Bilateral cavernous sinus and ophthalmic vein thrombosis secondary to Trueperella pyogenes rhinosinusitis.

Ophthalmic vein thrombosis is a severe clinical entity with proptosis, eyelid swelling, orbital pain and reduction of visual acuity; its incidence is rare with 3-4 cases /million /year. Clinical manifestations result from venous congestion caused by septic (orbital cellulitis) or aseptic aetiologies (coagulopathies, trauma) and in some cases it could be associated with cavernous sinus thrombosis. In this paper, we describe a case report unique in the literature, of bilateral cavernous sinus and ophthalmic veins thrombosis due to both septic and aseptic causes characterized by unilateral sphenoid sinusitis sustained by Trueperella pyogenes infection. Trueperella pyogenes is an opportunistic animal pathogen, and its infections occur in both domestic and wild animals worldwide but are rare in humans; this is the first instance of human infection in the head and neck with an unknown hypercoagulable state.

Metastases to the parotid glands: An institutional experience.

BACKGROUND: Metastatic lesions to the salivary gland are rare and mostly affect the parotids. Metastases represent 8% of all malignant lesions of the parotid gland. Around 80% originate from squamous cell carcinomas (SCC) of the head and neck region. Fine needle aspiration (FNA) plays a crucial role in distinguishing primary salivary gland lesions from metastases. Herein we describe our series of metastases to the parotid glands. MATERIALS AND METHODS: We analysed 630 parotid gland FNAs over a decade including conventional and liquid-based cytology specimens. Ancillary techniques such as immunocytochemistry (ICC) were conducted on cell blocks. RESULTS: Eighty (12.4%) cases were malignant lesions, of which 53 (63.75%) were metastases including 24% melanoma, 22.6% SCC, 19% renal carcinomas, 7.5% breast carcinomas, 11.3% lung, 9% intestinal and 1.8% testicular, malignant solitary fibrous tumour and Merkel cell carcinoma. The 53 cases, classified according to the Milan system for salivary cytopathology, belonged to 5 Suspicious for malignancy (SFM) and 48 malignant (M) categories. Forty had a known history of primary malignancy (75.4%), while 13 were suspicious to be a metastatic localisation (24.5%), distributed as 5SFM (2SCC and 3Melanoma) and 8 M. A combination of clinical history, cytomorphology and ICC identified 100% of them. CONCLUSIONS: Fine needle aspiration plays a central role in the diagnostic workup of patients with metastatic lesions to their parotid glands, thereby defining the correct management. Diagnostic accuracy may be enhanced by applying ICC. Although melanoma and SCC are the most common histological types, several other malignancies may also metastasize to the parotid glands and should be kept into consideration.

A Potential Partnership between Genetics and the Oral Microbiome in Children Displaying Periodic Fever/Aphthosis/Pharyngitis/Adenitis Syndrome.

Periodic fever/aphthosis/pharyngitis/adenitis (PFAPA) syndrome was initially described in a small cohort of American children [...].

Combined treatment of muscle tension dysphonia: voice therapy with instrumental postural rehabilitation.

Objectives: This investigation aimed to propose a new rehabilitation technique that combines voice exercises and instrumental postural rehabilitation for patients with muscle tension dysphonia (MTD). Methods: We enrolled nine dysphonic patients (8 women and 1 man, aged 22-55 years). Voice assessment included strobovideolaryngoscopy, Maximum Phonation Time (MPT), perceptual evaluation by GRBAS scale and patient's self-rating by Italian version of the Voice Handicap Index (VHI). Vestibular function was evaluated by the Bed Side Examination and Video Head Impulse test (VHIT). Postural control was evaluated by Dynamic Posturography (DP) using the Sensory Organization Test (SOT) and analysing the Equilibrium Score (ES) and balance subsystems (somatosensorial, visual, vestibular). Results: All cases underwent different types of voice exercises combined with balance training based on NeuroCom Balance Master Protocols, once a week for six 35-minutes sessions. After therapy, an improvement in MPT, VHI, GRBAS scores and endoscopic laryngeal features was obtained. DP results at baseline were normal and after therapy we showed a slight improvement of ES (somatosensorial and visual components). Conclusions: A combined rehabilitation technique for MTD, by improving the attention to postural control, allows for significant improvement in vocal symptoms.

Pediatric oropharyngeal microbiome: Mapping in chronic tonsillitis and tonsillar hypertrophy.

