BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lac...BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lack of evidence hampers the acceptance of the technique in clinical practice.CASE SUMMARY A girl was taken to North Sichuan Medical University Affiliated Hospital for a hearing screen by her parents.Her parents reported that her hearing level was the same as when she was born.The girl was diagnosed with sensorineural hearing loss(SNHL)by a doctor in the otolaryngology department.After we introduced the foot reflexology project,the parents agreed to participate in the experiment.After 6 months of foot reflexology treatment,the hearing threshold of the girl recovered to a normal level,below 30 dB.CONCLUSION Foot reflexology should be encouraged in clinical practice and for families of infants with SNHL.展开更多
BACKGROUND Variants in the MYO7A gene commonly result in Usher syndrome,and in rare cases lead to autosomal dominant non-syndromic deafness(DFNA11).Currently,only nine variants have been reported to be responsible for...BACKGROUND Variants in the MYO7A gene commonly result in Usher syndrome,and in rare cases lead to autosomal dominant non-syndromic deafness(DFNA11).Currently,only nine variants have been reported to be responsible for DFNA11 and their clinical phenotypes are not identical.Here we present a novel variant causing DFNA11 identified in a three-generation Chinese family.CASE SUMMARY The proband was a 53-year-old Han male who presented with post-lingual bilateral symmetrical moderate sensorineural hearing loss.We learned from the patient’s medical history collection that multiple family members also had similar hearing loss,generally occurring around the age of 40.Subsequent investigation by high-throughput sequencing identified a novel MYO7A variant.To provide evidence supporting that this variant is responsible for the hearing loss in the studied family,we performed Sanger sequencing on 11 family members and found that the variant co-segregated with the deafness phenotype.In addition,the clinical manifestation of the 11 affected family members was found to be lateonset bilateral slowly progressive hearing loss,inherited in this family in an autosomal dominant manner.None of the affected family members had visual impairment or vestibular symptoms;therefore,we believe that this novel MYO7A variant is responsible for the rare DFNA11 in this family.CONCLUSION We report a novel variant leading to DFNA11 which further enriches the collection of MYO7A variants,and our review of the nine previous variants that have been identified to cause DFNA11 provides a reference for clinical genetic counseling.展开更多
BACKGROUND Brucellosis is one of the most common zoonotic infectious diseases in the world,with approximately 500000 new cases of human brucellosis diagnosed each year.Brucellosis can simulate various multi-system dis...BACKGROUND Brucellosis is one of the most common zoonotic infectious diseases in the world,with approximately 500000 new cases of human brucellosis diagnosed each year.Brucellosis can simulate various multi-system diseases,presenting atypical symptoms.Very few brucellosis cases with pancytopenia accompanied by a severe hearing loss have been reported.In the literature review,we could find only one similar case reported in the past.Moreover,this disease is easily misdiagnosed as a blood system disease leading to delayed treatment.Thus,it is important to improve clinicians'awareness of this disease.CASE SUMMARY A 64-year-old woman presented with dizziness and fatigue,accompanied by pancytopenia and severe hearing loss.Brucella melitensis was identified on blood culture.Anti-infective therapy with rifampicin(900 mg/d)and doxycycline(100 mg twice a day)was prescribed for 4 mo along with ceftriaxone 2 g/d for 1 mo.The patient showed a good response to antibiotic therapy.Her blood counts returned to normal followed by significant improvement in hearing.CONCLUSION Brucellosis should be considered in the differential diagnosis of patients presenting with pancytopenia and hearing loss.展开更多
BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early w...BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early warning sign of impending vertebrobasilar ischemic stroke.It is important to distinguish stroke from benign disease.CASE SUMMARY A 48-year-old male patient presented with SHL and vertigo as first symptoms.Diffusion-weighted imaging revealed high signal intensity in the left posterior inferior cerebellar artery territory of the cerebellar hemisphere and high signal intensity in the right pons and bridge cerebellar arm,confirming that the patient had cerebral infarction.Treatment with antiplatelet drugs,steroid antiinflammatory drugs,and neurotrophic nerve therapy promoted blood circulation and removed blood stasis,and the symptoms of the patient were significantly improved.CONCLUSION SHL and vertigo could be the initial symptoms of vertebrobasilar ischemic stroke.展开更多
