Alterations in serum lipid profile patterns have long been associated with malignancies, and their role remains controversial with respect to head and neck cancer. Due to an increased rate of neoplastic cell multiplic...Alterations in serum lipid profile patterns have long been associated with malignancies, and their role remains controversial with respect to head and neck cancer. Due to an increased rate of neoplastic cell multiplication and reduced supply, there is increased utilization of lipids causing Hypolipidemia. Adding to this, tobacco contains carcinogens capable of damaging the cell membrane components including lipids resulting in further hypolipidemia. Thus the purpose of the present case control study is to discuss the alterations in plasma lipid profile in head and neck cancer patients in association with tobacco consumption. This hospital based study includes 80 cases of head and neck cancer patients and 50 controls. Plasma Lipids included are total cholesterol (TC), high density lipoprotein cholesterol (HDL), low density lipoprotein cholesterol (LDL), very low density lipoprotein cholesterol (LDL), triglycerides (TG). Student’s “t test” was applied to the data acquired. Values of P 0.05 were considered statistically insignificant.展开更多
Several technical variations of tympanoplasty have been reported,usually involving the type of graft and/or instruments used.Few studies have focused on the outcome of type-1 tympanoplasty specifically in teaching sce...Several technical variations of tympanoplasty have been reported,usually involving the type of graft and/or instruments used.Few studies have focused on the outcome of type-1 tympanoplasty specifically in teaching scenarios.We aimed to describe the results of type-1 tympanoplasty performed by residents,and to investigate potential predictive factors of surgical success.To do so,we did a retrospective analysis of medical records of patients who underwent type-1 tympanoplasty in a tertiary university hospital.We evaluated the tympanic membrane closure and audiometric outcomes during the first year of follow-up,and compared the results according to some clinical and surgical factors.130 operated ears were included in the study.The closure rate reached 84.12%after one month and 72.72%after twelve months of surgery.The mean air-bone gap was 22.98 dB preoperatively,and reached 10.55 dB after surgery.Perforation closure rates were 85%and 57.14%for those operated by endoscopic and microscopic-assisted approaches,respectively(p?0.004).The use of cartilage grafts and time without otorrhea of more than three months prior to surgery were also predictors of surgical success(p?0.002 and 0.041,respectively).Gender,age,perforation size,contralateral disease,operated side,reoperation,and degree of hearing loss did not significantly interfere with outcomes.Tympanoplasty showed good overall results when performed by residents,although inferior to those reported by experienced surgeons.The use of the endoscope,cartilage grafting,and longer preoperative time without otorrhea were predictors of surgical success in this scenario.展开更多
Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the cal...Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the caloric vestibular and video head-impulse tests(vHIT)diagnosing the disorder.Methods:68 patients suffering from posterior canal BPPV(25 male,43 females,mean age±SD,54.5±13.2 years)and 56 patients with a normal functioning vestibular system as control were investigated.Bithermal caloric test and vHIT was performed during the same medical check-up.Canal paresis(CP%),gain(GA)and asymmetry(GA%)parameters were calculated.Results:The Dix-Hallpike manoeuvre was only positive in 4%of this population.The CP%parameter was only pathologic in two patients,and there was no significant difference between control and BPPV patients(p=0.76).The GA value was never under 0.8 in this population,but GA%was abnormal in 63.2%.A significant difference comparing the GA%values to the control group was seen(p=0.034).There was no correlation detected between the CP%and GA%values in BPPV.Regarding the GA%value,61%sensitivity and 76%specificity was seen.Conclusion:The Dix-Hallpike manoeuvre was not often positive in the non-acute phase of BPPV;therefore,objective testing is essential.The caloric test does not have clinical significance in BPPV,but vHIT can be helpful based on the GA%parameter。展开更多
BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and ...BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.展开更多
Background:Tinnitus is a sound precepted without an external sound stimulus.Its background can be categorised into primary and secondary cases.The secondary cases include pathologies of the external,middle and inner e...Background:Tinnitus is a sound precepted without an external sound stimulus.Its background can be categorised into primary and secondary cases.The secondary cases include pathologies of the external,middle and inner ear.Tinnitus can be objective or subjective;the latter can only identified by the sufferer.Previous research results have shown that tinnitus significantly affects the quality of life and daily functioning.Objectives:To analyse the impact of tinnitus on the daily functioning and the possible influence of demographical data and tinnitus duration on it.Methods:630 patients(265 males and 365 females,25e85 years of