OBJECTIVES: Aim of our study was to map the adenotonsillar lymphoid tissues' microbiome identifying its potential etiopathogenetic role in children affected by chronic tonsillitis or tonsillar hypertrophy with Obstructive Sleep Apnea Syndrome (OSAS). METHODS: In our study, we examined tonsillar swabs from healthy children and children affected by chronic tonsillitis or by tonsillar hypertrophy with Obstructive Sleep Apnea Syndrome (OSAS). Microbiome's analysis was performed and bacterial 16Sr RNA gene was sequenced according to metagenomic principles. Variability was described according to the biodiversity concept, indicating species found in a certain environment and changes they undergo adapting to different environmental conditions. RESULTS: The most significant differences concern variation of microbes in a single sample (alpha diversity) of some phyla in children affected by chronic tonsillitis compared with alpha diversity in healthy children and in children affected by OSAS with tonsillar hyperplasia. Proteobacteria are prevalent in chronic tonsillitis group, Fusobacteria and Spirochete in OSAS and Firmicutes, Actinobacteria, and Bacteroidetes were found in healthy children. Finally, comparison between the groups showed that children with OSAS with tonsillar hypertrophy had a higher presence of the Fusobacterium genus. CONCLUSION: Recurrent upper airway inflammatory and/or infectious processes are polymicrobial; chronicity of such processes appear to be related to variations in microbiome's composition and interaction among various taxonomic units. Knowledge of the microbiomes' composition together with traditional clinical biomarkers can also determine relationships between oropharyngeal microbiome and systemic pathologies to determine preventive changes in lifestyle, eating habits, environmental exposure and use of probiotics.

Biofilm's Role in Chronic Cholesteatomatous Otitis Media: A Pilot Study.

Cholesteatoma is a destructive lesion involving the temporal bone, which may induce severe complications due to its expansion and erosion of adjacent structures. Bacterial biofilm plays a crucial role in the pathogenesis of many otolaryngologic inflammatory/infectious chronic diseases. In this pilot study, we investigated, by means of cultural examination and with scanning electron microscope, the presence of bacterial biofilm in a series of samples from the epitympanic and mastoid region in patients affected by cholesteatoma and from the promontory region in patients with healthy mucosa who were undergoing to stapes surgery. The preliminary data support the association between biofilm and cholesteatoma (81.3% of the cases) and allow us to hypothesize that keratinized matrix of cholesteatoma may represent the ideal substrate for biofilm colonization and survival; this finding is consistent with the clinical course of aural cholesteatoma, characterized by recurrent exacerbations and recalcitrant course.

Chrono-proteomics of human saliva: variations of the salivary proteome during human development.

An important contribution to the variability of any proteome is given by the time dimension that should be carefully considered to define physiological modifications. To this purpose, whole saliva proteome was investigated in a wide age range. Whole saliva was collected from 17 preterm newborns with a postconceptional age at birth of 178-217 days. In these subjects sample collection was performed serially starting immediately after birth and within about 1 year follow-up, gathering a total of 111 specimens. Furthermore, whole saliva was collected from 182 subjects aged between 0 and 17 years and from 23 adults aged between 27 and 57 years. The naturally occurring intact salivary proteome of the 316 samples was analyzed by low- and high-resolution HPLC-ESI-MS platforms. Proteins peculiar of the adults appeared in saliva with different time courses during human development. Acidic proline-rich proteins encoded by PRH2 locus and glycosylated basic proline-rich proteins encoded by PRB3 locus appeared following 180 days of postconceptional age, followed at 7 months (±2 weeks) by histatin 1, statherin, and P-B peptide. The other histatins and acidic proline-rich proteins encoded by PRH1 locus appeared in whole saliva of babies from 1 to 3 weeks after the normal term of delivery, S-type cystatins appeared at 1 year (±3 months), and basic proline-rich proteins appeared at 4 years (±1 year) of age. All of the proteinases involved in the maturation of salivary proteins were more active in preterm than in at-term newborns, on the basis of the truncated forms detected. The activity of the Fam20C kinase, involved in the phosphorylation of various proteins, started around 180 days of postconceptional age, slowly increased reaching values comparable to adults at about 2 years (±6 months) of age. Instead, MAPK14 involved in the phosphorylation of S100A9 was fully active since birth also in preterm newborns.