To investigate the high-risk factors for newborn hearing loss and to provide information for preventing the development of hearing loss and delaying its progression, from May 2003 to June 2006, neonates who failed to ...To investigate the high-risk factors for newborn hearing loss and to provide information for preventing the development of hearing loss and delaying its progression, from May 2003 to June 2006, neonates who failed to pass the universal newborn hearing screening （UNHS） were referred to Jinan Newborn Hearing Screening and Rehabilitation Center from 7 newborn hearing screening centers in seven cities of Shandong province. One-to-one pair-matched case-control method was employed for statistical analysis of the basic features of definitely identified cases. High-risk factors relating to the bilateral hearing loss were evaluated by univariate and multivariate Logistic regression analysis. Our results revealed that 721 transferred newborns who didn＇t pass the heating screening received audiological and medical evaluation and 367 were confirmed to have hearing loss. Of them, 177 neonates with hearing loss who met the matching requirements were included in the study as subjects. Univariate analysis showed that high-risk factors related to hearing loss incuded age of father, education backgrounds of parents, parity, birth weight, gestational weeks, craniofacial deformity, history of receiving treatment in neonatal intensive care unit （NICU）, neonatal disease, family history of otopathy and family history of congenital hearing loss. Multivariate Logistic regression analysis revealed that 4 independent risk factors were related to bilateral hearing loss, including parity （OR=16.285, 95% CI 3.379--78,481）, neonatal disease （OR=34;968, 95% CI 2.720 449.534）, family history of congenital hearing loss （OR=69.488, 95% CI 4.417--1093.300） and birth weight （OR=0.241, 95% CI 0.090--0.648）. It is concluded that parity, neonatal disease and family history of heating loss are the promoting factors of bilateral hearing loss in neonates and appropriate intervention measures should be taken to deal with the risk factors.展开更多
BACKGROUND Cupping therapy is used across the globe since ancient Egypt.It is used as a complementary or alternative to daily clinical practice.Cupping therapy could be dry or wet(Al-hijamah)type.It is considered a re...BACKGROUND Cupping therapy is used across the globe since ancient Egypt.It is used as a complementary or alternative to daily clinical practice.Cupping therapy could be dry or wet(Al-hijamah)type.It is considered a relatively safe procedure for a variety of clinical problems like lower backache,shoulder pain,neck pain,headache,and migraine.Application of cupping therapy over body orifices is contraindicated.CASE SUMMARY A 27-year-old gentleman presented with sudden left-sided hearing loss and tinnitus for 7 d.He gave a history of upper respiratory tract infection 14 d before his complaints.He received Al-hijamah over his left ear 3 d before seeking advice from the Otolaryngology clinic,but without benefit.Physical examination showed a dull-looking left ear drum,hemotympanum in the posterior part of the tympanic cavity,two blood clots over the eardrum,and multiple bleeding points over the external ear canal and tympanic membrane.A pure tone audiogram and tympanogram confirmed the diagnosis of middle ear effusion.Conservative treatment(avoidance of water entry,antibiotics,and local and systemic decongestants)was given for 5 d.The presenting symptoms as well as ear injuries resolved completely two weeks after the follow-up.CONCLUSION We do not recommend using Al-hijamah directly over the ear as a treatment option for hearing loss to avoid unwanted ear trauma as well as there is no benefit from this kind of treatment.展开更多