age)suffering from primary tinnitus were enrolled.In the Hungarian language,these patients completed the Tinnitus Handicap Inventory(THI)questionnaire and underwent a complete otorhinolaryngological examination.IBM SPSS V24 software was used for data processing;correlation tests,the Mann-Whitney U and Kruskal-Wallis nonparametric tests were used.Results:According to the THI questionnaires outcomes,most patients(62.5%)were presented with a mild handicap.Based on statistical analysis,no significant correlation was observed between the total THI points and the age of the patients,along with the duration and localisation of the symptoms.However,the total THI scores of male and female patients significantly differed,indicating higher THI values in the female group(p=0.00052*).Conclusions:The tinnitus severity was not affected by the duration,localisation of the symptoms and age but by gender,indicating higher values in the case of females.展开更多
Waardenburg syndrome is a rare disease characterized by sensorineural deafness in association with pigmentary defects. Depending on additional symptoms, WS have been classified into four types.Waardenburg syndrome typ...Waardenburg syndrome is a rare disease characterized by sensorineural deafness in association with pigmentary defects. Depending on additional symptoms, WS have been classified into four types.Waardenburg syndrome type 4, also called as Waardenburg Shah Syndrome is a very rare congenital disorder with astounding variable clinical expression, characterized by pigmentary abnormalities of the hair(A white forelock of hair, premature graying) and pigmentary changes of the iris such as heterochromia or homochromia irides, sensorineural deafness and Hirschsprung disease. Three genes have been bestowed so far in consociation with EDNRB, EDN3, and SOX10 genes. The pattern of inheritance is multifarious with the SOX10 mutation affiliation with autosomal dominant inheritance whereas the EDNRB and EDN3 genes are passed down in an autosomally recessive pattern.展开更多
Endoscopic ear surgery(EES) is an exciting, rapidly developing and innovative field of otologic surgery.Technically and conceptually, EES is a significant departure from traditional microscopic transcanal approaches t...Endoscopic ear surgery(EES) is an exciting, rapidly developing and innovative field of otologic surgery.Technically and conceptually, EES is a significant departure from traditional microscopic transcanal approaches to the middle ear and canal that has shown very positive results with respect to patient outcomes. This review serves as a primer for the otologist and otology resident embarking on EES and discusses the theory surrounding the learning process, the optical chain for endoscopic surgery as well as other important underlying principles.展开更多
Pediatric cholesteatoma occurs in one of two forms:congenital cholesteatoma,developing from embryonic epidermal cell rests or acquired cholesteatoma,associated with a focal defect in the tympanic membrane.This disease...Pediatric cholesteatoma occurs in one of two forms:congenital cholesteatoma,developing from embryonic epidermal cell rests or acquired cholesteatoma,associated with a focal defect in the tympanic membrane.This disease has been traditionally managed with the operating microscope,often requiring mastoidectomy for adequate visualization of and access to the middle ear and mastoid cavities.Recently,advances in endoscopic equipment have enabled otologists to manage most cases of pediatric cholesteatoma via a minimally-invasive,transcanal endoscopic approach.This review discusses the current literature relating to the etiopathogenesis,assessment and endoscopic management of pediatric cholesteatoma.Early outcomes of endoscopic treatment,emerging trends and technologies are also reviewed.展开更多
Objective: To assess the outcomes of hearing and vertigo in patients with Meniere’s disease who were treated with intratympanic methylprednisolone. Study Design: Retrospective study. Setting: Tertiary referral hospit...Objective: To assess the outcomes of hearing and vertigo in patients with Meniere’s disease who were treated with intratympanic methylprednisolone. Study Design: Retrospective study. Setting: Tertiary referral hospital. Methods: In our study with 151 patients [191 ears] were diagnosed as Menieres as per 1995 AAO-HNS guidelines. As an office based procedure these patients were treated with intratympanic methyl prednisolone. Results: There was a 98.6% improvement in the vertigo control rate, a 73.82% improvement in hearing and 29.31% had a definitive improvement in tinnitus. Conclusion: We conclude from our study that intratympanic steroid injection significantly controls vertigo, tinnitus and improves hearing in Meniere’s disease with minimal complications. Hence, it can be tried as a first line treatment for patients with Meniere’s disease.展开更多