Vocal fold nodules in school age children: attention deficit hyperactivity disorder as a potential risk factor.

OBJECTIVE: To evaluate the presence of symptoms of inattention and hyperactivity/impulsivity in a population of school age children affected by vocal fold nodules. METHODS: Parents and teachers of 18 children with vocal fold nodules (10 males, eight females; aged between 6 and 12 years) and 20 matched controls without dysphonia and/or vocal fold diseases (11 males, nine females; aged between 6 and 12 years) completed Attention-Deficit/Hyperactivity Disorder (ADHD) rating scale for parents (SDAG [Scala per i Disturbi di Attenzione/Iperattività per Genitori]) and teachers (SDAI [Scala per i Disturbi di Attenzione/Iperattività per Insegnanti) rating scales containing in two subscales items that specifically evaluate the symptoms of ADHD according to the DSM-IV. All children were subjected to videolaryngoscopy. RESULTS: The group with vocal fold nodules scored significantly higher than the controls; the difference between the two groups was statistically significant for both the subscales of both questionnaires (SDAG and SDAI) (P < 0.05). Four children in the group with vocal fold nodules who scored higher than 14 in at least one subscale were referred for psychiatric evaluation. For two of the children, both male, a diagnosis of combined ADHD was formulated. CONCLUSIONS: ADHD is a possible risk factor for the development of vocal fold nodules in childhood. SDAG and SDAI rating scales may supplement the diagnostic assessment of children with vocal fold nodules.

Vestibular evoked myogenic potentials in children affected by myelomeningocele.

PURPOSE: The aim of the present study is to establish if the vestibular evoked myogenic potentials (VEMPs) could be used as a clinical test for the evaluation of vestibular function in children affected by myelomeningocele (MMC). MATERIALS AND METHODS: Fifteen children, aged between 3 and 17 years, who had been affected by MMC were investigated. Data obtained from these children were compared with normal data from healthy children of the same age. Electromyographic activity of sternocleidomastoid muscle was recorded, while children were laid supine and asked to raise their head off the bed in order to activate their neck flexors bilaterally. The saccular receptors were acoustically stimulated with a logon of 500 Hz at an intensity of 130 dB peSPL presented monaurally through earphones. In each recording, we analyzed latencies and amplitudes of the p13-and n23 waves and the amplitude ratio between the two ears. RESULTS: VEMPs were detected to be normal in 13 patients. In particular, the mean p13 and mean n23 latencies were 15.7 (±1.4) and 21.7 (±1.1) ms, respectively; the mean amplitude value was 84.7 (±36.6), while the mean amplitude ratio was 17.4 (±12). A comparison of latencies and amplitude ratios between the children and healthy control group did not reveal any significant difference. On the contrary, a comparison of amplitude values between the two groups showed significant differences. CONCLUSION: In conclusion, vestibulocollic reflex is normal in patients affected by MMC, and VEMPs could represent a valid and noninvasive technique eligible to investigate the vestibular functions in these children.

Cephalometric findings in facioscapulohumeral muscular dystrophy patients with obstructive sleep apneas.

PURPOSES: The purposes of the study are: (1) to establish if cephalometry and upper airway examination may provide tools for detecting facioscapulohumeral (FSHD) patients at risk for obstructive sleep apnea syndrome (OSAS); and (2) to correlate cephalometry and otorhinolaryngologic evaluation with clinical and polysomnographic features of FHSD patients with OSAS. METHODS: Patients were 13 adults affected by genetically confirmed FSHD and OSAS, 11 men, with mean age 47.1 ± 12.8 years (range, 33-72 years). All underwent clinical evaluation, Manual Muscle Test, Clinical Severity Scale for FSHD, Epworth Sleepiness Scale, polysomnography, otorhinolaryngologic evaluation, and cephalometry. RESULTS: Cephalometric evidence of pharyngeal narrowing [posterior airways space (PAS) < 10 mm] was present in only one patient. The mandibular planus and hyoid (MP-H) distance ranged from 6.5 to 33.1 mm (mean, 17.5 ± 7.8 mm). The mean length of soft palate (PNS-P) was 31.9 ± 4.8 mm (range, 22.2 to 39.7 mm). No patient presented an ANB angle > 7°. There was no significant correlation between cephalometric measures, clinical scores, and PSG indexes. PAS and MP-H were not related to the severity of the disease. CONCLUSIONS: Upper airway morphological evaluation is of poor utility in the clinical assessment of FSHD patients and do not allow to predict the occurrence of sleep-related upper airway obstruction. This suggests that the pathogenesis of OSAS in FSHD is dependent on the muscular impairment, rather than to the anatomy of upper airways.

