Cochlear implantation in children with auditory neuropathy: Lessons from Brown–Vialetto–Van Laere syndrome

Objective: Brown–Vialetto–Van Laere syndrome (BVVL) is a rare neurodegenerative disorder associated with auditory neuropathy (AN). The decision process for CI in AN is evolving with increasing evidence of efficacy. We evaluated the benefit of CI in children with BVVL syndrome.

Methods: A retrospective study reviewed the pre- and post-operative hearing outcomes of three patients with BVVL who presented for CI. A fourth patient with BVVL who was not suitable for CI is also discussed. The primary outcomes were hearing thresholds and auditory perception. Outcome measurement instruments included visual reinforcement audiometry (VRA) or Play Audiometry (PA), Categories of Auditory Performance (CAP) and Auditory Speech Sound Evaluation (ASSE). Secondary outcomes were parental report (BAPP questionnaire), the perception of our SaLT and compliance.

Results: Patient 1 had ASSE levels of 40–45?dB HL 1 year post-operatively, and CAP score had improved from 2 to 5. At 2-year review, aided thresholds were 40?dB at 2–4?kHz. Three months following CI, the CAP score of Patient 2 had improved from 3 to 5. At 6 months, thresholds were 25–30?dB at 2–4?kHz. Single words/phrases are used by both patients and benefit is reported by both families. Patient 3 has recently undergone CI, having been previously rejected at another centre. Three months following CI, his thresholds were 35–40?dB at 2–4?kHz and increased use of sign and vocalization is reported.

Conclusion: CI in children with AN complicating BVVL has a variable, but a positive effect. Other manifestations of BVVL make measuring benefit challenging, in the absence of a ‘bespoke’ measurement instrument for children with complex needs. This study provides further evidence for the benefit of CI in children with AN.     

Wound Complications in Head and Neck Squamous Cell Carcinomas After Anti–PD‐1 Therapy

Immune checkpoint inhibitors have demonstrated activity in recurrent/metastatic head and neck squamous cell cancer, but less is known regarding their long‐term sequelae. We describe four patients who, after complete responses to anti–PD‐1 therapy, developed complications requiring surgical intervention. Patient 1 is a 57‐year‐old female whose marked tumor regression exposed some mandibular hardware. Patient 2 is a 39‐year‐old male who developed an ulcerated buccal lesion with exposed mandible. Patient 3 is a 66‐year‐old male with craniofacial osteoradionecrosis. Patient 4 is a 71‐year‐old male who developed an exposed and fractured mandible. All patients successfully underwent surgical intervention and remain disease free. Laryngoscope, 129:E428–E433, 2019     

Computer imaging and patient satisfaction in rhinoplasty surgery

The measurement and achievement of improved patient benefit following a particular medical or surgical intervention has become an increasingly relevant part of the provision of effective healthcare. We have retrospectively analysed patient satisfaction in 56 patients following rhinoplasty via the Glasgow Benefit Inventory (GBI), 25 of whom underwent pre-operative computer imaging planning. We have also audited patient reaction to this technique via a concurrent questionnaire in those subjects who underwent imaging, and correlated this with overall patient outcome. Patient satisfaction with cosmetic rhinoplasty following computer imaging was significantly improved compared to those patients who did not receive imaging.     

Angiogenic non-Hodgkin T/natural killer (NK)-cell lymphoma: report of three cases

Angiogenic T/natural killer (NK)-cell lymphoma is a non-Hodgkin lymphoma characterized by necrosis and vascular destruction that is strongly associated with Epstein-Barr virus and AIDS. Early diagnosis is essential to improve the chances of patient survival, but severe local inflammatory infiltrate impairs histologic diagnosis by obscuring neoplastic cells. The most common markers are CD2, CD56, cytoplasmic CD3, and CD43 EBV. We describe 3 cases of angiogenic T/NK-cell lymphoma that show the diverse presentation of the same disease. Patient 1 was HIV positive and had nasal obstruction, facial edema, and ulceration of the nasal mucosa. Patient 2 had fever, a sore throat, and weight loss. Patient 3 had facial edema, fever, proptosis, and rapid development of neurologic alterations. Several biopsies were needed for histologic confirmation in these patients, despite positivity for the CD3 and CD56 markers.     