BACKGROUND Craniometaphyseal dysplasia(CMD)is a rare genetic disorder.Autosomal dominant CMD(AD-CMD)is caused by mutations in the ANKH gene.Affected individuals typically have distinctive facial features including pro...BACKGROUND Craniometaphyseal dysplasia(CMD)is a rare genetic disorder.Autosomal dominant CMD(AD-CMD)is caused by mutations in the ANKH gene.Affected individuals typically have distinctive facial features including progressive thickening of the craniofacial bones.Treatment for AD-CMD primarily consists of surgical intervention to release compression of the cranial nerves and the brain stem/spinal cord.To alleviate progression of the clinical course and improve the quality of life in children waiting to undergo the necessary surgery,we investigated clinical changes in a diagnosed patient with AD-CMD over three years.CASE SUMMARY A 17-mo-old boy presented with progressive nasal obstruction,snoring and hearing loss symptoms.Physical examination showed enlargement of the head circumference and clinical features such as wide nasal bridge,paranasal bossing,widely spaced eyes with an increased bizygomatic width,and a prominent mandible.The patient underwent otolaryngological examination,endoscopy,hearing test,laboratory examination of phosphorus and bone metabolism,cranial and femoral computed tomography,X-ray and next-generation sequencing.The patient was diagnosed with AD-CMD due to p.Phe377 deletion(c.1129_1131del)on exon 9 of the ANKH gene.After adherence to a prescribed low-calcium diet,the boy’s alkaline phosphatase(ALP)levels continuously decreased to within the normal range.However,after 14 mo of dietary intervention,his parents altered his diet to an intermittent low-calcium diet to include milk and eggs.The patient’s ALP was slightly higher than normal after the dietary change but remained close to the normal range.His serum osteocalcin changed to within normal levels after dietary regulation for 33 mo.His serum combined beta C-terminal telopeptide of type I collagen also continuously decreased after the nutritional intervention,although still slightly higher than normal levels.Despite fluctuating blood test results,the boy’s nasal symptoms were markedly relieved and steadily improved after dietary intervention.No significant changes were found in the craniofacial bones by cranial radiography.Close monitoring of clinical features is still ongoing.Calcitriol treatment is currently under consideration and a surgical procedure is planned as necessary in the future.CONCLUSION We herein report the first Chinese case of AD-CMD with heterozygous mutation of p.Phe377 deletion(c.1129_1131del)on the ANKH gene.Biochemical alterations were significantly improved after dietary intervention indicating that a lowcalcium diet may be applied in pediatric AD-CMD patients with ANKH mutations to help alleviate phenotypic manifestations and improve the quality of life before surgical intervention.Further large scale studies are needed to replicate these findings and to establish the appropriate timing for nutritional and surgical interventions。展开更多
Objective: The aim is to review the effectiveness of sialendoscopy in treating various salivary gland disorders related to the duct. Methods: A total of 133 patients who underwent sialendoscopy were included in this s...Objective: The aim is to review the effectiveness of sialendoscopy in treating various salivary gland disorders related to the duct. Methods: A total of 133 patients who underwent sialendoscopy were included in this study. Data was collected from case records of patients and was analysed. A detailed history examination was taken and the operative findings were analysed. Causes of obstructive sialadenitis were evaluated and treated. Results: A total of 159 glands were subjected to sialendoscopy and pathology was identified in 149 glands. Forty one submandibular glands had calculi and twelve had non-calcular obstruction. Fifteen parotid glands had calculi and 81 had non-calcular obstruction, of which stricture was the most common. Calculi retrieval or dilation of stricture was achieved with a sialendoscope in 112 glands. In 23 patients, the calculi were retrieved by combined approach. In three patients, the calculi were fragmented with intra luminal Holmium laser and the fragments were removed. All these patients had a good relief from their symptoms. Duct perforation was seen in two patients and a false passage was formed in one. Conclusion: Sialendoscopy was a safe and effective procedure for managing ductal pathologies of salivary glands.展开更多
Syringocystadenoma papilliferum(SCAP)is a rare benign skin tumor that develops from the apocrine or eccrine glands.Here,we report the case of a 2-year-old girl who presented with an asymptomatic,slowly growing lesion ...Syringocystadenoma papilliferum(SCAP)is a rare benign skin tumor that develops from the apocrine or eccrine glands.Here,we report the case of a 2-year-old girl who presented with an asymptomatic,slowly growing lesion on her scalp.On physical examination,a 2 cm×1 cm red papillary plaque with a crusted surface was noted on her scalp.Histopathological examination revealed papillomatous expansion of the epidermis and cystic invaginations extending from the epidermis down to the deep dermis.The cystic structures were lined by papillae with two layers of columnar epithelium,which showed decapitation secretion.Based on the above clinicopathological findings,a diagnosis of congenital scalp SCAP was made.展开更多