How relevant is the impairment of smell for the quality of life in allergic rhinitis?

PURPOSE OF REVIEW: In the present review, the authors try to evaluate how relevant smell impairment is in patients suffering from allergic rhinitis and how it affects their quality of life. Smell dysfunction has a significant impact on the quality of life as it can lead to a wrong choice of food and intake, a reduction in appetite and eventually to weight loss, malnutrition, immunity reduction and worsening of medical illness. Patients with smell impairment are reported to use larger quantities of sugar and salt to highlight flavours, thus worsening their general health condition and increasing the risk of developing diabetes and hypertension. RECENT FINDINGS: Recent studies estimate that a complete loss of the sense of smell can be found in at least 1% of the US population, and that an impairment in the olfactory function can be highlighted in about 24% of individuals aged 53-97 years and 19% of individuals aged 20-92 years. Despite the high prevalence, subjective complaints do not accurately reflect the real disturbance experienced by the patient, and usually go unnoticed. SUMMARY: Current information in literature highlights the need for additional studies that concentrate on the impact of olfactory dysfunction on the quality of life of patients affected by allergic rhinitis.

Rhino sinusal bilateral hamartoma: a case report.

The hamartoma is a benign rare tumor constituted by a mixture of tissue. It is very unusual in the nasal cavity. The objective of the study is to describe an unusual case of bilateral nasal hamartoma. We report a 52-year-old male patient with a bilateral paranasal hamartoma of the ethmoid and maxillary sinus. Functional endoscopic sinus surgery was performed to completely remove the masses. The reported localization is unusual because the most common site in the nose is the posterior septum. Although hamartoma arising from the rhino sinusal region is very rare, head and neck surgeons must know this entity in order to differentiate it from inverted papilloma and adenocarcinoma. Misinterpretation of this lesion may result in aggressive surgery for a benign lesion.

Trafficking and postsecretory events responsible for the formation of secreted human salivary peptides: a proteomics approach.

To elucidate the localization of post-translational modifications of different classes of human salivary proteins and peptides (acidic and basic proline-rich proteins (PRPs), Histatins, Statherin, P-B peptide, and "S type" Cystatins) a comparative reversed phase HPLC-ESI-MS analysis on intact proteins of enriched granule preparations from parotid and submandibular glands as well as parotid, submandibular/sublingual (Sm/Sl), and whole saliva was performed. The main results of this study indicate the following. (i) Phosphorylation of all salivary peptides, sulfation of Histatin 1, proteolytic cleavages of acidic and precursor basic PRPs occur before granule storage. (ii) In agreement with previous studies, basic PRPs are secreted by the parotid gland only, whereas all isoforms of acidic PRPs (aPRPs) are secreted by both parotid and Sm/Sl glands. (iii) Phosphorylation levels of aPRPs, Histatin 1, and Statherin are higher in the parotid gland, whereas the extent of cleavage of aPRP is higher in Sm/Sl glands. (iv) O-Sulfation of tyrosines of Histatin 1 is a post-translational modification specific for the submandibular gland. (v) The concentration of Histatin 3, Histatin 5, and Histatin 6, but not Histatin 1, is higher in parotid saliva. (vi) Histatin 3 is submitted to the first proteolytic cleavage (generating Histatins 6 and 5) during granule maturation, and it occurs to the same relative extent in both glands. (vii) The proteolytic cleavages of Histatin 5 and 6, generating a cascade of Histatin 3 fragments, take place after granule secretion and are more extensive in parotid secretion. (viii) Basic PRPs are cleaved in the oral cavity by unknown peptidases, generating various small proline-rich peptides. (ix) C-terminal removal from Statherin is more extensive in parotid saliva. (x) P-B peptide is secreted by both glands, and its relative quantity is higher in submandibular/sublingual secretion. (xi) In agreement with previous studies, S type Cystatins are mainly the product of Sm/Sl glands.

Damage to ciliated epithelium in chronic rhinosinusitis: what is the role of bacterial biofilms?