BACKGROUND Hereditary spastic paraplegias (HSPs) refer to a group of heterogeneous neurodegenerative diseases characterized by lower limbs spasticity and weakness. So far, over 72 genes have been found to cause HSP (S...BACKGROUND Hereditary spastic paraplegias (HSPs) refer to a group of heterogeneous neurodegenerative diseases characterized by lower limbs spasticity and weakness. So far, over 72 genes have been found to cause HSP (SPG1-SPG72). Among autosomal dominant HSP patients, spastic paraplegia 4 (SPG4/SPAST) gene is the most common pathogenic gene, and atlastin-1 (ATL1) is the second most common one. Here we reported a novel ATL1 mutation in a Chinese spastic paraplegia 3A (SPG3A) family, which expands the clinical and genetic spectrum of ATL1 mutations. CASE SUMMARY A 9-year-old boy with progressive spastic paraplegia accompanied by right hearing loss and mental retardation for five years was admitted to our hospital.Past history was unremarkable. The family history was positive, and his grandfather and mother had similar symptoms. Neurological examinations revealed hypermyotonia in his lower limbs, hyperreflexia in knee reflex, bilateral positive Babinski signs and scissors gait. The results of blood routine test, liver function test, blood glucose test, ceruloplasmin test and vitamin test were all normal. The serum lactic acid level was significantly increased. The testing for brainstem auditory evoked potential demonstrated that the right side hearing was impaired while the left was normal. Magnetic resonance imaging showed mild atrophy of the spinal cord. The gene panel test revealed that the proband carried an ATL1 c.752A>G p.Gln251Arg (p.Q251R) mutation, and Sanger sequencing confirmed the existence of family co-segregation. CONCLUSION We reported a novel ATL1 Q251R mutation and a novel clinical phenotype of hearing loss in a Chinese SPG3A family.展开更多
BACKGROUND Sporadic Creutzfeldt-Jakob disease(s CJD)is a prion disease characterized as a fatal transmissible neurodegenerative disorder.Dizziness is often the first presenting symptom of s CJD,but hearing loss as an ...BACKGROUND Sporadic Creutzfeldt-Jakob disease(s CJD)is a prion disease characterized as a fatal transmissible neurodegenerative disorder.Dizziness is often the first presenting symptom of s CJD,but hearing loss as an early manifestation is very rare.CASE SUMMARY A 76-year-old man presented with bilateral sudden hearing impairment and dizziness for 10 d.He was taking medications for hypertension and diabetes.He denied any difficulty with activities of daily living or hearing impairment before the onset of symptoms.Pure tone audiometry showed bilateral severe hearing impairment.Brain magnetic resonance imaging(MRI)and laboratory tests were within normal limits.Given his diagnosis of sudden sensory hearing loss,the patient received corticosteroid treatment but it was ineffective.Two weeks later,he complained of aggravated gait impairment,disorientation,and cognitive impairment.Repeat brain MRI showed diffuse cortical high signal intensities on diffusion-weighted imaging.In cerebrospinal fluid analysis,the real-time quaking-induced conversion assay was positive,and 14-3-3 protein was detected in the by western blotting.Considering all the data,we diagnosed probable s CJD,and the patient’s symptoms rapidly progressed into akinetic mutism.CONCLUSION For patients with abrupt bilateral hearing impairment,especially in the elderly,various differential diagnoses,including s CJD,should be considered.展开更多
BACKGROUND Stapedial tendon ossification is a rare disease,with only a few reports.The stapedial tendon originates from the apex of the pyramidal eminence and is attached to the neck of the stapes.In stapedial tendon ...BACKGROUND Stapedial tendon ossification is a rare disease,with only a few reports.The stapedial tendon originates from the apex of the pyramidal eminence and is attached to the neck of the stapes.In stapedial tendon ossification,the stapes is fixed,causing conductive hearing loss.In most cases,complete hearing restoration is achieved by dividing the stapedial tendon after exploratory tympanotomy.CASE SUMMARY A 28-year-old woman presented to our hospital with the major complaint of bilateral hearing loss that started during childhood.Exploratory tympanotomy was performed due to suspicion of otosclerosis or middle ear anomalies.We found bilateral conductive hearing loss due to stapedial tendon ossification with a middle ear anomaly during surgery.There have been several reports of complete recovery of hearing after resection of the stapedial tendon.However,in this case,recovery of hearing was insufficient,even with the division of the stapedial tendon.In the second surgery,the stapes anomaly and footplate fixation were confirmed,and hearing was completely recovered after stapedotomy.Therefore,we report this case with a review of the relevant literature.CONCLUSION This is the first case of stapedial tendon ossification and fixation of the footplate surgically diagnosed on both sides.With surgical treatment,successful results are expected.展开更多
Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered p...Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered poor prognostic factors in SSNHL[2-4].The most common diseases associated with vertigo in SSNHL include BPPV,vestibular neu-[3]展开更多
BACKGROUND The auditory brainstem implant(ABI)is a significant treatment to restore hearing sensations for neurofibromatosis type 2(NF2)patients.However,there is no ideal method in assisting the placement of ABIs.In t...BACKGROUND The auditory brainstem implant(ABI)is a significant treatment to restore hearing sensations for neurofibromatosis type 2(NF2)patients.However,there is no ideal method in assisting the placement of ABIs.In this case series,intraoperative cochlear nucleus mapping was performed in awake craniotomy to help guide the placement of the electrode array.CASE SUMMARY We applied the asleep-awake-asleep technique for awake craniotomy and hearing test via the retrosigmoid approach for acoustic neuroma resections and ABIs,using mechanical ventilation with a laryngeal mask during the asleep phases,utilizing a ropivacaine-based regional anesthesia,and sevoflurane combined with propofol/remifentanil as the sedative/analgesic agents in four NF2 patients.ABI electrode arrays were placed in the awake phase with successful intraoperative hearing tests in three patients.There was one uncooperative patient whose awake hearing test needed to be aborted.In all cases,tumor resection and ABI were performed safely.Satisfactory electrode effectiveness was achieved in awake ABI placement.CONCLUSION This case series suggests that awake craniotomy with an intraoperative hearing test for ABI placement is safe and well tolerated.Awake craniotomy is beneficial for improving the accuracy of ABI electrode placement and meanwhile reduces non-auditory side effects.展开更多
基金Graduate Student Project of Xi’an International Studies University,No.2021BS012Nanchong City-Universities Project,No.22SXCXTD0004.
文摘BACKGROUND Our study contributes to the further understanding of the mechanism of foot reflexology.Foot reflexology has been reported to affect hearing recovery,but no physiological evidence has been provided.This lack of evidence hampers the acceptance of the technique in clinical practice.CASE SUMMARY A girl was taken to North Sichuan Medical University Affiliated Hospital for a hearing screen by her parents.Her parents reported that her hearing level was the same as when she was born.The girl was diagnosed with sensorineural hearing loss(SNHL)by a doctor in the otolaryngology department.After we introduced the foot reflexology project,the parents agreed to participate in the experiment.After 6 months of foot reflexology treatment,the hearing threshold of the girl recovered to a normal level,below 30 dB.CONCLUSION Foot reflexology should be encouraged in clinical practice and for families of infants with SNHL.
文摘BACKGROUND Variants in the MYO7A gene commonly result in Usher syndrome,and in rare cases lead to autosomal dominant non-syndromic deafness(DFNA11).Currently,only nine variants have been reported to be responsible for DFNA11 and their clinical phenotypes are not identical.Here we present a novel variant causing DFNA11 identified in a three-generation Chinese family.CASE SUMMARY The proband was a 53-year-old Han male who presented with post-lingual bilateral symmetrical moderate sensorineural hearing loss.We learned from the patient’s medical history collection that multiple family members also had similar hearing loss,generally occurring around the age of 40.Subsequent investigation by high-throughput sequencing identified a novel MYO7A variant.To provide evidence supporting that this variant is responsible for the hearing loss in the studied family,we performed Sanger sequencing on 11 family members and found that the variant co-segregated with the deafness phenotype.In addition,the clinical manifestation of the 11 affected family members was found to be lateonset bilateral slowly progressive hearing loss,inherited in this family in an autosomal dominant manner.None of the affected family members had visual impairment or vestibular symptoms;therefore,we believe that this novel MYO7A variant is responsible for the rare DFNA11 in this family.CONCLUSION We report a novel variant leading to DFNA11 which further enriches the collection of MYO7A variants,and our review of the nine previous variants that have been identified to cause DFNA11 provides a reference for clinical genetic counseling.
