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BACKGROUND: Outcomes of surgical management of obstructive sleep apnea-hypopnea syndrome (OSAHS) can be difficult to predict preoperatively. AIMS/OBJECTIVES: To study the effect of applying traction to the velum during drug-induced sleep endoscopy (DISE) in OSAHS patients, and to describe the use of traction velum in predicting surgical success. MATERIALS AND METHODS: 41 adult surgical patients with OSA (Friedman tongue position II and III) were evaluated by DISE. All patients subsequently underwent velopharyngeal surgery in the form of uvulopalatopharyngoplasty with tonsillectomy plus barbed reposition pharyngoplasty. RESULTS: Surgical responders (n = 26, 63.4%) and nonresponders (n = 15, 36.6%) demonstrated no significant differences with regard to preoperative AHI, age, sex, body mass index, and mean/lowest O2 saturation. Responders had a marked decrease in desaturation events (2.96 vs 0.03, p < 0.001) and percentage change in number of desaturation events before/after traction velum (56.7 vs 4.5, p < 0.001). Regression analysis revealed that reduction in mean desaturation events, with traction velum, by a percentage > 26.8% (odds ratio [OR] 1.046; 95% confidence interval [CI] 1.018-1.075; p = 0.001) was the only independent predictor of surgery success. CONCLUSIONS AND SIGNIFICANCE: OSAHS patients' velopharyngeal surgical outcome can be predicted by measuring the percentage change in the number of oxygen desaturation events before and after traction velum in DISE.
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Tração , Endoscopia , Humanos , Preparações Farmacêuticas , Polissonografia , Sono , Resultado do TratamentoRESUMO
BACKGROUND: Several otologic conditions can present with fluctuating sensorineural hearing loss, including Ménière's disease, autoimmune inner ear disease, and enlarged vestibular aqueduct. Although these 3 etiologies vary greatly, distinguishing between these conditions at initial presentation can be challenging. Furthermore, initial treatment of these conditions is often similar. In this review, we discuss historical and current perspectives on diagnosis and treatment of these conditions. SUMMARY: A literature search was performed regarding fluctuating hearing loss, and current treatment of these etiologies of fluctuating hearing loss was summarized. Immediate measures at the onset of acute hearing loss include corticosteroid therapy, while preventative and chronic therapies, which can limit disease severity and frequency, vary based on the specific condition treated. Key Messages: Fluctuating hearing loss can represent a range of pathologies, but the precise etiology may not be clear at initial presentation. Timely treatment and long-term follow-up, along with appropriate diagnostics, are necessary to optimize long-term hearing.
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Hidropisia Endolinfática/complicações , Perda Auditiva Neurossensorial/etiologia , Doença de Meniere/complicações , Hidropisia Endolinfática/terapia , Perda Auditiva Neurossensorial/terapia , Testes Auditivos , Humanos , Doença de Meniere/terapiaRESUMO
Hereditary hemorrhagic telangiectasia (HHT) is an inherited vascular disorder which manifests as recurrent, episodic, and potentially debilitating epistaxis. In this study, we aim to (1) characterize baseline sinonasal symptoms for HHT patients and to (2) analyze changes in sinonasal symptoms before and after laser surgical treatment for HHT. We performed a retrospective chart review of sinonasal outcome test-22 (SNOT-22) scores before and after one or more laser surgical treatments for HHT-related epistaxis between January 1, 2010 and December 1, 2015 at a tertiary academic medical center with an HHT Foundation-approved Center of Excellence. Preoperative and all subsequent postoperative SNOT-22 scores (short-term, <45 days and long-term, ≥45 days) were compared. Twenty consecutive HHT patients underwent 51 laser surgeries for recurrent epistaxis. Mean preoperative, short-term postoperative, and long-term postoperative SNOT-22 scores were 34.6 ± 5.4, 33.9 ± 5.5, and 18.8 ± 4.6, respectively. When analyzing subcategory scores, there was a significant improvement in the rhinologic domain from short-term to long-term postoperatively (13.5 vs. 7.3; p = 0.004), in the non-rhinologic otolaryngic domain from short-term to long-term postoperatively (2.8 vs. 1.7; p = 0.014), and in the psychological domain from preoperative and short-term postoperative to long-term postoperatively (12.2 and 10.0 vs. 6.0; p = 0.015 and 0.01, respectively). Following laser surgery for HHT-related epistaxis, patients' rhinologic symptoms worsened on the short run postoperatively but improved over time. The main benefit of laser treatment appears to be long-term improvement in psychological factors. This study once again underscores the important role of the otolaryngologist in managing sinonasal manifestations of HHT.