大环内酯类药物治疗慢性鼻-鼻窦炎的Meta分析

目的：系统评价大环内酯类药物治疗慢性鼻-鼻窦炎（CRS）的临床疗效及安全性。方法计算机检索PubMed、OVID、EMBASE、中国学术期刊全文数据库、万方数据库中关于大环内酯类药物治疗CRS的随机对照试验（Randomized controlled trials,RCT）,同时追索纳入文献的参考文献。检索年限均从建库检索至2016年1月。由2名评价员独立筛查文献,对纳入的文献进行质量评价,并提取资料,对符合质量标准的RCTs进行Meta分析,比较大环内酯类药物组与安慰剂组鼻腔鼻窦结局评分（sinonasal outcome test,SNOT）、患者反应量表评分和安全性评估。统计学分析采用RevMan 5.3软件。结果共纳入3篇RCTs,Meta分析结果显示,长期低剂量应用大环内酯类药物第24周时患者SNOT评分（WMD=-0.43,P=0.03）降低,第12周时SNOT变化（WMD=-0.23,P=0.25）和患者反应量表评分变化（WMD=-0.40,P=0.28）无统计学意义。大环内酯类药物的不良反应发生率与安慰剂组相似。结论长期低剂量应用大环内酯类药物不能明显改善CRS患者的临床症状,其不良反应发生率与对照组相近。     

Temporal bone histopathology of noise-induced hearing loss

To examine the relationship between hearing and changes in the inner ear, we investigated human temporal bone specimens from 2 patients with noise-induced hearing loss and prepared audio-cytocochleograms as described by Schuknecht et al. Patient 1 was a 50-year-old male who died of thyroid cancer and had worked at a printing house for 38 years. Patient 2 was a 58-year old male who died of maxillary sinus cancer and had worked in construction for 22 years. A pure-tone audiogram showed high-tone sensorineural hearing loss with c5-dip-type hearing disorder in both ears in Patient 1, and a high-tone abrupt form of sensorineural hearing loss in Patient 2. Pathological examination of the temporal bone revealed degeneration and disappearance of the organ of Corti at the basal turn and disappearance of cochlear neurons in both patients. Audio-cytocochleograms revealed hearing disorder consistent with the changes in the inner ear in both patients. Marked degeneration and disappearance of the organ of Corti and stria vascularis were present in patient 1. It is generally known that disorders of the organ of Corti for a long period is involved in the etiology of noise-induced hearing loss. This degeneration of the organ of Corti is produced at a basilar membrane with the maximum amplitude related to exposure to noise according to a physical and mechanical factors. Moreover, animal experiments have shown that exposure to noise decrease cochlear blood flow. In Patient 1 both the organ of Corti and the stria vascularis exhibited degeneration, suggesting that not only physical and mechanical factors but a cochlear circulatory disorder related to exposure to noise was involved in the etiology of the pathological changes in the temporal bone related to noise-induced hearing loss.     

Comparative case series of exostoses and osteomas of the internal auditory canal

Exostoses and osteomas are benign bony lesions of the auditory canal. Although common in the external auditory canal, they are rare and difficult to distinguish in the internal auditory canal (IAC). In this literature review and case presentation, we define radiologic and histologic criteria to differentiate exostoses from osteomas of the IAC. Two patients with exostoses and 1 patient with an osteoma of the IAC are described here. Patient 1 presented with disabling vertigo and was found to have bilateral exostoses with nerve impingement on the right. After removal of the right-sided exostoses via retrosigmoid craniotomy, the patient had complete resolution of her symptoms over 1 year. Patient 2 presented with bilateral pulsatile tinnitus and vertigo and was found to have bilateral IAC exostoses. Patient 3 presented with hearing loss and tinnitus, and a unilateral IAC osteoma was ultimately discovered. Because of the mild nature of their symptoms, patients 2 and 3 were managed without surgery. We show that IAC osteomas can be differentiated from exostoses by radiographic evidence of bone marrow in high-resolution computed tomography scans, or by the presence of fibrovascular channels on histologic analysis. Management of these rare entities is customized on the basis of patient symptoms.     

