Rosai–Dorfman disease in the submandibular salivary glands of a pediatric patient

Neck mass is a common presentation in the pediatric patient. A case is discussed of a boy complaining of submandibular salivary gland enlargement, eventually leading to compressive dysphagia. Excisional biopsy yielded diagnosis of sinus histiocystosis with massive lymphadenopathy (Rosai Dorfman disease). Clinical findings, histopathology, and management of this rare disease are reviewed.     

Sequential bilateral cochlear implantation in a patient with bilateral Ménière's disease

This case study describes a 45-yr-old female with bilateral, profound sensorineural hearing loss due to Ménière's disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient's ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Ménière's disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work.     

Cochlear implantation in a patient with sensori-neural deafness secondary to Charcot−Marie−Tooth disease

Abstract

Objective and importance

Charcot?Marie?Tooth (CMT) disease is the most common hereditary motor and sensory neuropathy and can result in profound sensori-neural hearing loss with deficiency in speech perception out of proportion to that which would be expected if the loss was cochlear in origin. This study investigates whether the reintroduction of auditory synchrony by means of cochlear implantation will improve speech perception in those with dys-synchrony related to impairment of temporal processing abilities secondary to CMT.

Clinical presentation

A 67-year-old male presented with a gradual but significant decrease in his hearing as part of a slowly progressing demyelinating peripheral neuropathy. On open-set speech discrimination he scored 0%.

Intervention

A Med-el Flex^SOFT cochlear implant (CI) was fully inserted into the left ear with no surgical complications. The CI speech processor was fitted 1 month post-implantation and standardized speech assessments conducted at 1 week, 3 months, 9 months, and 21 months following initial fitting, gave open-set speech discrimination scores of 0, 0, 53, and 54%, respectively.

Conclusion

This report demonstrates that cochlear implantation is an option to rehabilitate severe-to-profound hearing loss in adults with auditory dys-synchrony secondary to CMT disease. Progress post-implantation is likely to be slower than for the average CI user.     

Streptomycin perfusion of the labyrinth in the treatment of Menière's disease: a modified technique

The ideal surgical procedure for Menière's disease would combine the high rate of vertigo control and the good hearing preservation of vestibular nerve section with the low morbidity of labyrinthectomy. Shea's technique of streptomycin perfusion of the labyrinth has been modified by making an additional opening into the posterior semicircular canal in an effort to limit the amount of streptomycin going into the cochlea. Seventeen patients with definite Menière's with poor hearing have had this procedure. Vertigo was controlled in 94% and the hearing preserved in 55%. Vestibular rehabilitation was not a problem. It is speculated that hearing preservation would be better if the procedure were not restricted to those with poor hearing. This method of destruction of the vestibular system carries the possibility of hearing preservation and maintains the possibility of cochlear implantation should this ever be required.     

Parapharyngeal space tumor surgery using a modified cervical–parotid approach

Objectives: The cervical approach to parapharyngeal space tumors is blind in cases involving an approach to the vicinity of the skull base from below. We therefore devised a procedure to improve the safety of surgery in the vicinity of the skull base.

Methods: With this surgical technique, the temporal branch from the main trunk is first identified, as in the case of parotid gland tumor surgery. We then approach the skull base by entering the posterior aspect of the parotid gland tissue from above the temporal branch and anterior auricular space, allowing clear visual confirmation of the upper part of the tumor. Combining this approach with the conventional cervical approach, which is used for the lower part, the tumor can be separated from top to bottom.

Results: We have applied this combined approach in eight patients to date, and post-operative courses have been good, with no cases of facial paralysis.

Conclusion: This modified cervical–parotid approach offers a very useful surgical procedure for parapharyngeal space tumors with respect to preservation of the facial nerve and tumor separation in the vicinity of the skull base.     

