Report from a Consensus Conference on the treatment of Ménière's disease with betahistine: rationale, methodology and results.

Ménière's disease is a disorder of the inner ear that causes vertigo, tinnitus, fullness and hearing loss. Although several treatments are available, the success rate is reported to be around 70%, similar to placebo. Betahistine, a weak H1 receptor agonist and an effective H3 receptor antagonist, is frequently prescribed for Ménière's disease, especially to reduce recurrent vertigo attacks. The effects of this drug on hearing and other audiological symptoms remains unclear. Given the inconclusive reports in the literature, we proposed a consensus conference on the use of betahistine in Ménière's disease. The aim was to define best practice criteria for therapy for Ménière's disease, improve clinical suitability and reduce heterogeneity of the therapeutic approach. The consensus conference on betahistine for Ménière's disease involved a group of Italian experts in vestibular disorders who were asked a series of questions prepared by opinion leaders. The Delphi method, an iterative investigation method, was used to increase consensus. Via a tele-voting system, each participant anonymously evaluated all statements using a Likert 5-point scale. Betahistine was considered useful for the treatment of dizziness and vertigo during the intercritical phase of the disease (87% agreeing answers). However, during the acute phase of the disease betahistine was considered less effective and useful only when associated with other drugs (71% agreement). Similarly, the efficacy of the drug was considered low when used to reduce progressive hearing loss, tinnitus, and ear fullness. The experts advocated the use of betahistine during the intercritical phase of Ménière's disease to reduce the number and severity of vertigo attacks. Its use seems to be at low risk of major side effects.

Paroxysmal positional vertigo despite complete vestibular impairment: the role of instrumental assessment.

Lindsay-Hemenway syndrome is characterised by a posterior canal benign paroxysmal positional vertigo following a partial unilateral vestibular loss affecting the same side. The syndrome is caused by damage of structures innervated by the superior division of the vestibular nerve and perfused by the anterior vestibular artery; the detached otoconia can cause vertigo in the still intact posterior semicircular canal. The most recent vestibular instrumental techniques allow reaching an accurate topodiagnosis in case of peripheral vestibular failure. We report on two cases of Lindsay-Hemenway syndrome despite complete vestibular failure demonstrated by vestibular instrumental assessment. After making some critical considerations on these findings, we underline the importance of not disregarding the diagnosis of paroxysmal positional vertigo in an established complete labyrinthine loss of function.

Italian survey on benign paroxysmal positional vertigo.

Benign paroxysmal positional vertigo (BPPV) is the most common type of peripheral vertigo. BPPV often relapses after the first episode, with a recurrence rate between 15% and 50%. To date both the aetiopathogenetic processes that lead to otoconia detachment and the factors that make BPPV a relapsing disease are still unclear, but recent epidemiological studies have shown a possible association with cardiovascular risk factors. The aim of the present study (Sesto Senso Survey) was to evaluate in the Italian population through an observational survey, the main demographic and clinical characteristics of patients with BPPV (first episode or recurrent) with particular focus on the potential cardiovascular risk factors. The survey was conducted in 158 vestibology centres across Italy on 2,682 patients (mean age 59.3 ± 15.0 years; 39.1% males and 60.9% females) suffering from BPPV, from January 2013 to December 2014. The results showed a high prevalence of cardiovascular risk factors such as high blood pressure (55.8%), hypercholesterolaemia (38.6%) and diabetes (17.7%), as well as a family history of cardiovascular disease (49.4%). A high percentage of patients also had hearing loss (42.9%), tinnitus (41.2%), or both (26.8%). The presence of hypertension, dyslipidaemia and pre-existing cardiovascular comorbidities were significantly related to recurrent BPPV episodes (OR range between 1.84 and 2.31). In addition, the association with diabetes and thyroid/autoimmune disease (OR range between 1.73 and 1.89) was relevant. The survey results confirm the significant association between cardiovascular comorbidities and recurrent BPPV and identify them as a potential important risk factor for recurrence of BPPV in the Italian population, paving the way for the evaluation of new therapeutic strategies in the treatment of this disease.