The cochlear auditory epithelium contains two types of sound receptors,inner hair cells(IHCs)and outer hair cells(OHCs).Mouse models for labelling juvenile and adult IHCs or OHCs exist;however,labelling for embryonic ...The cochlear auditory epithelium contains two types of sound receptors,inner hair cells(IHCs)and outer hair cells(OHCs).Mouse models for labelling juvenile and adult IHCs or OHCs exist;however,labelling for embryonic and perinatal IHCs or OHCs are lacking.Here,we generated a new knock-in Fgf8^(P2A-3×GFP/+)(Fgf8^(GFP)/+)strain,in which the expression of a series of three GFP fragments is controlled by endogenous Fgf8 cis-regulatory elements.After confirming that GFP expression accurately reflects the expression of Fgf8,we successfully obtained both embryonic and neonatal IHCs with high purity,highlighting the power of Fgf8^(GFP)/+.Furthermore,our fate-mapping analysis revealed,unexpectedly,that IHCs are also derived from inner ear progenitors expressing Insm1,which is currently regarded as an OHC marker.Thus,besides serving as a highly favorable tool for sorting early IHCs,Fgf8^(GFP)/+will facilitate the isolation of pure early OHCs by excluding IHCs from the entire hair cell pool.展开更多
Objectives:2019 novel coronavirus disease(COVID-19)infection is commonly associated with olfactory dysfunctions,but the basic pathogenesis of these complications remains controversial.This study seeks to evaluate the ...Objectives:2019 novel coronavirus disease(COVID-19)infection is commonly associated with olfactory dysfunctions,but the basic pathogenesis of these complications remains controversial.This study seeks to evaluate the value of magnetic resonance spectroscopy(MRS)in determining the molecular neurometabolite alterations within the main brain olfactory areas in patients with COVID-19-related anosmia.Methods:In a cross-sectional study,seven patients with persistent COVID-19-related anosmia(mean age:29.57 years)and seven healthy volunteers(mean age:27.28 years)underwent MRS in which N-acetyl-aspartate(NAA),choline(Cho),creatine(Cr),and their ratios were measured in the anterior cingulate cortex,dorsolateral prefrontal cortex,orbitofrontal cortex(OFC),insular cortex,and ventromedial prefrontal cortex.Data were analyzed using TARQUIN software(version 4.3.10),and the results were compared with an independent sample t-test and nonparametric Mann-Whitney test based on the normality of the MRS data distribution.Results:The mean duration of anosmia before imaging was 8.5 months in COVID-19-related anosmia group.MRS analysis elucidated a significant association between MRS findings within OFC and COVID-19-related anosmia(P_(disease)<0.01),and NAA was among the most important neurometabolites(P_(interaction)=0.006).Reduced levels of NAA(P<0.001),Cr(P<0.001)and^(NAA)/Cho ratio(P=0.007)within OFC characterize COVID-19-related anosmia.Conclusions:This study emphasizes that MRS can be illuminating in COVID-19-related anosmia and indicates a possible association between central nervous system impairment and persistent COVID-19-related anosmia.展开更多
Acoustic reflex is a sensitive indicator of middle ear lesions when the tympanic membrane is in-tact and tympanometry is normal. Acoustic reflex is not usually observed in conductive hearing loss caused by disruption ...Acoustic reflex is a sensitive indicator of middle ear lesions when the tympanic membrane is in-tact and tympanometry is normal. Acoustic reflex is not usually observed in conductive hearing loss caused by disruption of the ossicular chain including ossicular discontinuity and fixation. Ossicular disruption can show at least partially intact acoustic reflex under a certain condition. Moreover, ossicular discontinuity with a nearly intact acoustic reflex is quite rare and there have been few reports published to date. We here present a rare case of conductive hearing loss with a nearly intact acoustic reflex, and the patient was surgically confirmed to have ossicular discontinuity.展开更多
A large variety of foreign bodies are swallowed by children, but the majority of those pass through the gastrointestinal tract without any adverse effects. The highest incidence of swallowed foreign bodies occurs in c...A large variety of foreign bodies are swallowed by children, but the majority of those pass through the gastrointestinal tract without any adverse effects. The highest incidence of swallowed foreign bodies occurs in children between 6 months and 3 years. We reported to you an unusually large foreign body ingested by a 2-year-old male patient who underwent a rigid oesophagoscopy but the foreign body had to be removed by open surgical technique. It is advisable to have a multidisciplinary approach while dealing with such cases.展开更多
文摘Alterations in serum lipid profile patterns have long been associated with malignancies, and their role remains controversial with respect to head and neck cancer. Due to an increased rate of neoplastic cell multiplication and reduced supply, there is increased utilization of lipids causing Hypolipidemia. Adding to this, tobacco contains carcinogens capable of damaging the cell membrane components including lipids resulting in further hypolipidemia. Thus the purpose of the present case control study is to discuss the alterations in plasma lipid profile in head and neck cancer patients in association with tobacco consumption. This hospital based study includes 80 cases of head and neck cancer patients and 50 controls. Plasma Lipids included are total cholesterol (TC), high density lipoprotein cholesterol (HDL), low density lipoprotein cholesterol (LDL), very low density lipoprotein cholesterol (LDL), triglycerides (TG). Student’s “t test” was applied to the data acquired. Values of P 0.05 were considered statistically insignificant.