OBJECTIVES: We assess the association between the presence of biofilms and cilial damage in patients with chronic rhinosinusitis (CRS), describe the microorganisms associated with samples that exhibited cilial loss and biofilms, and demonstrate the absence of ciliary injury and biofilms in similarly prepared "normal" controls. METHODS: We examined samples of ethmoid mucosa obtained from 24 patients who underwent functional endoscopic sinus surgery for CRS. Samples from a control group (20 healthy subjects) were also examined. The specimens were divided into 2 fragments; the first was processed for bacterial cultures, and the second was subjected to scanning electron microscopy. Statistical analysis was performed. RESULTS: All CRS samples had positive bacterial cultures. The scanning electron microscopy analysis showed bacterial biofilms in 10 of the 24 specimens. A marked destruction of the epithelium was observed in samples positive for biofilms (p < 0.001), and the presence of Haemophilus influenzae was associated with ciliary abnormalities (partial damage in 55.6% and absence of cilia in 50%; p = 0.041). CONCLUSIONS: The high percentage of biofilms in our specimens confirms the association between biofilms and CRS. Our data support the hypothesis that biofilm formation represents the latter phase of an inflammatory process that leads to complete epithelial destruction.

Vestibular evoked myogenic potentials in children.

OBJECTIVE: The aim of this work is to establish if the vestibular evoked myogenic potentials (VEMPs) could be used as a clinical test of vestibular function in children. MATERIALS AND METHODS: Forty normal hearing children, aged between 3 and 15 years, and classified in preschool and scholar group, have been investigated in order to study normal development of vestibular potentials and to define fundamental parameters of VEMPs, establishing normal data of latencies and amplitude ratio. Electromyographic activity of sternocleidomastoid muscle was recorded while children were laid supine on a bed and asked to raise their head off of the bed in order to activate their neck flexors bilaterally. The saccular receptors were acoustically stimulated with a logon of 500Hz at an intensity of 130dB peSPL presented monaurally through earphones. In each recording, we analysed latencies and amplitudes of the p13 and n23 waves and the amplitude ratio between the two ears. RESULTS: VEMPs were normally detected in all subjects. In preschool group mean p13 and mean n23 latencies were, respectively, 16.13 (+/-2.12)ms and 21.17 (+/-2.77)ms; mean amplitude ratio was 28.49 (+/-18.10). In scholar group mean p13 and n23 were respectively 16.14 (+/-3.48)ms and 21.78 (+/-3.39)ms, while mean amplitude ratio 20.44 (+/-13.24). Comparison of latencies and amplitude ratio between the children groups and control adult group did not showed any significant differences. CONCLUSION: In conclusion, VEMPs could represent a valid and non-invasive technique able to investigate vestibular function in children and, in particular, vestibulo collic reflex.

Wegener's granulomatosis: a challenging disease for otorhinolaryngologists.

Diagnosis of Wegener's granulomatosis (WG) can be delayed because of its aspecific presenting symptoms. Detection of serum circulating antineutrophil cytoplasm antibodies (c-ANCAs), in combination with histology, permits one to identify WG at an early stage and to implement stage-adapted therapy. c-ANCA levels may also help to evaluate the response to medical therapy. Recently, the quality of life of WG patients has been improved by administering cotrimoxazole in order to prevent infections and recurrent diseases during the remission period.WG is of special significance to the otorhinolaryngologist because it is often initially limited to the upper respiratory tract before becoming systemic. The aim of this paper was to describe a series of WG patients and underline the difficulties involved in diagnosing and treating this challenging disease. This was a prospective study in 23 consecutive patients with head and neck manifestations of WG (17 systemic, 6 limited). Diagnosis was performed by means of both c-ANCAs detection using indirect immunofluorescence and histology in biopsy specimens. Treatment consisted of daily cyclophosphamide (CYC; 2 mg/kg/day) and glucocorticoids (prednisone; 1 mg/kg/day). If an improvement or toxic events occurred, CYC was discontinued and methotrexate was started. If, during remission of the disease, low serum c-ANCAs levels were detected, CYC was suspended and cotrimoxazole (1 g/day) was introduced. Serum c-ANCAs detection was positive for all patients. Biopsy was diagnostic from the beginning in 19/23 cases. The six patients with limited WG did not show a progression to systemic disease. Only 3 patients with a diagnosis of delayed systemic WG died, whereas 19/23 patients were alive with good control of relapses.