Surgical complications in orbital decompression for Graves' orbitopathy.

The objective of this study is to analyse the complications of orbital decompression in Graves' orbitopathy. The clinical records of 946 patients who had been operated on with orbital decompression for Graves' orbitopathy were reviewed and the intra- and post-operative complications with minimum follow-up of six months were analysed. An extensive review of the literature was carried out to compare results. In the case-series reported here the most frequent complications were: wasting of the temporal region (100%) in patients operated on using a coronal approach; permanent hypoesthesia of V2 (13%) and V1 (8%) in patients operated on with an upper eyelid incision. In only one patient was a total monolateral lesion of V2 reported. The most severe complications consisted in reduction of visual acuity in 5 patients, and CSF leak with cerebral complications in 2 patients, who were operated on with a non-endoscopic endonasal approach. Three patients had intra-operative haemorrhages and 3 patients had post-operative haemorrhages requiring further surgical intervention. The incidence of symptomatic sinusitis/mucoceles was 0.75%. In conclusion, orbital decompression carried out with endoscopic endonasal technique and via transpalpebral accesses appears to be associated with a low incidence of complications. Knowledge of the causes of the possible complications in the different surgical approaches can definitely help to reduce their incidence.

Isolated laryngeal leishmaniasis in an immunocompetent patient: a case report.

Isolated laryngeal leishmaniasis in an immunocompetent patient: a case report. OBJECTIVE: Isolated laryngeal Leishmaniasis presents neither explicit laryngeal lesions nor specific symptoms. In fact, it may mimic many inflammatory and neoplastic diseases. Considering the low incidence of this atypical localization, laryngeal Leishmaniasis, is rarely contemplated by physicians in differential diagnoses of laryngeal tumour lesions. We present the case of a 62-year-old immunocompetent subject who developed a single Leishmania mucosal lesion on the left vocal cord, simulating a laryngeal cancer. METHODS: A case report and discussion of the clinical case by referring to the literature. RESULTS: Specific therapy with miltefosine led to clinical and endoscopic improvement. We had no relapse after three years of follow-up. CONCLUSIONS: This clinical case highlights the need to consider isolated laryngeal Leishmaniasis in the differential diagnoses of laryngeal tumour lesions, even in immunocompetent subjects who live in endemic zones, in order to ensure an early and correct therapeutic approach.

Vertigo in childhood: proposal for a diagnostic algorithm based upon clinical experience.

The aim of this paper is to analyse, after clinical experience with a series of patients with established diagnoses and review of the literature, all relevant anamnestic features in order to build a simple diagnostic algorithm for vertigo in childhood. This study is a retrospective chart review. A series of 37 children underwent complete clinical and instrumental vestibular examination. Only neurological disorders or genetic diseases represented exclusion criteria. All diagnoses were reviewed after applying the most recent diagnostic guidelines. In our experience, the most common aetiology for dizziness is vestibular migraine (38%), followed by acute labyrinthitis/neuritis (16%) and somatoform vertigo (16%). Benign paroxysmal vertigo was diagnosed in 4 patients (11%) and paroxysmal torticollis was diagnosed in a 1-year-old child. In 8% (3 patients) of cases, the dizziness had a post-traumatic origin: 1 canalolithiasis of the posterior semicircular canal and 2 labyrinthine concussions, respectively. Menière's disease was diagnosed in 2 cases. A bilateral vestibular failure of unknown origin caused chronic dizziness in 1 patient. In conclusion, this algorithm could represent a good tool for guiding clinical suspicion to correct diagnostic assessment in dizzy children where no neurological findings are detectable. The algorithm has just a few simple steps, based mainly on two aspects to be investigated early: temporal features of vertigo and presence of hearing impairment. A different algorithm has been proposed for cases in which a traumatic origin is suspected.

Benign tumours affecting the deep lobe of the parotid gland: how to select the optimal surgical approach.