Miniature language system acquisition by children with different learning proficiencies

Sixteen children, aged 7:8 to 9:10, learned two miniature languages while playing a communication game. Both languages expressed Action (Agent, Patient) meanings and incorporated a Patient suffix. They differed in word order: VSO (Language I) versus SOV (Language II). Children found the SOV language easier; they also made more suffix errors and fewer word order errors in this language. The results suggest that the perceptual salience of an utterance-final particle may hinder grammatical analysis, at least if capacity limits and perseverative learning strategies intervene.     

Relationship between Sudden Sensorineural Hearing Loss and Vascular Risk Factors

Objective To explore the relationship between sudden sensorineural hearing loss(SSNHL) and vascular risk factors (including serum lipids and uric acid). Method This is a retrospective analysis of 100 cases of SSNHL seen at the Drum Tower Hospital, Nanjing Medical University, between Jan. 2007 and Apr. 20080 Patient history, blood test results and imaging scans were analyzed. Levels of triglyceride (TG), cholesterols (CHO), high density lipoprotein-cholesterol (HDL-CH), low density lipoprotein-cholesterol (LDL-CH), apolipoprotein AI (ApoAI), apolipoprotein B(ApoB) and uric acid(UA) from these patients were compared with a control group of 56 patients treated for vocal cord polyps or nasal septum deviation during the same period. Patients with hyperten-sion, diabetes, heart, brain, liver or kidney disorders are excluded from the present investigation. Results HDL-CH level was higher and UA level lower in the study group than the control group (P< 0.05). HDL-CH and UA showed no significant differences among different age-groups (P > 0.05). There were no significant differences in the levels of TG, CHO, LDL-CH, ApoAI and ApoB (P > 0.05). Conclusion These data indicate that metabolic disturbances of serum lipids and/or uric acid may be potential risk factors for SSNHL     

Objective and subjective scar aesthetics in minimal access vs conventional access parathyroidectomy and thyroidectomy surgical procedures: a paired cohort study

OBJECTIVE: To determine if performing parathyroidectomy surgery through minimal access (MA) incisions has any notable aesthetic or quality-of-life impact on patients compared with conventional access (CON) techniques. DESIGN: Paired cohort with (1) a prospective MA incision and scar cohort and (2) a sex- and age-matched (within 3 years) retrospective CON incision and scar cohort. SETTING: Tertiary care center. PATIENTS: Fifteen patients enrolled in prospective study protocol over a 2-year period; 11 patients met inclusion criteria. A sex- and age-matched retrospective cohort of patients was selected from a patient population undergoing surgical treatment of thyroid neoplastic diseases using a CON approach. Inclusion criteria were use of MA incision for parathyroidectomy and return for long-term follow-up scar assessment. INTERVENTIONS: Minimal access parathyroidectomy surgery vs CON thyroidectomy surgical procedures, postoperative follow-up assessment of scar aesthetics by patient and naive viewers, and digital photography and analysis of the surgical incision site. All patients were followed for at least 8 months after surgery. MAIN OUTCOME MEASURES: The Patient and Observer Scar Assessment Scale (POSAS), Vancouver Scar Scale, and photographic scar analysis by naive viewers. RESULTS: There was no significant difference in scar assessment scale scores between the MA and CON cohorts and no clinically significant difference in overall patient satisfaction with scars between cohorts (POSAS: Patient Scar Assessment Scale, P = .14, and Observer Scar Assessment Scale, P = .79; Vancouver Scar Scale, P = .76). There was increased visibility of scars in the CON cohort to naive viewers. CONCLUSIONS: Although they were more readily visible to naive viewers, CON (larger) cervical scars created in parathyroidectomy or thyroidectomy surgery do not translate into decreased patient satisfaction with their scar result. This may indicate a limited quality-of-life benefit in using MA approaches in transcervical surgical procedures.     

A case of adult T cell leukemia with laryngeal tumor

Adult T cell leukemia (ATL) is one of malignant tumors originated from T-cell system, first described by Takatsuki in 1976. Most cases of ATL have been diagnosed in department of internal medicine and dermatology, because they usually show lymphoadenopathy and skin rash. However, it is rare that ATL has symptoms of otolaryngology. We experienced a case of hoarseness with smoldering ATL. Patient was 51 years old male. He had a tumor in left vocal cord. Pathologically the tumor was malignant lymphoma originated from T cell. HTLV-1 was provided in his serum and proviral DNA was found in ATL cells. First cisplatin was given generally and soon radiotherapy was applied. Reaction of therapy was good and tumor of larynx disappeared gradually. Patient has been alive 6 years from the onset.     