Quality of life after gamma knife radiosurgery treatment in patients with a vestibular schwannoma: the patient’s perspective

This study evaluates the impact of gamma knife radiosurgery (GKRS) on the quality of life (QOL) of patients with a sporadic vestibular schwannoma (VS). This study pertains to 108 VS patients who had GKRS in the years 2003 through 2007. Two different QOL questionnaires were used: medical outcome study short form 36 (SF36) and Glasgow benefit inventory (GBI). Radiosurgery was performed using a Leksell 4C gamma knife. The results of the QOL questionnaires in relation to prospectively and retrospectively gathered data of the VS patients treated by GKRS. Eventually, 97 patients could be included in the study. Their mean tumor size was 17 mm (range 6–39 mm); the mean maximum dose on the tumor was 19.9 Gy (range 16–25.5 Gy) and the mean marginal dose on the tumor was 11.1 (range 9.3–12.5 Gy). SF36 scores showed results comparable to those for a normal Dutch population. GBI showed a marginal decline in QOL. No correlation was found between QOL and gender, age, tumor size, or radiation dose. Increased audiovestibular symptoms after GKRS were correlated with a decreased GBI score, and decreased symptoms were correlated with a higher QOL post-GKRS. In this study shows that GKRS for VS has little impact on the general QOL of the VS patient. However, there is a wide range in individual QOL results. Individual QOL was influenced by the audiovestibular symptoms. No predictive patient, tumor, or treatment factors for QOL outcome after GKRS could be determined. Comparison with microsurgery is difficult because of intra group variability.     

Is deafness a disease of poverty? The association between socio-economic deprivation and congenital hearing impairment

An association between congenital hearing impairment and deprivation has been suggested, but evidence is limited. We studied children born in Greater Glasgow, 1985-94, with bilateral congenital hearing impairment. The children were divided into seven deprivation categories using the Carstairs Deprivation Index. One hundred and twenty-four hearing-impaired children were born over the study period, an incidence of 1.18/1000 live births. There was a clear association between deprivation category and incidence, ranging from 0.47/1000 to 1.72/1000. An association with deprivation was seen for children with a family history and perinatal problems (such as prematurity and low birth weight). No association was found for other aetiological groups such as craniofacial syndromes or early postnatal infection. Deprivation had no effect on age of diagnosis or hearing aid provision. Deprivation is associated with congenital hearing impairment, due to more prematurity and low birth weight in deprived families, and the fact that families with many hearing-impaired members are economically disadvantaged. There should be a strong emphasis on the needs of the socio-economically disadvantaged when planning services for hearing-impaired children.     

VEMPs and dynamic posturography after intratympanic gentamycin in Menière's disease

In this study, we investigated whether vestibular evoked myogenic potentials (VEMPs) and dynamic posturography (DP) are useful in the evaluation of the vestibular function in patients affected by active monoaural Menière's disease (MD) treated with intratympanic gentamycin. Twelve subjects affected by monoaural MD were treated with weekly intratympanic injections of 0.5 ml of gentamycin at a concentration of 20 mg/ml. VEMPs were used to test saccular function, while postural control was analyzed by DP. The results obtained with these two techniques were compared with those obtained by using bithermal caloric test. The mean follow-up was 15.3 months (6--28 months). Therapy resulted in complete absence of the caloric response in six subjects (50) and in caloric test-induced asymmetry in the remaining individuals, ranging from 83 to 27. At follow-up, eleven patients (91.6%) were free of vertigo, while one patient had two vertigo spells 9 months after treatment. Before treatment, VEMPs were present in the affected ear of eleven patients. After treatment VEMPs were absent in all the patients. At the end of follow-up, reappearance of VEMPs was observed in two patients, with no changes in latency values and amplitude ratio. DP demonstrated a reduction of the Composite Score (CS) one week after therapy, with a prevalent reduction of the vestibular component. After 6 months, there was an improvement of the CS and, in particular, of the vestibular component. The present study demonstrates that bithermal caloric test and VEMPs allow for the functional evaluation of both the horizontal semicircular canal and the sacculus, suggesting that these techniques might be used together to monitor the efficacy of intratympanic gentamycin therapy. In addition, our data indicate that DP might provide important information on compensation phenomena and show that intratympanic gentamycin can improve postural control in MD patients.     

Tracheostomy: a must or elective procedure in laryngeal resection for cancer?

The paper outlines 15-year experience in anesthesia perfection in laryngeal resections with polymeric endoprosthesis (tube endoprosthesis EG 1-4, TU 42-2-467-85) made in 115 patients with laryngeal cancer stage III; presents the analysis of complications in the above resection depending on the time of postoperative and decanulation and anesthesiological procedure. The authors propose methods of shortening the time to decanulation and even to avoid tracheostomy in transcutaneous jet high-frequency artificial lung ventilation.     

