Surgical treatment of acoustic neuroma: Outcomes and indications.

AIM: Surgery for acoustic neuroma has developed over the years with a gradual improvement in outcome. The goal of surgery should be cure of the disease and a low morbidity, preserving facial nerve function - and hearing, too, whenever feasible (i.e. in small tumors). The most appropriate approach must be decided case by case. BACKGROUND: Results of microsurgery remain heterogeneous, however, probably due to the different amount of experience gained at different centers. MATERIALS AND METHODS: Surgical series reported in the recent literature (2012-2015) were critically reviewed, along with some older papers with particularly representative series. RESULTS AND CONCLUSION: Considering patients' age, surgical morbidity of different approaches, tumor size and hearing, some principles can be identified on which to base surgical indications with a view to achieving the goals of curing the disease with a low morbidity, preserving facial nerve function and hearing, too, whenever feasible (i.e. in patients with small tumors). Different therapeutic approaches are discussed.

Bilateral chondrosarcoma of the jugular foramen: literature review and personal experience.

Chondrosarcomas (CS) are slow-growing malignant cartilaginous tumors with locally invasive behavior. They account for only 0.15% head and neck neoplasia. There have been no reports in the management of bilateral skull base CS in the literature to date. The synchronous presentation of bilateral CS of the jugular foramen (JF) was diagnosed in a 22-year-old woman with right abducens nerve palsy. Once evaluated the collateral intracranial venous discharge, the lesions were removed in two surgical stages through a bilateral petro-occipital trans-sigmoid (POTS) approach performing a bilateral closure of sigmoid sinus. The patient is disease free 15 years after surgery. No complications occurred. Diplopia improved after excision of the tumor on the right side. A review of relevant English literature was performed. The POTS approach to the JF proved to be safe and effective. Staged radical surgery alone, assessing intracranial venous flow at all stages of surgery, was a valid strategy for bilateral CS, achieving long-term disease control, avoiding early adjuvant radiotherapy, and carrying no complications.

Poor readers but compelled to read: Stroop effects in developmental dyslexia.

We studied a group of 24 children with dyslexia in second to fifth primary school grades by using a discrete-trial computerized version of the Stroop Color-Word Test. Since the classic Stroop effect depends on the interference of reading with color naming, one would expect these children to show no interference or, at least, less interference than normal readers. Children with dyslexia showed, however, a Stroop effect larger than normal readers of the same age. This suggests that reading, although difficult and slow, is an inescapable step that precedes naming both in poor and in normal readers.

Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes.

Ear, nose and throat (ENT) manifestations in granulomatosis with polyangiitis (GPA) represent the most frequent symptoms at disease onset. The aim of the study was to analyse ENT involvement at diagnosis, as well as how it could influence relapse rate, mortality and disease severity. A retrospective non-controlled cohort study was performed including all consecutive diagnosed GPA from 1996 to 2016 in two rheumatology centres of Northern Italy, focusing particularly on ENT presenting signs and symptoms at baseline. Eighty-nine patients (48.3% females) with new onset GPA were evaluated. They were mostly Caucasian (97.7%), middle aged (mean 54.5 years) and more frequently anti-neutrophil cytoplasmic antibodies (ANCA) positive (78.6%) with PR3 specificity (81.4%). At diagnosis, ENT involvement was reported in 71.9% patients, second only to systemic symptoms. These patients were significantly younger at disease onset (0.013), with less frequent renal involvement (0.014) irrespectively to ANCA status, but with significantly higher Vasculitis Damage Index (VDI) (0.001). The most frequent ENT manifestation was sinonasal involvement (58.4%, 73% of which with nasal inflammation/chronic sinusitis and 48% with nasal crusting), while otologic involvement (mainly otitis media/otomastoiditis) was observed in 34.8%. ENT-GPA patients presented a higher survival rate at 5 years (98.1 vs 77.7%, 0.049), and ENT involvement resulted to be an independent predictor of better outcome (OR 0.37, 95% CI 0.2-0.8, 0.019). Our data confirms that ENT involvement is not only one of the key clinical features of GPA, but also could point out a milder GPA subset with lower renal involvement and lower mortality rate, irrespectively to ANCA status.

YAP, TAZ and AREG expression in eighth cranial nerve schwannoma.

