Chiara Faccioli

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.

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).

Dizziness in the elderly

Abstract Dizziness is largely a problem in the elderly, being the most common reason patients over the age of 75 years seek medical attention. The term is used to describe many sensations, including vertigo, disequilibrium and light-headedness. There are different causes of dizziness in older people such as cardiovascular, neurological or locomotor disease, deterioration in sensory organs, vestibular dysfunction and adverse drug effects. Distinctions between the effects of aging and borderline pathology on instability and falls are not always clear-cut. The elderlys greater risk of balance disorders stems from a higher likelihood of impairments or diseases affecting the physiological subsystems underlying the complex skill of balancing. Diagnosing a specific cause of dizziness or vertigo in older people can be a challenge, since the symptom descriptions are often vague and examination findings overlap among potential causes. Once a differential diagnosis is formulated, it is not clear what tests – if any – will add clinical value. Intervention needs to be tailored to each patient to obtain the maximum effect, designing different exercises for individuals with different kinds of deficiency. Vestibular dysfunction in the elderly can be treated effectively with vestibular rehabilitation, which comprises both a ‘generic’ type and more specific protocols. Moreover, all the following interventions may have a role: exercises designed specifically to improve muscle function and to address sarcopenia, visual deficit treatment, pacemakers, vitamin D supplementation, gradual withdrawal of psychotropic medication, multifaceted podiatry and hip protectors, cognitive behavioural interventions, home safety assessment and modification interventions. In many cases, a multifactorial approach could be the best solution, as the elderly are frequently affected by multiple deficiencies. There is strong scientific evidence that rehabilitation has a significant role for multisensory dizziness, vestibular hypofunction and locomotor pathologies. More research is needed to evaluate the benefit of rehabilitation for neurological causes and benign positional paroxysmal vertigo.

Acoustic schwannoma with intracochlear extension and primary intracochlear schwannoma: removal through translabyrinthine approach with facial bridge cochleostomy and transcanal approach

Intracochlear schwannomas can occur either as an extension of a larger tumor from the internal auditory canal, or as a solitary labyrinthine tumor. They are currently removed via a translabyrinthine approach extended to the basal turn, adding a transotic approach for tumors lying beyond the basal turn. Facial bridge cochleostomy may be associated with the translabyrinthine approach to enable the whole cochlea to be approached without sacrificing the external auditory canal and tympanum. We describe seven cases, five of which underwent cochlear schwannoma resection with facial bridge cochleostomy, one case with the same procedure for a suspect tumor and one, previously subjected to radical tympanomastoidectomy, who underwent schwannoma resection via a transotic approach. Facial bridge cochleostomy involved removing the bone between the labyrinthine and tympanic portions of the fallopian canal, and exposing the cochlea from the basal to the apical turn. Patients’ recovery was uneventful, and long-term magnetic resonance imaging showed no residual tumor. Facial bridge cochleostomy can be a flexible extension of the translabyrinthine approach for tumors extending from the internal auditory canal to the cochlea. The transcanal approach is suitable for the primary exclusive intralabyrinthine tumor. The indications for the different approaches are discussed.

Hearing preservation and growth of small vestibular schwannomas: preliminary outcomes from patients under observation

Abstract Objective: Conservative management in sporadic vestibular schwannomas (VS) represents a valid treatment option, however, tumour growth and hearing decay during observation are still a matter of controversy and shift to alternative therapies. The present study aims at evaluating patients diagnosed with VS submitted to a wait and scan protocol of treatment. Study design: Case series review from a tertiary referral centre. Data collected from an in-house prospectively-maintained database of 108 patients with sporadic small VS, managed with primary observation, were evaluated. Inclusion criteria were tumour diameter less than 10 mm in the cerebello-pontine angle. At serial follow-up evaluations, objective measures of hearing status and tumour growth through magnetic resonance imaging were conducted. Hearing status was stratified according to the Tokyo Consensus Meeting Guidelines. Tumour growth and hearing function represented the main endpoints and were analysed according to tumour site at diagnosis. Results: Eighty-one patients were included in the study. During observation with a mean follow-up time of 27 months, 29.6% of the patients demonstrated tumour growth, with extrameatal lesions growing faster than intrameatal tumours (p = .049). Decay of good hearing was observed in 32.5% of patients, with a higher frequency of hearing instability revealed in the intrameatal group of VS (p = .004). Conservative treatment was maintained in 75.3% of cases. In 18% of cases, patients underwent surgical VS removal, while only 6.2% of patients favoured radiotherapy. Conclusions: Conservative treatment led to low rates of tumour growth after a mean 27 months observation. The cases with good hearing at diagnosis evidenced hearing deterioration in 33.3% of patients. Wait and scan outcomes need to be evaluated in comparison with other active treatments in terms of hearing and growth and further studies are required in this sense.