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Epistaxe/etiologia , Lasers de Estado Sólido/uso terapêutico , Fotocoagulação/métodos , Qualidade de Vida , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
PURPOSE: Angiosarcoma of the tongue is an exceedingly rare malignancy of the head and neck. Such lesions can be primary in nature or occur in a previously irradiated field. We examine a series of cases with relation to clinical presentation, diagnosis, management, and outcomes. MATERIALS AND METHODS: Retrospective chart review of all patients with angiosarcoma of the tongue at a tertiary academic institution yielded a single case between 2005 and 2016. The MEDLINE database was additionally searched for all case series or reports of angiosarcoma arising in the tongue, and pertinent clinical data were extracted. RESULTS: The clinical presentation, disease course, and management of a patient with angiosarcoma of the tongue are presented. Institutional and literature search yielded a total of eight patients with angiosarcoma of the tongue. The most common primary sites were dorsal and lateral oral tongue. Treatment consisted of surgical resection in 63% of cases with adjuvant therapy administered in 75% of cases. Follow-up times varied per patient, but 63% had persistent or recurrent disease and 67% died of or with disease within two years of index presentation. CONCLUSION: Angiosarcoma of the tongue is a rare and highly aggressive tumor, accounting for fewer than 1% of all head and neck malignancies. The mainstay of treatment is surgical resection with negative margins followed by adjuvant chemoradiation for high-risk features. Due to rarity of the disease, consensus on optimal treatment approach is lacking, and multi-center prospective studies would be helpful to set clinical guidelines.
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Hemangiossarcoma/diagnóstico , Hemangiossarcoma/terapia , Neoplasias da Língua/diagnóstico , Neoplasias da Língua/terapia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJECTIVE: To describe an experience with laryngeal oncocytic cystadenomas and review the published literature regarding this uncommon diagnosis. METHODS AND RESULTS: A clinical review of patients presenting with cystic laryngeal masses in an urban academic medical center between January and December 2013 was performed. Three patients, two female and one male, with a mean age of 68 years, were diagnosed with oncocytic cystadenomata of the larynx. Major presenting symptoms included dysphonia, globus, and ipsilateral otalgia. Endoscopic examinations revealed a cystic structure arising from varied subsites of the larynx: laryngeal ventricle, aryepiglottic fold, and pre-epiglottic space. Cross-sectional radiographic imaging was obtained in each case. The patients were treated with transoral (CO2) laser microsurgery (TLM). In all three cases, pathological analysis revealed oncocytic cystadenoma with clear margins. CONCLUSIONS: Oncocytic cystadenoma is a rare entity of the larynx predominantly affecting elderly patients. Clinical presentation and imaging may suggest the diagnosis of an internal laryngocele. Complete excision is both diagnostic and therapeutic, and typically can be achieved using TLM. When clear margins are obtained, no adjuvant therapy is indicated. Although laryngoceles and malignancies are more commonly encountered, oncocytic cystadenomas should remain in the differential of cystic laryngeal masses.
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Cistadenoma/diagnóstico , Neoplasias Laríngeas/diagnóstico , Idoso , Cistadenoma/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Neoplasias Laríngeas/cirurgia , Laringocele/diagnóstico , Laringoscopia , Terapia a Laser , MasculinoRESUMO
OBJECTIVE: This study aimed to describe management of esophageal stenosis after chemoradiation therapy for head and neck squamous cell carcinoma (HNSCC), with particular emphasis on techniques and outcomes with the use of the transnasal esophagoscope (TNE) in the office as well as operating room settings. METHODS: Retrospective analysis of all patients with esophageal stenosis following head and neck cancer radiation, with or without chemotherapy, and managed with TNE-assisted esophageal dilation over a 5-year period. Preoperative and postoperative swallowing function were assessed objectively with the Functional Outcome Swallowing Scale (FOSS; ranging from score 0, a normal diet, to score 5, complete dependence on nonoral nutrition). RESULTS: Twenty-five patients met inclusion criteria. The mean pretreatment FOSS score was 4.4, whereas the mean posttreatment FOSS score was 2.7 (Wilcoxon signed-rank test, P<.001). Prior to dilation, 16 patients were completely gastrostomy-tube dependent (FOSS 5), of whom 12 (75%) were able to tolerate oral nutrition for a majority of their diet following treatment according to our protocol. No complications were noted. CONCLUSION: Dysphagia following chemoradiation therapy for HNSCC is often related to esophageal stenosis. With the aid of TNE, we have developed a successful treatment strategy for esophageal stenosis with improved success rates.