Nasal brushing: a clinically useful procedure in pediatric patients with rhinosinusitis?

Sinusitis is a common complication of non-allergic and allergic rhinitis, and can trigger lower respiratory diseases, such as bronchitis and asthma. Standard radiography is unable to give any data about the underlying pathological mechanisms (infectious or allergic) involved and infectious rhinosinusitis is very common in pediatric age, even in allergic patients. We investigated the possibility of obtaining more useful diagnostic information, performing nasal brushing (NB) on 117 children with recurrent respiratory symptoms. The following hypothesis were evaluated: (1) whether NB neutrophil/eosinophil percentages and/or NB culture could predict the radiological evidence of maxillary sinusitis; and (2) whether differences between nonallergic and allergic patients could be detected. In the total patient group and in the nonallergic group, the comparison of NB neutrophil percentages in patients with and without maxillary sinusitis showed a statistically significant difference (median 2 and 18%, respectively; P < 0.001). In the nonallergic group, a NB neutrophil rate > or = 5% was chosen as a cut-off between positive and negative NB diagnosis of rhinosinusitis and NB data were compared with radiological investigations. The results obtained showed that NB was fairly sensitive (91%) and predictive (84%). In allergic patients, neither neutrophil nor eosinophil percentages significantly correlated with the presence of sinusitis. Microbiological studies showed that, even if the presence of bacteria in NB resulted associated with sinusitis, a negative culture was not predictive of the absence of the disease. We therefore suggest that NB describes the present inflammatory status of the upper airways, hence, it is more suitable to describe the inflammation related to ongoing upper respiratory tract infections rather than chronic inflammation due to allergic rhinitis, characterized by relapsing episodes of acute inflammation. In conclusion, we propose to consider NB a reliable tool in the diagnosis of rhinosinusitis, particularly in nonallergic pediatric patients. Compared to standard radiological techniques, NB makes it possible to avoid radiation exposure and gives information about the pathological mechanisms involved in the single patient.     

Bacteriology in disease: failure of a micro-ecological balance?

Both aerobic (mainly alpha-haemolytic streptococci) and anaerobic micro-organisms (like Prevotella and Peptostreptococcus species) are able to interfere with the growth of potential pathogens such as group A beta-haemolytic streptococci, Streptococcus pneumoniae, Haemophilus influenzae and Moraxella catarrhalis. The present paper reviews the application of this concept of interference with respect to the development of common acute ear-, nose- and throat infections.     

Speech outcomes after palatal closure in 3–7-year-old children

IntroductionOne of the main goals of the team approach in management of oro-facial clefts is to help the children with cleft palate have adequate speech development.ObjectiveThe present study aimed to investigate the prevalence of articulation and resonance disorders following palate closure in children who were visited for routine examination by the Isfahan Cleft Care Team between 2011 and 2015, and to study the impact of cleft type and age at the time of palatoplasty on speech outcomes.MethodsClinical records of 180 preschool children with repaired cleft palate were reviewed. The percentage of children demonstrating hypernasality, nasal emission, nasal turbulence, and compensatory misarticulations was calculated. The relationship between cleft type and age at the time of palatal surgery, as independent variables, and speech outcomes were examined.Results67.7 and 64.5 percent of the children demonstrated respectively moderate/severe hypernasality and nasal emission, and 71.1 percent produced compensatory misarticulations. Age at the time of palatal repair was significantly associated with compensatory misarticulations and also with moderate/severe hypernasality. The prevalence of compensatory misarticulations, significant hypernasality, nasal emission and also nasal turbulence was not significantly different in various types of cleft.ConclusionsWe observed a high prevalence of different speech disorders in preschool children with repaired cleft palate compared to other studies. This can be partly due to late palatal repair in the studied population. Despite many advances in cleft palate management programs in Iran, there are still many children who do not access the interdisciplinary team cares in their early childhood. We should, therefore, try to increase accessibility of appropriate and timely management services to all Iranian children with cleft lip/palate.