文摘BACKGROUND Brucellosis is one of the most common zoonotic infectious diseases in the world,with approximately 500000 new cases of human brucellosis diagnosed each year.Brucellosis can simulate various multi-system diseases,presenting atypical symptoms.Very few brucellosis cases with pancytopenia accompanied by a severe hearing loss have been reported.In the literature review,we could find only one similar case reported in the past.Moreover,this disease is easily misdiagnosed as a blood system disease leading to delayed treatment.Thus,it is important to improve clinicians'awareness of this disease.CASE SUMMARY A 64-year-old woman presented with dizziness and fatigue,accompanied by pancytopenia and severe hearing loss.Brucella melitensis was identified on blood culture.Anti-infective therapy with rifampicin(900 mg/d)and doxycycline(100 mg twice a day)was prescribed for 4 mo along with ceftriaxone 2 g/d for 1 mo.The patient showed a good response to antibiotic therapy.Her blood counts returned to normal followed by significant improvement in hearing.CONCLUSION Brucellosis should be considered in the differential diagnosis of patients presenting with pancytopenia and hearing loss.
文摘BACKGROUND Sudden hearing loss(SHL)is associated with serious systematic conditions such as neoplasms,vascular events,autoimmune diseases,infections,and iatrogenic injury.Some authors report that SHL can be an early warning sign of impending vertebrobasilar ischemic stroke.It is important to distinguish stroke from benign disease.CASE SUMMARY A 48-year-old male patient presented with SHL and vertigo as first symptoms.Diffusion-weighted imaging revealed high signal intensity in the left posterior inferior cerebellar artery territory of the cerebellar hemisphere and high signal intensity in the right pons and bridge cerebellar arm,confirming that the patient had cerebral infarction.Treatment with antiplatelet drugs,steroid antiinflammatory drugs,and neurotrophic nerve therapy promoted blood circulation and removed blood stasis,and the symptoms of the patient were significantly improved.CONCLUSION SHL and vertigo could be the initial symptoms of vertebrobasilar ischemic stroke.
基金This project is supported by a grant from the National Natural Sciences Foundation (No. 30100207)a grant from the Top Ten Scientific Research Programs of Shandong Province (No. 2004GG3202003)
文摘To investigate the high-risk factors for newborn hearing loss and to provide information for preventing the development of hearing loss and delaying its progression, from May 2003 to June 2006, neonates who failed to pass the universal newborn hearing screening （UNHS） were referred to Jinan Newborn Hearing Screening and Rehabilitation Center from 7 newborn hearing screening centers in seven cities of Shandong province. One-to-one pair-matched case-control method was employed for statistical analysis of the basic features of definitely identified cases. High-risk factors relating to the bilateral hearing loss were evaluated by univariate and multivariate Logistic regression analysis. Our results revealed that 721 transferred newborns who didn＇t pass the heating screening received audiological and medical evaluation and 367 were confirmed to have hearing loss. Of them, 177 neonates with hearing loss who met the matching requirements were included in the study as subjects. Univariate analysis showed that high-risk factors related to hearing loss incuded age of father, education backgrounds of parents, parity, birth weight, gestational weeks, craniofacial deformity, history of receiving treatment in neonatal intensive care unit （NICU）, neonatal disease, family history of otopathy and family history of congenital hearing loss. Multivariate Logistic regression analysis revealed that 4 independent risk factors were related to bilateral hearing loss, including parity （OR=16.285, 95% CI 3.379--78,481）, neonatal disease （OR=34;968, 95% CI 2.720 449.534）, family history of congenital hearing loss （OR=69.488, 95% CI 4.417--1093.300） and birth weight （OR=0.241, 95% CI 0.090--0.648）. It is concluded that parity, neonatal disease and family history of heating loss are the promoting factors of bilateral hearing loss in neonates and appropriate intervention measures should be taken to deal with the risk factors.