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Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/radioterapia , Dilatação/métodos , Estenose Esofágica/terapia , Esofagoscopia/métodos , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimiorradioterapia/efeitos adversos , Deglutição/fisiologia , Estenose Esofágica/etiologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Nariz , Estudos Retrospectivos , Carcinoma de Células Escamosas de Cabeça e Pescoço , Resultado do TratamentoRESUMO
OBJECTIVES: Acromegalics present with a wide range of otolaryngic symptoms, including rhinosinusitis, changes in facial appearance, obstructive sleep apnea (OSA), and voice disturbances. Treatment typically involves transnasal-transsphenoidal (TNTS) resection of the offending pituitary adenoma. In this study, we identify the prevalence of otolaryngic symptoms of acromegalic patients, and evaluate Sinonasal Outcome Test (SNOT-22) scores preceding and following pituitary resection. DESIGN: Retrospective chart review. SETTING: Tertiary academic medical center. PARTICIPANTS: Patients diagnosed with acromegaly who underwent surgical resection of a growth-hormone secreting pituitary adenoma between August 2010 and September 2013. MAIN OUTCOME MEASURES: Subjects were asked to complete questionnaires detailing otolaryngic symptoms as well as SNOT-22 surveys before and after TNTS surgery. A Student's t-test was used to compare preoperative and postoperative SNOT-22 scores. RESULTS: Twenty-five patients underwent pituitary surgery for acromegaly. Acromegalic patients were found to have macroglossia (60%), OSA or sleep-disordered breathing (52%), thyroid neoplasia (20%), hearing loss/tinnitus (20%), sinonasal symptoms (16%), and parathyroid pathology (8%). Differences in preoperative and postoperative SNOT-22 scores were not statistically significant. CONCLUSION: Acromegalics present with assorted otolaryngic complaints. Routine screening of all acromegalics with sleep evaluations (for both surgical and perioperative planning), thyroid ultrasound, and audiologic testing should be strongly considered.
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Acromegalia/complicações , Adenoma/diagnóstico , Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico , Procedimentos Neurocirúrgicos/efeitos adversos , Otorrinolaringopatias/etiologia , Acromegalia/diagnóstico , Adenoma/complicações , Adenoma/cirurgia , Adolescente , Adulto , Idoso , Feminino , Adenoma Hipofisário Secretor de Hormônio do Crescimento/complicações , Adenoma Hipofisário Secretor de Hormônio do Crescimento/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Cirurgia Endoscópica por Orifício Natural/efeitos adversos , Cirurgia Endoscópica por Orifício Natural/métodos , Otorrinolaringopatias/diagnóstico , Estudos Retrospectivos , Adulto JovemRESUMO
PURPOSE: Basaloid squamous cell carcinoma (BSCC) is a rare variant of squamous cell carcinoma characterized by a highly aggressive clinical course. Though typically found in the larynx, oropharynx, and hypopharynx, we report a rare case of BSCC originating in the maxillary sinus in an otherwise healthy 32-year-old male. MATERIALS AND METHODS: Single case report of a patient with BSCC of the maxillary sinus and retrospective chart review of all cases of BSCC of the maxilla at a single academic institution between January 1, 1986 and December 31, 2013. The MEDLINE database was additionally queried for all case series or reports of BSCC arising in the maxilla, and pertinent clinical data were extracted. RESULTS: The clinical presentation, disease course, and management of a patient with BSCC of the maxilla are presented. In this recent case, the patient presented with persistent alveolar pain and a nonhealing tooth infection. Radiographic studies demonstrated a large necrotic mass in the left maxillary sinus that was biopsy-proven as BSCC. The patient underwent surgical resection followed by postoperative radiation without complications. CONCLUSIONS: BSCC of the maxilla is a rare oncologic entity that may progress to late disease stage without obvious clinical signs or symptoms. Optimal treatment involves complete surgical resection followed by postoperative.