文摘Several technical variations of tympanoplasty have been reported,usually involving the type of graft and/or instruments used.Few studies have focused on the outcome of type-1 tympanoplasty specifically in teaching scenarios.We aimed to describe the results of type-1 tympanoplasty performed by residents,and to investigate potential predictive factors of surgical success.To do so,we did a retrospective analysis of medical records of patients who underwent type-1 tympanoplasty in a tertiary university hospital.We evaluated the tympanic membrane closure and audiometric outcomes during the first year of follow-up,and compared the results according to some clinical and surgical factors.130 operated ears were included in the study.The closure rate reached 84.12%after one month and 72.72%after twelve months of surgery.The mean air-bone gap was 22.98 dB preoperatively,and reached 10.55 dB after surgery.Perforation closure rates were 85%and 57.14%for those operated by endoscopic and microscopic-assisted approaches,respectively(p?0.004).The use of cartilage grafts and time without otorrhea of more than three months prior to surgery were also predictors of surgical success(p?0.002 and 0.041,respectively).Gender,age,perforation size,contralateral disease,operated side,reoperation,and degree of hearing loss did not significantly interfere with outcomes.Tympanoplasty showed good overall results when performed by residents,although inferior to those reported by experienced surgeons.The use of the endoscope,cartilage grafting,and longer preoperative time without otorrhea were predictors of surgical success in this scenario.
基金supported by EFOP-3.6.3-VEKOP-16-2017-00009 Project and by theÚNKP-20-4-I New National Excellence Program of The Ministry for Innovation and Technology from the Source of The National Research,Development and Innovation Fund.
文摘Background:Benign paroxysmal positional vertigo(BPPV)is characterized by vertigo lasting from seconds to minutes,induced by head movements.Objectives:Our study aimed to investigate the clinical significance of the caloric vestibular and video head-impulse tests(vHIT)diagnosing the disorder.Methods:68 patients suffering from posterior canal BPPV(25 male,43 females,mean age±SD,54.5±13.2 years)and 56 patients with a normal functioning vestibular system as control were investigated.Bithermal caloric test and vHIT was performed during the same medical check-up.Canal paresis(CP%),gain(GA)and asymmetry(GA%)parameters were calculated.Results:The Dix-Hallpike manoeuvre was only positive in 4%of this population.The CP%parameter was only pathologic in two patients,and there was no significant difference between control and BPPV patients(p=0.76).The GA value was never under 0.8 in this population,but GA%was abnormal in 63.2%.A significant difference comparing the GA%values to the control group was seen(p=0.034).There was no correlation detected between the CP%and GA%values in BPPV.Regarding the GA%value,61%sensitivity and 76%specificity was seen.Conclusion:The Dix-Hallpike manoeuvre was not often positive in the non-acute phase of BPPV;therefore,objective testing is essential.The caloric test does not have clinical significance in BPPV,but vHIT can be helpful based on the GA%parameter。
文摘BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is an extremely rare tumor characterized by the malignant proliferation of myofibroblasts.LGMS most commonly develops in adults,predominantly in males,in the head and neck region,oral cavity,especially on the tongue,mandible,and larynx.This article presents 2 cases of LGMS localized to the maxillary sinus and provides an overview of the available literature.CASE SUMMARY Two patients with LGMS located in the maxillary sinus underwent surgery at the Department of Head and Neck Surgery.Case 1:A 46-year-old patient was admitted to the clinic with suspected LGMS recurrence in the right maxillary sinus(rT4aN0M0),with symptoms of pain in the suborbital area,watering of the right eye,thick discharge from the right nostril,and augmented facial asymmetry.After open biopsy-confirmed LGMS,the patient underwent expanded maxillectomy of the right side with immediate palate reconstruction using a microvascular skin flap harvested surgically from the middle arm.The patient qualified for adjuvant radiotherapy for the postoperative bed,with an additional margin.Currently,the patient is under 1.5 years of observation with no evidence of disease.Case 2:A 45-year-old man was admitted to our clinic with facial asymmetry,strabismus,exophthalmos,and visual impairment in the right eye.Six months earlier,the patient had undergone partial jaw resection at another hospital for fibromatosis.A contrast-enhanced computed tomography scan revealed a tumor mass in the postoperative log after an earlier procedure.An open biopsy confirmed lowgrade fibrosarcoma(rT4aN0M0).The patient qualified for an extended total right maxillectomy with orbital excision and right hemimandibulectomy