AB0551 Clinical Application of Two Different Disease Activity Scores for ENT Involvement in Granulomatosis with Polyangiitis

Background In the management of Granulomatosis with Polyangiitis (GPA), is critical to assess correctly the disease activity in each organ to define the better therapeutic approach and follow up strategy. This statement is also valid for ENT involvement, that we decide to assess with two different organ specific disease activity scores, the already validated Del Pero ENT/GPA DAS1 and a new ENT score developed by our Otolaryngology Unit (Padova,ENT/GPA DAS), composed by 8 items: nasal crusting, reported bloody rhinorrhea, nasal inflammation, laryngoscopy inflammation, objective stridor, inflamed TM/middle ear, siero-mucous otitis and hearing loss. Objectives Compare the accuracy of two different ENT scores (ENT/GPA DAS and Padova ENT/GPA DAS) and correlate them with the most commonly used GPA Activity Indexes and identify if they are effective to predict relapses that require therapeutic changes. Methods We selected retrospectively the GPA patients with ENT involvement followed by Rheumatology and Otolaryngology Units from 2006 to 2015. Each ENT evaluation of our patients were matched with temporally nearest Rheumatologic visit to assess the ENT/GPA DAS, the Padova ENT/GPA DAS, the Birmingham Vasculitis Activity Scores (BVAS v3, BVAS-WG), the Physician Global Assessment (PGA), the Vasculatis Damage Index (VDI), the serological biomarkers (ESR and CRP) and treatment strategy. For statistical analysis, we used Mann Whitney test, Spearman test and Fisher test. Results The selected ENT/GPA patients were 25 (41.6% of all GPA patient cohort), mainly middle aged (51.7±14.9 years), women (64%) and ANCA positive (80%) with PR3 specificity (75%). The total of ENT assessment were 67, so some patient were seen more than once (mean 2.68, min 1, max 8). The two considered ENT scores presented a significant difference (p<0.0004) from each other and the correlation analysis showed that ENT/GPA DAS was directly correlated to ESR (p<0.0001), CRP (p=0.03), BVAS v3 (p=0.005), BVAS-WG (p=0.004) and PGA (p<0.0001), while our Padova ENT/GPA DAS presented a significant correlation only with VES (p<0.0001) and PGA (p=0.027). The therapeutic strategies analysis observed that, in all cohort, the change of the immunosuppressive drug (32/67) presented higher BVAS v3 (p<0.0001), BVAS-WG (p<0.0001) and PGA (p<0.0001). Furthermore, in follow up visits of patient in maintenance therapy or induction of remission therapy, excluded those with Cyclophosphamide or Rituximab (25/44), we observed a significant increased rate of therapeutic change in those with ENT/GPA DAS higher than 0 (p=0.025, OR 5.8 IC95% 1.16–24.9) Conclusions Our analysis showed that ENT/GPA DAS is more performing than Padova ENT/GPA DAS to reflect the disease activity. This is probably a consequence that some items considered in our ENT/GPA DAS, such as all type of hearing loss, express better the damage than active disease. Moreover, the data showed that ENT/GPA DAS could identify patients with an active disease worthy of therapeutic changes, especially in particular patient subsets. This data should be confirmed with further evidences on larger GPA patients cohorts and prospective studies. References Del Pero MM et al Laryngoscope, 123:622–628, 2013. Disclosure of Interest None declared