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Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/terapia , Neoplasias do Seio Maxilar/diagnóstico , Neoplasias do Seio Maxilar/terapia , Adulto , Humanos , MasculinoRESUMO
PURPOSE: We sought to examine prognostic and therapeutic implications, including cost-effectiveness, of elective neck dissection in the management of patients with clinically-determined T1N0 oral tongue carcinoma. MATERIALS AND METHODS: A retrospective review of patients with cT1N0 oral tongue squamous cell carcinoma who underwent surgical extirpation of primary tumor, with or without elective neck dissection, at UCLA Medical Center from 1990 to 2009 was performed. Cox proportional hazards regression was used to assess effects of variables on time to first loco-regional recurrence. A healthcare costs analysis of elective neck dissection was performed by querying the SEER-Medicare linked database. RESULTS: Of the 123 patients identified with cT1N0 squamous cell carcinoma of the oral tongue, 88 underwent elective neck dissection at the time of tumor resection while 35 did not. For all patients, disease-free survival at 3, 5, and 10 years was 93%, 82%, and 79%. Of the 88 patients undergoing elective neck dissection, 20 (23%) demonstrated occult metastatic disease. Male gender, tumor size, perineural invasion, and occult metastatic disease were individually associated with higher rates of loco-regional recurrence. There was no significant difference in loco-regional recurrence between those who underwent elective neck dissection and those who did not (HR=0.76, p=0.52). On cost analysis, neck dissection was not associated with any significant difference in Medicare payments. CONCLUSIONS: The high rate of occult metastasis (23%) following elective neck dissection, which did not confer additional healthcare costs, leads to the recommendation of elective neck dissection in patients with cT1N0 oral tongue squamous cell carcinoma.
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Carcinoma de Células Escamosas/cirurgia , Neoplasias de Cabeça e Pescoço/cirurgia , Esvaziamento Cervical , Neoplasias da Língua/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas/economia , Carcinoma de Células Escamosas/mortalidade , Carcinoma de Células Escamosas/patologia , Efeitos Psicossociais da Doença , Feminino , Neoplasias de Cabeça e Pescoço/economia , Neoplasias de Cabeça e Pescoço/mortalidade , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Medicare , Pessoa de Meia-Idade , Invasividade Neoplásica , Prognóstico , Estudos Retrospectivos , Carcinoma de Células Escamosas de Cabeça e Pescoço , Neoplasias da Língua/economia , Neoplasias da Língua/mortalidade , Neoplasias da Língua/patologia , Estados UnidosRESUMO
ABSTRACT: Cochlear spiral ganglion neurons (SGNs) are bipolar ganglion cells and are the first neurons in the auditory transduction pathway. They transmit complex acoustic information from hair cells to second-order sensory neurons in the cochlear nucleus for sound processing. Injury to SGNs causes largely irreversible hearing impairment because these neurons are highly differentiated cells and cannot regenerate, making treatment of sensorineural hearing loss (SNHL) arising from SGN injury difficult. When exposed to ototoxic drugs or damaging levels of noise or when there is loss of neurotrophic factors (NTFs), aging, and presence of other factors, SGNs can be irreversibly damaged, resulting in SNHL. It has been found that NTFs and stem cells can induce regeneration among dead spiral ganglion cells. In this paper, we summarized the present knowledge regarding injury, protection, and regeneration of SGNs.
