests while organic hearing losses were further subjected to proper evaluation and treatment. 26 cases (54%) of pseudohypacusis and 22 cases (46%) of organic sudden hearing loss were diagnosed. In the pseudohypacustic group, girls outnumbered boys (16:10) and their mean age was 10.5 years. Pseudohypacusis represents the most frequent etiology of sudden hearing loss in children. Its detection is relatively simple using conventional audiological tests though in some cases even experienced clinicians may come to incorrect diagnosis.

Airway obstruction in children with cerebral palsy: need for tracheostomy?

OBJECTIVE To examine the progress of the airway obstruction over time in children with cerebral palsy (CP) and the timing of any interventions. METHODS The medical notes of patients with CP younger than 16 years admitted with airway obstruction to a tertiary referral Pediatric Otolaryngology Center from 2006 to 2012 were retrospectively reviewed. The gender, age of referral, co-morbidities, type of surgical intervention and age this was performed and the time interval between sequential surgeries were documented. RESULTS Fifteen children with CP and airway obstruction were admitted, eight boys and seven girls with an average age of referral 8 years (range 3-13.3 years). Adenotonsillectomy was performed in 11/15 patients at a mean age of 9.1 years (range 4.5-14 years). Tracheostomy was performed in 8/15 children at an average age of 11.6 years (range 7.5-15 years). Seven out of 11 patients having undergone adenotonsillectomy, required tracheostomy after an average time interval of 1.9 years (range 0.5-3.5 years). Tracheostomy was performed in 80% of referred patients with CP older than 10 years, while surgical intervention was uncommon in children younger than 5 years. There was a statistically significant correlation between the age of the children and the performance of a tracheostomy (Pearsons correlation coefficient 0.68, p = 0.005). CONCLUSIONS The severity of the airway obstruction in children with CP tends to increase with age. We postulate that this increase results from worsening hypotonia of pharyngeal musculature. Children with CP and severe upper airway obstruction are likely to require tracheostomy as they grow older.

Aplasia of the cochlea: radiologic assessment and options for hearing rehabilitation.

Objective Hearing rehabilitation of patients with severe inner ear malformations remains controversial. Our objective was to describe the radiologic findings of aplasia of the cochlea (AC) and evaluate the existing therapeutic options in such patients. Study Design Retrospective cohort study. Setting Tertiary referral center. Patients Pediatric and adult patients with AC evaluated at our institution from 1995 to 2010. Interventions The precise radiologic findings were identified using high-resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) scans of the inner ear. In cases of auditory implantation on the AC side, the achieved outcome was recorded using categories of auditory performance (CAP). Related surgical aspects were analyzed. Results Twenty-three patients (28 ears) with AC were found. In 5 patients AC was bilateral. The remaining unilateral cases had contralateral normal ears (2 patients), cochlea hypoplasia (5 patients), common cavity (6 patients), incomplete partition Type I (4 patients), and atresia of the internal auditory canal (1 patient). Four patients (3 bilateral, 1 unilateral ACs) were treated with cochlear implants in ears with AC, and 1 patient underwent auditory brainstem implantation. All implanted patients achieved speech perception with limited vocabulary (CAP scores between 4 and 5). Conclusion AC is defined as the total absence of the cochlea, with a present, although malformed, vestibule. Although a distinct auditory nerve was not seen in these cases of AC, results following cochlear implantation suggest functional cochlear nerve fibers in the remaining dysplastic inner ear structures. In selected cases, cochlear implantation may be a reasonable option for the habilitation of deafness associated with AC.

Geographic distribution of vestibular schwannomas in West Scotland between 2000-2015

BACKGROUND Although the natural history of vestibular schwannomas (VS) has been previously studied, few studies have investigated associated epidemiological factors, primarily because of the lack of large available cohorts. OBJECTIVE The objective of this study was to perform a multi-scale geographical analysis of the period prevalence of VS in West Scotland from 2000 to 2015. METHODS Adults diagnosed with sporadic VS were identified through the National Health Services of West Scotland database and geocoded to the unit postcode. To assess whether the cohort of VS cases could be pooled into a period prevalence measure, the locations of VS cases were analyzed by sex using Cross-L and Difference-K functions. VS period prevalence was examined at two aggregate spatial scales: the postcode district and a coarser scale of NHS Health Boards. The spatial structure of period prevalence within each level of spatial aggregation was measured using univariate global and local Morans I. Bivariate local Morans I was used to examine the between-scale variability in period prevalence from the postcode district level to the NHS Health Boards levels. Prior to spatial autocorrelation analyses, the period prevalence at the postcode district was tested for stratified spatial heterogeneity within the NHS Health Boards using Wangs q-Statistic. RESULTS A total of 512 sporadic VS were identified in a population of over 3.1 million. Between 2000 and 2015, VS period prevalence was highest within the NHS Health Boards of Greater Glasgow and Clyde, Ayrshire and Arran and the Western Isles. However, at the NHS scale, period prevalence exhibited no spatial autocorrelation globally or locally. At the district scale, Highland exhibited the most unusual local spatial autocorrelation. Bivariate local Morans I results indicated general stability of period prevalence across the postcode district to Health Boards scales. However, locally, some postcode districts in Greater Glasgow and Clyde, Ayrshire and Arran exhibited unusually low district to zone spatial autocorrelation in period prevalence, as did the southern parts of the Western Isles. Some unusually high period prevalence values between the postcode district to Health Board scale were found in Tayside, Forth Valley and Dumfries and Galloway. CONCLUSION Geographic variability in VS in West Scotland was identified in this patient population, showing that there are areas, even remote, with unusually high or low period prevalence. This can be partially attributed to links between primary and tertiary care. Potential genetic or environmental risk factors that may contribute to geographic variation in this disease within Scotland are also a possibility but require further investigation.

Delayed Vestibular Schwannoma Regrowth Following Shrinkage After Stereotactic Radiosurgery: Implication for Life-Long Surveillance

OBJECTIVE To enhance understanding of the behavior of vestibular schwannomas (VS) after stereotactic radiosurgery (SRS), including the potential for late tumor regrowth, and the need for lifelong radiological and clinical surveillance. PATIENTS From a total of 540 patients with VS and out of 95 patients receiving SRS for their growing VS, the cases of two women patients aged 58 and 59 years with medium sized, growing VS are presented. INTERVENTION Both patients were treated with SRS. Following treatment, both patients had close clinical follow-up and serial magnetic resonance imaging (MRI). MAIN OUTCOME MEASURES Post-SRS clinical assessment and serial MRI to determine VS size and respond to treatment. RESULTS Close clinical follow-up after SRS identified no change in symptoms in one patient; the other developed ipsilateral facial nerve twitching without weakness. Serial MRI identified that in both patients, VS that had initially decreased in size following SRS, exhibited significant late regrowth after a 3-year period, reaching the pretreatment size. CONCLUSIONS This is the first report of late vestibular schwannoma regrowth following a period of significant shrinkage after SRS. Potential reason may be the presence of living cells within the tumor; the possibility of malignant transformation should also be taken into account. Following SRS, patients with VS warrant lifelong radiological and clinical surveillance, even following good response, as there seems to be a small chance of initial regression followed by further growth. Microsurgery for tumor removal and histological diagnosis is indicated in such cases.