文摘BACKGROUND Cupping therapy is used across the globe since ancient Egypt.It is used as a complementary or alternative to daily clinical practice.Cupping therapy could be dry or wet(Al-hijamah)type.It is considered a relatively safe procedure for a variety of clinical problems like lower backache,shoulder pain,neck pain,headache,and migraine.Application of cupping therapy over body orifices is contraindicated.CASE SUMMARY A 27-year-old gentleman presented with sudden left-sided hearing loss and tinnitus for 7 d.He gave a history of upper respiratory tract infection 14 d before his complaints.He received Al-hijamah over his left ear 3 d before seeking advice from the Otolaryngology clinic,but without benefit.Physical examination showed a dull-looking left ear drum,hemotympanum in the posterior part of the tympanic cavity,two blood clots over the eardrum,and multiple bleeding points over the external ear canal and tympanic membrane.A pure tone audiogram and tympanogram confirmed the diagnosis of middle ear effusion.Conservative treatment(avoidance of water entry,antibiotics,and local and systemic decongestants)was given for 5 d.The presenting symptoms as well as ear injuries resolved completely two weeks after the follow-up.CONCLUSION We do not recommend using Al-hijamah directly over the ear as a treatment option for hearing loss to avoid unwanted ear trauma as well as there is no benefit from this kind of treatment.
基金Supported by National Natural Science Foundation of China,No.81171068
文摘BACKGROUND Hereditary spastic paraplegias (HSPs) refer to a group of heterogeneous neurodegenerative diseases characterized by lower limbs spasticity and weakness. So far, over 72 genes have been found to cause HSP (SPG1-SPG72). Among autosomal dominant HSP patients, spastic paraplegia 4 (SPG4/SPAST) gene is the most common pathogenic gene, and atlastin-1 (ATL1) is the second most common one. Here we reported a novel ATL1 mutation in a Chinese spastic paraplegia 3A (SPG3A) family, which expands the clinical and genetic spectrum of ATL1 mutations. CASE SUMMARY A 9-year-old boy with progressive spastic paraplegia accompanied by right hearing loss and mental retardation for five years was admitted to our hospital.Past history was unremarkable. The family history was positive, and his grandfather and mother had similar symptoms. Neurological examinations revealed hypermyotonia in his lower limbs, hyperreflexia in knee reflex, bilateral positive Babinski signs and scissors gait. The results of blood routine test, liver function test, blood glucose test, ceruloplasmin test and vitamin test were all normal. The serum lactic acid level was significantly increased. The testing for brainstem auditory evoked potential demonstrated that the right side hearing was impaired while the left was normal. Magnetic resonance imaging showed mild atrophy of the spinal cord. The gene panel test revealed that the proband carried an ATL1 c.752A>G p.Gln251Arg (p.Q251R) mutation, and Sanger sequencing confirmed the existence of family co-segregation. CONCLUSION We reported a novel ATL1 Q251R mutation and a novel clinical phenotype of hearing loss in a Chinese SPG3A family.