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Perda Auditiva Neurossensorial , Gânglio Espiral da Cóclea , Humanos , Gânglio Espiral da Cóclea/metabolismo , Neurônios , Cóclea , Células Ciliadas Auditivas/metabolismoRESUMO
OBJECTIVE: The goal of this study was to evaluate the feasibility of a minimally invasive approach to the middle cranial fossa using a novel endaural keyhole. METHODS: The charts of all patients who underwent this novel minimally invasive approach to the middle cranial fossa were retrospectively reviewed. In addition, cadaveric dissection was performed to demonstrate the feasibility of the endaural keyhole to the middle cranial fossa. RESULTS: Six patients (5 female and 1 male; age range 47-77 years) who underwent craniotomy for CSF leak (n = 3), intracerebral hematoma evacuation (n = 2), and tumor resection (n = 1) via the endaural subtemporal approach were identified. There were no approach-related complications noted. Representative imaging from cadaveric dissection is provided with a stepwise discussion of the procedure. CONCLUSIONS: The endaural subtemporal keyhole craniotomy provides a novel approach to middle fossa skull base pathology, as well as a minimally invasive approach to intra-axial pathology of the temporal lobe and basal ganglia. Further research is needed to establish the limitations and potential complications of this novel approach.
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We present a case of a 58-year-old male with type II diabetes managed with metformin and insulin, who presented to the clinic with left chronic otitis media, persistent drainage, a stenotic meatus, and a prior history of 3 canal wall-down mastoidectomies and antibiotic therapy. A revision tympanoplasty with mastoidectomy was performed, and during the postoperative period, the patient had persistent pain and otorrhea, which were managed with opioids and several courses of antibiotic therapy. After symptoms persisted, imaging and culture ultimately led to the diagnosis of fungal skull base osteomyelitis, which was eventually treated successfully. While these complications are rare, their likelihood is increased with treatment delay and in the immunocompromised patient. Close management of immunocompromised patients, including diabetic patients, is vital in identifying complications early to aid in timely diagnosis and treatment to lead to the best possible outcome.
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Diabetes Mellitus Tipo 2 , Osteomielite , Otite Média , Masculino , Humanos , Pessoa de Meia-Idade , Processo Mastoide/cirurgia , Diabetes Mellitus Tipo 2/complicações , Osteomielite/diagnóstico , Osteomielite/etiologia , Osteomielite/terapia , Otite Média/complicações , Otite Média/cirurgia , Antibacterianos/uso terapêutico , Base do CrânioRESUMO
BACKGROUND: Cranial and spinal cerebrospinal fluid (CSF) leaks are associated with opposite CSF fluid dynamics. The differing pathophysiology between spontaneous cranial and spinal CSF leaks are, therefore, mutually exclusive in theory. OBSERVATIONS: A 66-year-old female presented with tension pneumocephalus. The patient underwent computed tomography (CT) scanning, which demonstrated left-sided tension pneumocephalus, with an expanding volume of air directly above a bony defect of the tegmen tympani and mastoideum. The patient underwent a left middle fossa craniotomy for repair of the tegmen CSF leak. In the week after discharge, she developed a recurrence of positional headaches and underwent head CT. Further magnetic resonance imaging of the brain and thoracic spine showed bilateral subdural hematomas and multiple meningeal diverticula. LESSONS: Cranial CSF leaks are caused by intracranial hypertension and are not associated with subdural hematomas. Clinicians should maintain a high index of suspicion for intracranial hypotension due to spinal CSF leak whenever "otogenic" pneumocephalus is found. Close postoperative follow-up and clinical monitoring for symptoms of intracranial hypotension in any patients who undergo repair of a tegmen defect for otogenic pneumocephalus is recommended.
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OBJECTIVE: To investigate the utility of the so-called "second window" of indocyanine green (ICG) as a near-infrared fluorescent dye for intraoperative visualization. PATIENTS: Three patients who underwent surgical resection of vestibular schwannoma (two retrosigmoid and one middle fossa approach). INTERVENTIONS: Patients underwent intravenous infusion of ICG at a mean dose of 4.8âmg/kg at a mean of 15.3âhours before surgical incision. Once tumor dissection began, near-infrared fluorescence was used alongside conventional operative microscopy to visualize tumor tissue. MAIN OUTCOME MEASURES: Ability to distinguish tumor tissue from adjacent nerves. RESULTS: Intraoperative fluorescence allowed for enhanced visualization of the tumor-nerve plane in all patients. However, the effect varied among patients, and the effect faded with increasing surgical time. CONCLUSIONS: ICG, a well-tolerated cyanine dye, demonstrates late fluorescence hours after administration secondary to diffusion into tumor as well as normal tissues (the so-called "second window" of fluorescence). Its fluorescence in the near-infrared spectrum is a promising adjunct for enhancing visualization of tumor planes during vestibular schwannoma surgery.