Review of management of laryngopharyngeal reflux disease

This review was conducted according to the Patient/problem Intervention Comparison Outcome (PICO) Statements. Some studies reported that 10–30% of patients consulting in ENT come with presenting symptoms of laryngopharyngeal reflux (LPR), but the exact prevalence of LPR is still unknown. Management has not changed in 20 years despite a significant increase in the number of publications on epidemiology, clinical presentation, diagnosis and treatment. The development of hypopharyngeal-esophageal multichannel intraluminal impedance pH monitoring (HEMII-pH) and saliva pepsin detection now allow a new multidimensional diagnostic approach associating clinical scores to HEMII-pH and saliva pepsin detection. This new approach may enable personalized treatment according to LPR profile on HEMII-pH (acid, non-acid, mixed; upright, recumbent reflux episodes). Updated treatment of LPR could consist in a 3-month association of dietary measures, proton pump inhibitors, alginate and magaldrate, followed by treatment adaptation.     

Heterotopic salivary gland imitating laryngeal cyst

Patient aged 56 with heterotropic salivary gland in vestibule of the larynx was described. Localization of heterotropic salivary tissue in the larynx is very rare.     

Thoracic duct cyst

Patient aged 48 with the cyst of the cervical part of thoracic duct was described. Cyst appeared as the result of inflammation. Histopathological examination excluded the cystic lymphangioma.     

The role of post-operative imaging in cochlear implant surgery: A review of 220 adult cases

Abstract

Objectives

To determine the incidence of abnormal radiological findings after cochlear implantation and their effect on clinical outcomes.

Methods

Retrospective review of 220 adult cochlear implants. Clinical records and post-operative plain X-rays were reviewed and compared with pre-operative and 6-month post-operative City University of New York (CUNY) speech scores.

Results

There were no cases of extra-cochlear array misplacement. Imaging showed 20 cases of incomplete array insertion (9.2%), 3 cases of kinking of the array (1.4%), 2 cases of tip rollover (0.9%), and 1 case of apparent array fracture (0.5%). Patient management was not altered by abnormal imaging. Patients with abnormal radiological findings had slightly minor improvements (median 39 vs. 56%) in City University of New York (CUNY) speech discrimination scores at 6 months (Mann–Whitney U test, P = 0.043).

Conclusion

All abnormalities on post-operative imaging were minor and did not alter patient management. The future role of post-operative imaging is discussed.     

Analysis of 2167 head and neck cancer patients' management, treatment compliance and outcomes from a regional cancer centre, Delhi, India

Head and neck cancer care was analysed in 2167 unselected patients for management compliance and outcome. Median age was 55 years, with a male to female ratio of 5.5ratio1. Major sites were oropharynx (32.4 per cent), larynx (19.8 per cent), oral (16.6 per cent) and hypopharynx (12.9 per cent). Stage-wise distribution was I-II=8.9 per cent, III=20.6 per cent and IV=60.3 per cent and unstaged=10.2 per cent. Squamous cell carcinoma was the dominant histology for 90.9 per cent. Clinic-based cancer-directed treatment decisions were made for 1905 patients: curative intent in 53 per cent, palliative in 35 per cent and for the remaining 262 (12 per cent) supportive care. Overall, 1209 (56 per cent) patients complied with the prescribed treatments; 62 per cent, 54 per cent, and 35 per cent of curative, palliative and supportive care intent groups, respectively. Modalities were radiotherapy alone (64.6 per cent), combined surgery with irradiation (17.6 per cent), and chemoradiotherapy (11.2 per cent). Median follow-up periods were 17.5 and three months in curative and palliative groups respectively. Overall, 712 (33 per cent) cases received curative therapy, with three-year disease-specific survival of 49 per cent. Patient compliance was a major obstacle. The comparison of this series with the USA, Canada and Norway showed wide disparities in stage of presentation and survival.     