基金Supported by National Research Foundation of Korea,No.2019R1C1C1006539。
文摘BACKGROUND Sporadic Creutzfeldt-Jakob disease(s CJD)is a prion disease characterized as a fatal transmissible neurodegenerative disorder.Dizziness is often the first presenting symptom of s CJD,but hearing loss as an early manifestation is very rare.CASE SUMMARY A 76-year-old man presented with bilateral sudden hearing impairment and dizziness for 10 d.He was taking medications for hypertension and diabetes.He denied any difficulty with activities of daily living or hearing impairment before the onset of symptoms.Pure tone audiometry showed bilateral severe hearing impairment.Brain magnetic resonance imaging(MRI)and laboratory tests were within normal limits.Given his diagnosis of sudden sensory hearing loss,the patient received corticosteroid treatment but it was ineffective.Two weeks later,he complained of aggravated gait impairment,disorientation,and cognitive impairment.Repeat brain MRI showed diffuse cortical high signal intensities on diffusion-weighted imaging.In cerebrospinal fluid analysis,the real-time quaking-induced conversion assay was positive,and 14-3-3 protein was detected in the by western blotting.Considering all the data,we diagnosed probable s CJD,and the patient’s symptoms rapidly progressed into akinetic mutism.CONCLUSION For patients with abrupt bilateral hearing impairment,especially in the elderly,various differential diagnoses,including s CJD,should be considered.
文摘BACKGROUND Stapedial tendon ossification is a rare disease,with only a few reports.The stapedial tendon originates from the apex of the pyramidal eminence and is attached to the neck of the stapes.In stapedial tendon ossification,the stapes is fixed,causing conductive hearing loss.In most cases,complete hearing restoration is achieved by dividing the stapedial tendon after exploratory tympanotomy.CASE SUMMARY A 28-year-old woman presented to our hospital with the major complaint of bilateral hearing loss that started during childhood.Exploratory tympanotomy was performed due to suspicion of otosclerosis or middle ear anomalies.We found bilateral conductive hearing loss due to stapedial tendon ossification with a middle ear anomaly during surgery.There have been several reports of complete recovery of hearing after resection of the stapedial tendon.However,in this case,recovery of hearing was insufficient,even with the division of the stapedial tendon.In the second surgery,the stapes anomaly and footplate fixation were confirmed,and hearing was completely recovered after stapedotomy.Therefore,we report this case with a review of the relevant literature.CONCLUSION This is the first case of stapedial tendon ossification and fixation of the footplate surgically diagnosed on both sides.With surgical treatment,successful results are expected.
文摘Introduction Sudden sensorineural hearing loss(SSNHL)is defined as a hearing loss of at least 30 dB over three contiguous frequencies occurring in less than 3 days[1].Vertigo and profound hearing loss are considered poor prognostic factors in SSNHL[2-4].The most common diseases associated with vertigo in SSNHL include BPPV,vestibular neu-[3]
基金Beijing Municipal Administration of Hospitals Ascent Plan,No.DFL20180502.
文摘BACKGROUND The auditory brainstem implant(ABI)is a significant treatment to restore hearing sensations for neurofibromatosis type 2(NF2)patients.However,there is no ideal method in assisting the placement of ABIs.In this case series,intraoperative cochlear nucleus mapping was performed in awake craniotomy to help guide the placement of the electrode array.CASE SUMMARY We applied the asleep-awake-asleep technique for awake craniotomy and hearing test via the retrosigmoid approach for acoustic neuroma resections and ABIs,using mechanical ventilation with a laryngeal mask during the asleep phases,utilizing a ropivacaine-based regional anesthesia,and sevoflurane combined with propofol/remifentanil as the sedative/analgesic agents in four NF2 patients.ABI electrode arrays were placed in the awake phase with successful intraoperative hearing tests in three patients.There was one uncooperative patient whose awake hearing test needed to be aborted.In all cases,tumor resection and ABI were performed safely.Satisfactory electrode effectiveness was achieved in awake ABI placement.CONCLUSION This case series suggests that awake craniotomy with an intraoperative hearing test for ABI placement is safe and well tolerated.Awake craniotomy is beneficial for improving the accuracy of ABI electrode placement and meanwhile reduces non-auditory side effects.