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Verde de Indocianina , Neuroma Acústico , Corantes Fluorescentes , Humanos , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/cirurgia , Imagem ÓpticaRESUMO
MYH9 is a gene that encodes for a subunit of the myosin heavy chain IIA protein. Mutations in MYH9 are associated with hematologic abnormalities, renal dysfunction, and hearing loss. Bony cochlear nerve canal stenosis (CNCS), which is diagnosed on computed tomography (CT) imaging, has been associated with congenital deafness, cochlear nerve aplasia/hypoplasia, and inner ear malformations. We report two cases of CNCS presenting with profound congenital hearing loss whom we diagnosed with mutations in MYH9 and discuss the genotype-phenotype association and implications for management.
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OBJECTIVE: To characterize the clinical features of patients with congenital hearing loss and unilateral cochlear nerve canal stenosis (CNCS). METHODS: A retrospective review of 12 patients with unilateral CNCS diagnosed between January 2018 and December 2019 at a tertiary referral hospital was performed. RESULTS: Of the 12 patients identified, there were 6 males and 6 females. All patients presented with hearing loss, with no other chief complaints. Two patients had accessory auricles. Eleven patients had a severe to profound sensorineural hearing loss on the affected side, while 1 patient had an isolated high-frequency hearing loss. Nine patients demonstrated atresia of the cochlear nerve canal (CNC), while three patients had a stenotic, but patent, CNC. CONCLUSION: Prompt radiologic diagnosis of patients with unilateral CNCS is important for patient counseling and appropriate rehabilitation.
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Adeno-associated virus (AAV) are potent vectors to achieve treatment against hearing loss resulting from genetic defects. However, the effects of delivery routes and the corresponding transduction efficiencies for clinical applications remain elusive. In this study, we screened AAV vectors of three serotypes (AAV 8 and 9 and Anc80L65) into the inner ears of adult normal guinea pigs through trans-stapes (oval window) and trans-round window delivery routes in vivo. Trans-stapes route is akin to stape surgeries in humans. Then, auditory brainstem response (ABR) measurements were conducted to evaluate postoperative hearing, and inner ear tissues were harvested for transduction efficiency analysis. Results showed that AAV8 targeted partial inner hair cells (IHCs) in cochlear basal turn; AAV9 targeted IHCs in cochlear basal and second turn, also a part of vestibular hair cells (VHCs). In contrast, Anc80L65 contributed to green fluorescent proteins (GFP) signals of 80 - 95% IHCs and 67 - 91% outer hair cells (OHCs), as well as 69% VHCs through the trans-round window route, with 15-20 decibel (dB) ABR threshold shifts. And, through the trans-stapes (oval window) route, there were 71 - 90% IHCs and 42 - 81% OHCs, along with 64% VHCs demonstrating GFP positive, and the ABR threshold shifts were within 10 dB. This study revealed AAV could be efficiently delivered into mammalian inner ear cells in vivo through the trans-stapes (oval window) route with postoperative hearing preservation, and both delivery routes showed promise of virus-based clinical translation of hearing impairment treatment.