Acute epiglottitis in adults. Our case load in 11 years

We report a retrospective study of 20 cases of acute epiglottitis in adults admitted at our hospital between 1991 and 2001. We gather a series of variables: Patient sex, age, year and month of admission, personal history, initial clinical symptoms, complementary tests asked, given treatment, evolution and hospital average stay. We found an obvious predominance in male (19:1 with respect to female), with an average age of 45 years. The main symptoms were dysphagia-odynophagia (85%), followed by fever (55%) and pharyngocervical pain. Dyspnea was confirmed in 9 of 20 patients (45%); 5 of them required airway control with the help of intubation in one case, urgent coniotomy in three cases and tracheotomy in another one. A patient suffered from an acute mediastinitis and required assistance and medical i.v. extended treatment in UCI. We show a case of another patient who suffered sudden cardiorespiratory arrest a followed by death. He did not present previous dyspnea. Acute epiglottitis in adults is a rare pathology with a good prognosis in general, but may lead to an unpredictable and serious complication even in the absence of airway blockage. We carry out a medical literature review in this respect.     

Lymphocyte response to phytohaemagglutinin before and after radiation therapy in patients with carcinomas of the head and neck.

The ability of peripheral blood lymphocytes to respond in vitro to phytohaemagglutinin (PHA) was studied before and after radiation therapy in patients with locally advanced squamous cell carcinomas of head and neck. Patient lymphocyte response to PHA was depressed before therapy and further declined following therapy. The pre-therapy PHA responsiveness was found to be prognostically significant in regard to patient survival.     

Resolution of auditory neuropathy spectrum disorder after shunt placement in a patient with hydrocephalus: A case report

PurposeThere have been previous case reports suggesting the resolution of both sensorineural hearing loss and retrocochlear involvement through the management of hydrocephalus with shunt placement. This is a case report of a patient with Auditory Neuropathy Spectrum Disorder (ANSD) that resolved after shunt placement in a patient with hydrocephalus.Materials and methodsChart review of a single patient with a diagnosis of ANSD and hydrocephalus. Type of audiometric testing and results were document.ResultsPatient is an infant who was diagnosed with hydrocephalus at birth and ANSD in the right ear at 3 months of age. Patient underwent shunt placement at 9 months old and had behavioral testing 2 months later. Audiometry showed normal behavioral audiometric thresholds with presence of ipsilateral and contralateral reflexes which is suggestive of resolution of ANSD.ConclusionsThis is a single case report of resolution of ANSD after shunt placement in a patient with hydrocephalus. Close monitoring and repeat audiological evaluation is recommended to follow these patients.     

Brown tumors of the jaws associated with primary or secondary hyperparathyroidism. A clinical study and review of the literature

PURPOSE: The aim of this article is to present the development of brown tumors in the jaws as a definite feature of hyperparathyroidism (HPT), whether primary or secondary. PATIENTS AND METHODS: The study included 2 cases of primary HPT and 3 cases of secondary HPT associated with brown tumors in the jaws. The patients presented in our clinic at the "G. Papanikolaou" Hospital in Thessaloniki for treatment of their disease. The patients were 4 women and 1 man, and patient's ages ranged from 21 to 76 years. Four cases of the brown tumors involved the mandible, and 1 case involved the maxilla and the maxillary sinus. The surgical excision of bone lesions and treatment of primary or secondary HPT were performed on the patients in our study. RESULTS: Patient 1 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with surgical removal of the maxillary lesion and excision of the adenoma. Three years later, the patient is free of the disease, with no recurrence of the brown tumor. Patient 2 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with excision of the adenoma. One year later, a second bone lesion developed in the mandible, and 3(1/2) of the fourth parathyroid (right) glands was removed. The patient is being observed. The other 3 patients with brown tumors associated with secondary HPT were treated with excision of the bone lesions; HPT was treated with hemodialysis (2 patients) and renal transplantation (1 patient). For the patients treated with hemodialysis, the disease is under control and there is no recurrence of the brown tumors. The patient who underwent transplantation is free of the disease 7 years later. CONCLUSIONS: Primary or secondary HPT may be recognized by the presence of an osteolytic lesion with giant cells, a condition referred as "brown tumor." The most useful therapy for patients with brown tumors is surgical excision of bone lesions and therapy (surgical or medical) for primary or secondary HPT.