Many types of approaches allow extra-capsular dissection in the deep parotid parenchyma in the treatment of benign tumours. A transcervical approach (TCA), transparotid approach (TPA) and a combined transcervical-transparotid approach (TPTCA) are the three main procedures performed to expose the deep parenchyma. We conducted a retrospective chart review enrolling 24 consecutive patients treated for benign tumours affecting the deep lobe of the parotid. Review of the surgical data was accompanied by careful follow-up to establish surgical morbidity, functional (Frey's Syndrome and first-bite syndrome) and aesthetical outcomes. A TPA was performed in the majority of cases; in 26% superficial parotidectomy was not required (selective deep parotidectomy). Minor's test showed a low rate of Frey's syndrome (3 cases of 23, 13%). No long-lasting first-bite syndrome was reported. Some additional procedures were easily performed in order to improve aesthetical results (rotational flap of sternocleidomastoid muscle, free abdominal fat transfer); these had the same results as selective deep parotidectomy. TCA (or TPTCA) ensures the best control of the facial nerve, providing good exposure and good functional and aesthetical results (without sparing the superficial parenchyma if additional techniques are performed with the aim of reducing skin depression in the treated area). The choice of the approach should have only the aim of safe resection and should not be influenced by aesthetical outcome; the craniocaudal level of the tumour seems to be the best indicator of the feasibility of the procedure also considering the branches of the facial nerve. In our experience, mandibulotomy can always be avoided.

Long-term follow up of sudden sensorineural hearing loss patients treated with intratympanic steroids: audiological and quality of life evaluation.

OBJECTIVE: To evaluate the long-term stability of intratympanic steroids and investigate the 'real' impact of sudden sensorineural hearing loss on patients. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

Intratympanic methylprednisolone as first-line therapy in sudden sensorineural hearing loss: preliminary results from a case-control series.

BACKGROUND: 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. RESULTS: There were no statistically significant differences between the two groups. CONCLUSION: 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.

A rare case of primary systemic amyloidosis of the neck with massive cervical lymph node involvement: a case report and review of the literature.

Amyloidosis is a term applied to a diverse group of disorders that share the deposition of amyloid protein in various extracellular tissues. Systemic amyloidosis may involve almost any organ system in the body including regions in the head and neck; however, neck lymph node involvement is rare, with only five previous cases reported. We present the case of a primary systemic AL amyloidosis with hepatic, cervical, retroperitoneal, axillary and inguinal lymphnode localizations, unresponsive to medical therapy and treated with a surgical approach followed by autologous bone marrow transplantation. We review the pertinent literature with exclusive attention to the otorhinolaryngologic aspect.

Correlations between transversal discrepancies of the upper maxilla and oral breathing.

AIM: The aim of the study was to evaluate the relationship between malocclusion with cross-bite and permeability of the upper airways, and to observe the cephalometric changes of the rhinopharyngeal space after rapid palatal expansion therapy. MATERIALS AND METHODS: The sample consisted of 17 patients (age 9-12) of which 10 were males, followed up for a period of 1 year at the Department of Paediatric Dentistry of the Dental Clinic of the University of Pisa. Clinical, radiographical and otolaryngological examinations were carried out before treatment with palatal expansor. After expansor activation, each patient underwent an otolaryngological and orthodontic evaluation followed by rhinomanometry, and, in the cooperating children, endoscopy was also performed. After 6 and 12 months from the beginning of the treatment, each patient was examined again and the radiographic examination was repeated. RESULTS AND CONCLUSION: The cephalometric analysis exhibited an increase of the rhinopharyngeal space in 16 children. Furthermore all the 17 patients showed, after therapy, an increase of the transverse dimension of the upper jaw, measured on the postero-anterior teleradiography. On the other hand, the otolaryngological examination, and in particular rhinomanometry, exhibited an improvement of the flow and of right and left nasal resistance only in 3 children, while in 6 children the graph remained unchanged, and in 8 children it worsened. The results show that the rapid palatal expansion produces an improvement of the transversal skeletal discrepancy, and an improvement of the permeability of the upper airways. To make a correct diagnosis lateral and postero-anterior teleradiography, and a cephalometric analysis are needed; instead the otolaryngological examination in our opinion it is not an essential diagnostic examination for this kind of pathology.