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Orelha Interna , Perda Auditiva , Adulto , Animais , Cóclea , Dependovirus/genética , Proteínas de Fluorescência Verde/genética , Cobaias , Células Ciliadas Auditivas Internas , Perda Auditiva/genética , Perda Auditiva/terapia , Humanos , Mamíferos , Estribo , SuínosRESUMO
OBJECTIVES/HYPOTHESIS: To identify medical risk factors associated with auditory neuropathy spectrum disorder (ANSD). STUDY DESIGN: Retrospective case-control study. METHODS: During a 2-year period (2013-2014) patients with newly diagnosed ANSD were identified at a tertiary care facility. Twenty-two patients (n = 22) were identified aged 0.5 to 8.1 years. There were 15 males and seven females. Sixteen had bilateral, four had left-sided, and two had right-sided ANSD. Two age-matched, side-matched, and gender-matched control groups were then collected. The first group was 22 normal-hearing children (n = 22). The second was 22 children with sensorineural hearing loss (SNHL) (n = 22) who did not meet the criteria for ANSD. The chart of each subject was reviewed for the following five-predictor variables: prematurity, low birth weight, jaundice, use of mechanical ventilation, and administration of ototoxic medications. Analysis of variance was performed to analyze the prevalence of perinatal risk factors among the three groups. Multivariate linear regression was then applied. RESULTS: When comparing the ANSD group to both the normal-hearing and SNHL groups, the subjects with ANSD had statistically significant higher rates of prematurity, low birth weight, jaundice, and mechanical ventilation. Multiple regression analysis was performed to identify predictors of ANSD compared to each control group individually. Jaundice in the first month of life approached significance when comparing the ANSD group to the normal-hearing group, and was the only medical risk factor found to be statistically significant when comparing the ANSD group to the SNHL group. CONCLUSIONS: A history of neonatal hyperbilirubinemia was significantly more common in children with ANSD compared to children with severe SNHL. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:671-674, 2021.
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Perda Auditiva Central/etiologia , Hiperbilirrubinemia/complicações , Recém-Nascido de Baixo Peso , Recém-Nascido Prematuro , Ototoxicidade/complicações , Respiração Artificial/efeitos adversos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Perda Auditiva Neurossensorial/complicações , Humanos , Lactente , Recém-Nascido , Icterícia Neonatal/complicações , Modelos Lineares , Masculino , Estudos Retrospectivos , Medição de Risco , Fatores de RiscoRESUMO
Objective Data regarding the surgical advantages and anatomic constraints of a hearing-preserving endoscopic-assisted retrolabyrinthine approach to the IAC are scarce. This study aimed to define the minimum amount of retrosigmoid dural exposure necessary for endoscopic exposure of the IAC and the surgical freedom of motion afforded by this approach. Methods Presigmoid retrolabyrinthine approaches were performed on fresh cadaveric heads. The IAC was exposed under endoscopic guidance. The retrosigmoid posterior fossa dura was decompressed until the fundus of the IAC was exposed. Surgical freedom of motion at the fundus was calculated after both retrolabyrinthine and translabyrinthine approaches. Results The IAC was entirely exposed in nine specimens with a median length of 12 mm (range: 10-13 mm). Complete IAC exposure could be achieved with 1 cm of retrosigmoid dural exposure in eight of nine mastoids. For the retrolabyrinthine approach, the median anterior-posterior surgical freedom was 13 degrees (range: 6-23 degrees) compared with 46 degrees (range: 36-53 degrees) for the translabyrinthine approach ( p = 0.014). For the retrolabyrinthine approach, the median superior-inferior surgical freedom was 40 degrees (range 33-46 degrees) compared with 47 degrees (range: 42-51 degrees) for the translabyrinthine approach ( p = 0.022). Conclusion Using endoscopic assistance, the retrolabyrinthine approach can expose the entire IAC. We recommend at least 1.5 cm of retrosigmoid posterior fossa dura exposure for this approach. Although this strategy provides significantly less instrument freedom of motion in both the horizontal and vertical axes than the translabyrinthine approach, it may be appropriate for carefully selected patients with intact hearing and small-to-medium sized tumors involving the IAC.
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To explore the correlation between the width of the bony cochlear nerve canal (CNC) and long-term auditory rehabilitation after unilateral cochlear implantation (CI) in pediatric patients with congenital deafness and bilateral cochlear nerve canal stenosis (CNCS). A retrospective review was performed on 10 patients with bilateral CNCS and bilateral congenital profound hearing loss who each underwent unilateral cochlear implantation. The width of the CNC was determined on computed tomography (CT) imaging and following CI, auditory and speech performance following CI were graded using categories of auditory performance (CAP), speech intelligibility rating (SIR), and the meaningful auditory integration scale (MAIS) at 24 months following implantation. No correlation was noted between CAP score and CNCS at 24 months post CI (P > .05). A positive correlation was noted between SIR score and CNC width (ρ = .81, P < .05). Similarly, a positive correlation was noted between MAIS and CNC width (ρ = .71, P < .05). The width of the CNC in patients with CNCS is positively correlated with some long-term auditory and speech outcomes after CI.