with immediate microvascular reconstruction using an anterolateral thigh flap.The patient subsequently underwent adjuvant radiotherapy to the postoperative area.After 9 months,recurrence occurred in the right mandibular arch below the irradiated area.The lesion infiltrated the base of the skull,which warranted the withdrawal of radiotherapy and salvage surgery.The patient qualified for palliative chemotherapy with a regimen of doxorubicin+dacarbazine+cyclophosphamide and palliative radiotherapy for bone metastases.The patient died 26 months after surgical treatment.The cases have been assessed and compared with cases in the literature.CONCLUSION No specific diagnostic criteria or treatment strategies have been developed for LGMS.The treatment used for LGMS is the same as that used for sinonasal cancer radical tumor excision;adjuvant radiotherapy or chemoradiotherapy should also be considered.They have low malignant potential but are highly invasive,tend to recur,and metastasize to distant sites.Patients should undergo regular follow-up examinations to detect recurrence or metastasis at an early stage.Patients should be treated and observed at the highest referral centers.
文摘Background:Tinnitus is a sound precepted without an external sound stimulus.Its background can be categorised into primary and secondary cases.The secondary cases include pathologies of the external,middle and inner ear.Tinnitus can be objective or subjective;the latter can only identified by the sufferer.Previous research results have shown that tinnitus significantly affects the quality of life and daily functioning.Objectives:To analyse the impact of tinnitus on the daily functioning and the possible influence of demographical data and tinnitus duration on it.Methods:630 patients(265 males and 365 females,25e85 years of age)suffering from primary tinnitus were enrolled.In the Hungarian language,these patients completed the Tinnitus Handicap Inventory(THI)questionnaire and underwent a complete otorhinolaryngological examination.IBM SPSS V24 software was used for data processing;correlation tests,the Mann-Whitney U and Kruskal-Wallis nonparametric tests were used.Results:According to the THI questionnaires outcomes,most patients(62.5%)were presented with a mild handicap.Based on statistical analysis,no significant correlation was observed between the total THI points and the age of the patients,along with the duration and localisation of the symptoms.However,the total THI scores of male and female patients significantly differed,indicating higher THI values in the female group(p=0.00052*).Conclusions:The tinnitus severity was not affected by the duration,localisation of the symptoms and age but by gender,indicating higher values in the case of females.
文摘Waardenburg syndrome is a rare disease characterized by sensorineural deafness in association with pigmentary defects. Depending on additional symptoms, WS have been classified into four types.Waardenburg syndrome type 4, also called as Waardenburg Shah Syndrome is a very rare congenital disorder with astounding variable clinical expression, characterized by pigmentary abnormalities of the hair(A white forelock of hair, premature graying) and pigmentary changes of the iris such as heterochromia or homochromia irides, sensorineural deafness and Hirschsprung disease. Three genes have been bestowed so far in consociation with EDNRB, EDN3, and SOX10 genes. The pattern of inheritance is multifarious with the SOX10 mutation affiliation with autosomal dominant inheritance whereas the EDNRB and EDN3 genes are passed down in an autosomally recessive pattern.
文摘Endoscopic ear surgery(EES) is an exciting, rapidly developing and innovative field of otologic surgery.Technically and conceptually, EES is a significant departure from traditional microscopic transcanal approaches to the middle ear and canal that has shown very positive results with respect to patient outcomes. This review serves as a primer for the otologist and otology resident embarking on EES and discusses the theory surrounding the learning process, the optical chain for endoscopic surgery as well as other important underlying principles.
文摘Pediatric cholesteatoma occurs in one of two forms:congenital cholesteatoma,developing from embryonic epidermal cell rests or acquired cholesteatoma,associated with a focal defect in the tympanic membrane.This disease has been traditionally managed with the operating microscope,often requiring mastoidectomy for adequate visualization of and access to the middle ear and mastoid cavities.Recently,advances in endoscopic equipment have enabled otologists to manage most cases of pediatric cholesteatoma via a minimally-invasive,transcanal endoscopic approach.This review discusses the current literature relating to the etiopathogenesis,assessment and endoscopic management of pediatric cholesteatoma.Early outcomes of endoscopic treatment,emerging trends and technologies are also reviewed.