Frontal mucocoele communicating with an arachnoid cyst of the anterior cranial fossa.

Mucocoeles usually involve the frontal sinus and can extend to the orbit or intracranially. In this case symptoms and radiological findings were typical of a left frontal mucocoele with intracranial extension. Intraoperative findings were compatible with a left frontal mucocoele communicating with an arachnoid cyst of the anterior cranial fossa.

Bilateral, isolated, lateral semicircular canal malformation without hearing loss.

HYPOTHESIS: 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. CARE REPORT: 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. CONCLUSIONS: 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.

Drop attacks and vertical vertigo after transtympanic gentamicin: diagnosis and management.

Drop attacks represent a significant problem during the natural course of Meniere's disease. They are characterized by a sudden fall to the ground without loss of consciousness. Diagnosis is clinical and based on the typical description of the patient. Involvement of vertical canal is possible during Meniere's disease and also after gentamicin application. Treatment of drop attacks is still a matter of discussion; most cases have a benign course with spontaneous remission and no treatment is necessary. In severe cases, aggressive treatment (surgical or pharmacological) is necessary. A case of drop attack associated with vertical vertigo is presented. Vestibular tests were performed in order to assess the involvement of inner ear. Caloric test and ice water test reveal no response. Vestibular Evoked Myogenic Potentials are present even after high doses of gentamicin. Drop attacks and vertical vertigo can occur after transtympanic gentamicin and can be well managed with high doses of local gentamicin.

Cranio-cervical necrotizing fascitiis: case report and review of the literature.

Necrotising fascitiis is a rapidly progressive bacterial infection of the soft tissues and generally attacks the walls of the abdomen, the perineum, the limbs or, to a lesser degree, the cranio-cervical area. In the latter region, the infection involves the soft tissues of the neck, in a more or less extensive manner, and causes diffuse necrosis. Crepitation, areas with linear infiltration and others with fluctuation are detected on manual examination. Systemic symptoms such as fever, tachycardia, tachypnoea and signs of septic shock are always present, at least during the more advanced stages of the disease. Computed tomography may prove fundamental since it reveals an increase in the thickness and degree of impregnation of the cervical soft tissues, as well as the presence of liquid or gaseous infiltration in the thoracic areas, especially in cases of mediastinitis. Personal experience in a case is described which led to a review of the literature. The best approach in the management of this devastating condition is early diagnosis, adequate antibiotic treatment and radical surgical procedures, which may often need to be repeated several times.

[Intratympanic gentamycin in association with human fibrin glue in the treatment of Meniere's disease: preliminary results]. / Gentamicina intratimpanica in associazione con colla di fibrina umana nel trattamento della malattia di Menière: risultati preliminari.

The treatment of Menierè's disease (Md) with intratympanic gentamicin has rapidly become one of the most widespread alternatives to surgery in this disorder. Numerous studies, employing different protocols, have reported the use of this antibiotic in the treatment of disabling forms of Md, with success rates in the control of vestibular symptoms varying from 73 to 100%, associated with a rate of hearing complications varying from 0 to 75%. We have reported the results of a preliminary experience carried out in 10 patients affected by monolateral Md who were managed with ablation treatment effected with a mixture of gentamicin and human fibrin glue. Upon follow-up examination after one year, all of the patients presented a marked reduction in vestibular reflectivity on the side treated. The vertigo score was zero in all of the patients, showing that the vertigo symptoms were entirely under control. When patients were asked to rate their disability, vertigo was assessed as completely under control in 7 patients and substantially under control in the remaining 3. None of the patients presented any loss of hearing. On the basis of this experience, we propose a standardized protocol which, using an extremely low overall dose of gentamicin, enables elevated success rates to be obtained with the lowest possible number of injections, thus minimizing the risks to hearing. Intratympanic gentamicin associated with a fibrin carrier in Md appears to enable a standardized dose of the drug to be employed, resulting in a decided reduction in the number of administrations and in the overall dose of the drug applied. This makes it possible, with equal benefits in vertigo control, to significantly minimize any hearing loss.