BACKGROUND: Although the diagnosis and treatment of eighth cranial nerve (VIII CN) schwannoma (acoustic neuroma) has improved over the years, no factors capable of predicting tumor growth have been identified as yet. This study is a preliminary investigation of the expression in sporadic VIII CN schwannomas of Yes-associated protein (YAP), transcriptional coactivator with PDZ-binding motif (TAZ), and amphiregulin (AREG), a direct target gene of YAP and TAZ. The expression of YAP, TAZ and AREG was correlated with the volumetric dimensions of tumors on contrast-enhanced magnetic resonance imaging (ceMRI). METHODS: YAP, TAZ and AREG expression was assessed immunohistochemically in surgical specimens of 36 consecutive sporadic VIII CN schwannomas. 3D reconstructions of the tumors and their corresponding volumes in cm3 were obtained from measurements on ceMRI images using the OsiriX® software. RESULTS: We found a significant direct correlation between TAZ expression and VIII CN schwannoma volumes on latest preoperative ceMRI (p<0.0003). Mean TAZ expression was also significantly higher in VIII CN schwannomas with a volume ≥2.1 cm3 than in those with a volume <2.1 cm3(p<0.0018). No significant correlations emerged for YAP or AREG expression and VIII CN schwannoma volume. CONCLUSIONS: The immunohistochemical expression of TAZ (but not YAP or AREG) correlated significantly with schwannoma volume measured on ceMRI. Further investigations are needed to identify the biological factors influencing tumor proliferation (ideally secreted proteins like AREG) that might be detected using non-invasive approaches (i.e., blood samples).

Intracranial venous sinus thrombosis as a complication of otitis media in children: Critical review of diagnosis and management.

OBJECTIVES: Otogenic lateral sinus thrombosis (LST) is a rare intracranial complication of acute otitis media (AOM), which can lead to severe neurological sequelae and death. The aim of this study was to analyze the clinical presentation, management and outcome of LST in children, investigating a possible correlation between clinical aspects, radiological findings and anatomical variations. METHODS: At a tertiary Italian hospital, a retrospective review was conducted on the medical records of eight patients diagnosed with otogenic LST over a 3-year period. Four children were males and mean age was 4.7 years. RESULTS: All patients had a history of otitis media at diagnosis and 4/8 presented also with more than one neurological sign or symptom. Mastoiditis signs were detected in 5/8 patients. Thrombosis was diagnosed by computed tomography, enhanced magnetic resonance and magnetic resonance venography. Treatment was medical, alone or combined with surgery. Medical treatment consisted in anticoagulants eventually combined with anti-edema medication on clinical basis. Mastoidectomy and/or myringotomy±trans-tympanic drainage placement were performed in 7/8 patients. Complete vessel recanalization was obtained in 6/8 children after a median follow-up time of 4.8 months. No complications, neither clinical sequelae occurred. In our series, neurological signs and symptoms were significantly associated with the presence of hypoplasia of the contralateral venous sinus (p=0.029). CONCLUSION: LST is a severe condition occurring even in absence of otological signs, and despite adequate antibiotic therapy for AOM, which should be ruled out and promptly treated. A dominant neurological presentation is associated in our series with anatomical variations of cerebral sinus venous drainage patterns. This should be carefully evaluated and considered in diagnosis, treatment planning and prognosis.

A soft-surgery approach to minimize hearing damage caused by the insertion of a cochlear implant electrode: a guinea pig animal model.

OBJECTIVE: A "soft surgery" technique was applied, using various types of specifically designed dummy electrodes, to mimic cochlear implantation in a guinea pig model, and the degree of hearing-preservation/cochlear damage was assessed. METHODS: Tricolor guinea pigs were divided into 3 groups: group A were implanted with electrodes without any contacts or wires (soft electrode), group B were implanted with electrodes having a metallic wire inside (stiff electrode), and group C underwent a cochleostomy procedure without implantation. Compound action potentials, in the range of 4 to 32 kHz, were used to assess electrophysiologic changes in the hearing function presurgery and postsurgery. Data were collected before surgery, at times t = 0 (immediately after surgery) and at 3, 7, 14, and 30 days. RESULTS: At low frequencies (4-8 kHz), an immediate elevation of hearing threshold was observed in all 3 groups. Higher threshold shifts were more consistent for group B implanted with a stiff electrode, in comparison to the other 2 groups. Animals from group C presented a recovery from hearing loss, starting 3 days after surgery. At high frequencies (16-32 kHz), the elevation of hearing threshold was higher, as compared with the data from the low frequencies. Group C animals presented oscillatory threshold shifts twice, and the recovery to normal threshold values occurred approximately at t = 14 days. CONCLUSION: The data suggest that cochleostomy is minimally harmful to the inner ear and that a soft electrode might better preserve the inner ear integrity than a rigid electrode.