文摘Objective: To assess the outcomes of hearing and vertigo in patients with Meniere’s disease who were treated with intratympanic methylprednisolone. Study Design: Retrospective study. Setting: Tertiary referral hospital. Methods: In our study with 151 patients [191 ears] were diagnosed as Menieres as per 1995 AAO-HNS guidelines. As an office based procedure these patients were treated with intratympanic methyl prednisolone. Results: There was a 98.6% improvement in the vertigo control rate, a 73.82% improvement in hearing and 29.31% had a definitive improvement in tinnitus. Conclusion: We conclude from our study that intratympanic steroid injection significantly controls vertigo, tinnitus and improves hearing in Meniere’s disease with minimal complications. Hence, it can be tried as a first line treatment for patients with Meniere’s disease.
文摘BACKGROUND Craniometaphyseal dysplasia(CMD)is a rare genetic disorder.Autosomal dominant CMD(AD-CMD)is caused by mutations in the ANKH gene.Affected individuals typically have distinctive facial features including progressive thickening of the craniofacial bones.Treatment for AD-CMD primarily consists of surgical intervention to release compression of the cranial nerves and the brain stem/spinal cord.To alleviate progression of the clinical course and improve the quality of life in children waiting to undergo the necessary surgery,we investigated clinical changes in a diagnosed patient with AD-CMD over three years.CASE SUMMARY A 17-mo-old boy presented with progressive nasal obstruction,snoring and hearing loss symptoms.Physical examination showed enlargement of the head circumference and clinical features such as wide nasal bridge,paranasal bossing,widely spaced eyes with an increased bizygomatic width,and a prominent mandible.The patient underwent otolaryngological examination,endoscopy,hearing test,laboratory examination of phosphorus and bone metabolism,cranial and femoral computed tomography,X-ray and next-generation sequencing.The patient was diagnosed with AD-CMD due to p.Phe377 deletion(c.1129_1131del)on exon 9 of the ANKH gene.After adherence to a prescribed low-calcium diet,the boy’s alkaline phosphatase(ALP)levels continuously decreased to within the normal range.However,after 14 mo of dietary intervention,his parents altered his diet to an intermittent low-calcium diet to include milk and eggs.The patient’s ALP was slightly higher than normal after the dietary change but remained close to the normal range.His serum osteocalcin changed to within normal levels after dietary regulation for 33 mo.His serum combined beta C-terminal telopeptide of type I collagen also continuously decreased after the nutritional intervention,although still slightly higher than normal levels.Despite fluctuating blood test results,the boy’s nasal symptoms were markedly relieved and steadily improved after dietary intervention.No significant changes were found in the craniofacial bones by cranial radiography.Close monitoring of clinical features is still ongoing.Calcitriol treatment is currently under consideration and a surgical procedure is planned as necessary in the future.CONCLUSION We herein report the first Chinese case of AD-CMD with heterozygous mutation of p.Phe377 deletion(c.1129_1131del)on the ANKH gene.Biochemical alterations were significantly improved after dietary intervention indicating that a lowcalcium diet may be applied in pediatric AD-CMD patients with ANKH mutations to help alleviate phenotypic manifestations and improve the quality of life before surgical intervention.Further large scale studies are needed to replicate these findings and to establish the appropriate timing for nutritional and surgical interventions。
文摘Objective: The aim is to review the effectiveness of sialendoscopy in treating various salivary gland disorders related to the duct. Methods: A total of 133 patients who underwent sialendoscopy were included in this study. Data was collected from case records of patients and was analysed. A detailed history examination was taken and the operative findings were analysed. Causes of obstructive sialadenitis were evaluated and treated. Results: A total of 159 glands were subjected to sialendoscopy and pathology was identified in 149 glands. Forty one submandibular glands had calculi and twelve had non-calcular obstruction. Fifteen parotid glands had calculi and 81 had non-calcular obstruction, of which stricture was the most common. Calculi retrieval or dilation of stricture was achieved with a sialendoscope in 112 glands. In 23 patients, the calculi were retrieved by combined approach. In three patients, the calculi were fragmented with intra luminal Holmium laser and the fragments were removed. All these patients had a good relief from their symptoms. Duct perforation was seen in two patients and a false passage was formed in one. Conclusion: Sialendoscopy was a safe and effective procedure for managing ductal pathologies of salivary glands.
