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Ecchordosis physaliphora is a congenital, benign lesion originating from notochordal remnants along the craniospinal axis, most frequently located at the level of the clivus and sacrum. Sometimes ecchordosis physaliphora is difficult to recognise and treat, with a total of twenty-six cases described in the literature.
This study reports on three cases of previously undiagnosed ecchordosis physaliphora presenting with cerebrospinal fluid rhinorrhoea and meningitis.
Endoscopic transclival or transsphenoid surgery including three-layer (fat, fascia and nasoseptal flap) reconstruction was used in all cases with complete resolution of the symptoms.
Eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.
A literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. All patients underwent fibre-optic laryngoscopy with narrow-band imaging, and completed health-related questionnaires.
The literature review showed only two cases of laryngeal involvement in eosinophilic granulomatosis with polyangiitis; in our cohort, no cases of subglottis stenosis were found, but local signs of laryngeal inflammation were present in 72 per cent of cases. Of the patients, 16.2 per cent had a pathological Reflux Finding Score (of 7 or higher).
Laryngeal inflammation in eosinophilic granulomatosis with polyangiitis is frequent. It is possibly due more to local factors than to eosinophilic granulomatosis with polyangiitis itself. However, ENT evaluation is needed to rule out possible subglottis inflammation. These findings are in line with current literature and worthy of confirmation in larger cohorts.
To evaluate the long-term stability of intratympanic steroids and investigate the ‘real’ impact of sudden sensorineural hearing loss on patients.
A total of 14 patients treated with intratympanic steroids were evaluated by audiometric and vestibular examinations. The modified Glasgow Benefit Inventory was used to evaluate quality of life changes after intratympanic steroid treatment.
There was no significant difference between pure tone average post-intratympanic steroids and at follow up. The general Glasgow Benefit Inventory score was not significantly associated with the presence of tinnitus or dizziness, or with patient age. The change in pure tone average after intratympanic steroid treatment did not correlate with social or physical scores, but correlated strongly with the general Glasgow Benefit Inventory score (p = 0.0023). Intratympanic steroid administration led to a stable improvement in hearing. Quality of life assessment showed that patients can feel satisfaction regardless of the hearing outcome. Patients who regained a social hearing level expressed greater satisfaction than patients without serviceable hearing. Overall, quality of life improvement was not related to hearing improvement.
Sudden sensorineural hearing loss is devastating. Considering the audiological effects alone ignores the ‘human’ perspective. Audiological success can correlate with poor quality of life outcome.
Sudden sensorineural hearing loss is a true audiological emergency, and its management is much discussed. Currently, no single therapy has been proven effective according to evidence criteria. Recently, intratympanic application of steroids has been increasingly used in refractory cases; however, it has only rarely been reported as first-line therapy.
Materials and methods:
Twenty consecutive patients with sudden sensorineural hearing loss treated between July 2008 and January 2010 were enrolled in this prospective, case–control study. Ten patients were treated with intratympanic steroids and 10 with systemic ‘shotgun’ therapy (including steroids, pentoxifylline, low molecular weight heparin and vitamin E). The two groups were homogeneous in all respects. Pure tone averages were assessed before and after treatment for both groups.
There were no statistically significant differences between the two groups.
Intratympanic steroids seem to offer a valid alternative to systemic therapy, with few risks, in sudden sensorineural hearing loss patients, and we recommend their use as first-line therapy.
To report our experience with endoscopic, endonasal management of sinonasal haemangiopericytoma.
Materials and methods:
Retrospective review of the medical records of 10 patients undergoing endoscopic, endonasal surgery for sinonasal haemangiopericytoma of the nose and paranasal sinuses, between 1997 and 2008.
Five men and five women were included. Their mean age at surgery was 59 years. All patients underwent endoscopic, endonasal resection of their tumour. Major post-operative complications were encountered in only one patient (stroke). Local recurrence was diagnosed in only one patient (10 per cent), who subsequently underwent a combined resection (endoscopic and external) with orbital exenteration.
Sinonasal haemangiopericytomas are rare tumours that are usually benign. The mainstay of treatment is wide surgical excision with free resection margins. Nowadays, the great majority of patients can be treated using a purely endoscopic, endonasal approach.
Inner-ear malformations are frequently found in patients with sensorineural hearing loss. However, isolated anomalies of the vestibular part of the inner ear are seldom described, and for this reason their impact on balance is poorly understood.
We present the case of a 38-year-old Caucasian man with recurrent vestibular complaints, with a sensation of linear tilting, but no hearing impairment. Clinical and neuro-otological examinations showed peripheral involvement of the vestibular system, while audiological investigation was within normal limits. High-resolution magnetic resonance imaging of the inner ear, with three-dimensional reconstruction, demonstrated isolated vestibular anomalies involving both the lateral semicircular canal and the utricle.
Bearing in mind this case, we speculate that isolated vestibular malformation may not be as rare as previously thought, and should be investigated with the aid of sophisticated imaging techniques. A review of the relative literature, focussing attention on the molecular aspects, is also reported.
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