文摘Syringocystadenoma papilliferum(SCAP)is a rare benign skin tumor that develops from the apocrine or eccrine glands.Here,we report the case of a 2-year-old girl who presented with an asymptomatic,slowly growing lesion on her scalp.On physical examination,a 2 cm×1 cm red papillary plaque with a crusted surface was noted on her scalp.Histopathological examination revealed papillomatous expansion of the epidermis and cystic invaginations extending from the epidermis down to the deep dermis.The cystic structures were lined by papillae with two layers of columnar epithelium,which showed decapitation secretion.Based on the above clinicopathological findings,a diagnosis of congenital scalp SCAP was made.
基金funded by the National Key R&D Program of China(2021YFA1101804)the Strategic Priority Research Program of the Chinese Academy of Science(XDB32060100)+3 种基金the National Natural Science Foundation of China(91949119,82101212,and 82101209)a Shanghai Municipal Science and Technology Major Project(2018SHZDZX05)the Science and Technology Commission of Shanghai Municipality(21ZR1440200)the Shanghai Sailing Program(20YF1426400).
文摘The cochlear auditory epithelium contains two types of sound receptors,inner hair cells(IHCs)and outer hair cells(OHCs).Mouse models for labelling juvenile and adult IHCs or OHCs exist;however,labelling for embryonic and perinatal IHCs or OHCs are lacking.Here,we generated a new knock-in Fgf8^(P2A-3×GFP/+)(Fgf8^(GFP)/+)strain,in which the expression of a series of three GFP fragments is controlled by endogenous Fgf8 cis-regulatory elements.After confirming that GFP expression accurately reflects the expression of Fgf8,we successfully obtained both embryonic and neonatal IHCs with high purity,highlighting the power of Fgf8^(GFP)/+.Furthermore,our fate-mapping analysis revealed,unexpectedly,that IHCs are also derived from inner ear progenitors expressing Insm1,which is currently regarded as an OHC marker.Thus,besides serving as a highly favorable tool for sorting early IHCs,Fgf8^(GFP)/+will facilitate the isolation of pure early OHCs by excluding IHCs from the entire hair cell pool.
文摘Objectives:2019 novel coronavirus disease(COVID-19)infection is commonly associated with olfactory dysfunctions,but the basic pathogenesis of these complications remains controversial.This study seeks to evaluate the value of magnetic resonance spectroscopy(MRS)in determining the molecular neurometabolite alterations within the main brain olfactory areas in patients with COVID-19-related anosmia.Methods:In a cross-sectional study,seven patients with persistent COVID-19-related anosmia(mean age:29.57 years)and seven healthy volunteers(mean age:27.28 years)underwent MRS in which N-acetyl-aspartate(NAA),choline(Cho),creatine(Cr),and their ratios were measured in the anterior cingulate cortex,dorsolateral prefrontal cortex,orbitofrontal cortex(OFC),insular cortex,and ventromedial prefrontal cortex.Data were analyzed using TARQUIN software(version 4.3.10),and the results were compared with an independent sample t-test and nonparametric Mann-Whitney test based on the normality of the MRS data distribution.Results:The mean duration of anosmia before imaging was 8.5 months in COVID-19-related anosmia group.MRS analysis elucidated a significant association between MRS findings within OFC and COVID-19-related anosmia(P_(disease)<0.01),and NAA was among the most important neurometabolites(P_(interaction)=0.006).Reduced levels of NAA(P<0.001),Cr(P<0.001)and^(NAA)/Cho ratio(P=0.007)within OFC characterize COVID-19-related anosmia.Conclusions:This study emphasizes that MRS can be illuminating in COVID-19-related anosmia and indicates a possible association between central nervous system impairment and persistent COVID-19-related anosmia.
文摘Acoustic reflex is a sensitive indicator of middle ear lesions when the tympanic membrane is in-tact and tympanometry is normal. Acoustic reflex is not usually observed in conductive hearing loss caused by disruption of the ossicular chain including ossicular discontinuity and fixation. Ossicular disruption can show at least partially intact acoustic reflex under a certain condition. Moreover, ossicular discontinuity with a nearly intact acoustic reflex is quite rare and there have been few reports published to date. We here present a rare case of conductive hearing loss with a nearly intact acoustic reflex, and the patient was surgically confirmed to have ossicular discontinuity.
文摘A large variety of foreign bodies are swallowed by children, but the majority of those pass through the gastrointestinal tract without any adverse effects. The highest incidence of swallowed foreign bodies occurs in children between 6 months and 3 years. We reported to you an unusually large foreign body ingested by a 2-year-old male patient who underwent a rigid oesophagoscopy but the foreign body had to be removed by open surgical technique. It is advisable to have a multidisciplinary approach while dealing with such cases.