Quantitative assessment of cochlear and vestibular ganglion neurons in temporal bones with chronic otitis media.

PURPOSE: In this study, we aimed to determine whether or not COM leads to loss of spiral and Scarpa ganglion neurons. METHODS: From the human temporal bone (HTB) collection at the University of Minnesota we selected human temporal bones with COM, defined as the presence of clinically intractable tissue abnormalities in the middle ear (cholesteatoma, perforation of the eardrum, granulation tissue, fibrosis, tympanosclerosis, and cholesterol granuloma). We also selected HTBs from donors with no ear diseases as controls. We quantitatively analyzed the number of spiral and Scarpa ganglion cells and compared the results obtained in the control and study groups. RESULTS: In both COM and control groups we observed a significant negative correlation between age and number of both spiral (R = -0.632; P < 0.001; 95% CI - 0.766 to - 0.434) and Scarpa ganglion (R = - 0.404; P = 0.008; 95% CI - 0.636 to - 0.051) cells. We did not find any significant differences in the number of spiral ganglion cells (in total or per segment) or in the density of Scarpa ganglion cells (in each vestibular nerve or both) in the COM group as compared with controls (P > 0.05). CONCLUSIONS AND RELEVANCE: Our results did not demonstrate significant loss of cochlear or vestibular peripheral ganglion neuron loss in HTBs with COM as compared with controls.

Histopathologic ear findings of syphilis: a temporal bone study.

To the best of our knowledge, histopathologic studies of syphilitic ears have generally focused on hydropic changes; so far, no such studies have investigated peripheral vestibular otopathology using differential interference contrast microscopy, in patients with syphilis. For this study, we examined 13 human temporal bone samples from 8 patients with a history of syphilis. Using conventional light microscopy, we performed qualitative histopathologic assessment. In addition, using differential interference contrast microscopy, we performed type I and type II vestibular hair cell counts on each vestibular sense organ with minimal autolysis; in which the neuroepithelium was oriented perpendicular to the plane of section. We then compared vestibular hair cell densities (cells per 0.01 mm² surface area) in the syphilis group vs. the control group. In the syphilis group, we observed precipitate in the endolymphatic or perilymphatic spaces in 1 (7.7 %) of the samples and endolymphatic hydrops in eight (61.5 %) of the samples. Hydrops involved the cochlea (four samples) and/or saccule (four samples). In addition, the syphilis group experienced a significant loss of type II vestibular hair cells in the maculae of the utricle and saccule, and in the cristae of the lateral and posterior semicircular canals, as compared with the control group (P < 0.05).

Relationship Between Cochlear Lateral Wall Changes and Endolymphatic Hydrops in Otitis Media.

OBJECTIVES: Despite otitis media and various disease processes being associated with endolymphatic hydrops (EH), an exact explanation of the pathophysiology has yet to be reported. This study aimed to investigate the changes in the cochlear lateral wall structures and their potential correlation with the presence and severity of cochlear EH in acute and chronic otitis media cases. The investigations were conducted in both chinchilla animal model and human temporal bone specimens. METHODS: We studied a total of 15 chinchilla and 25 human temporal bones from our collection, which were categorized into acute otitis media, chronic otitis media (COM), and control groups. Through quantitative analysis, we measured the area of cochlear lateral wall structures and observed the presence and the degree of EH using light microscopy. RESULTS: No significant changes were determined in the area of the spiral ligament (p > 0.05) across the species. However, a significant (p < 0.05) decrease in the mean area of the stria vascularis in the basal turn was identified in COM groups compared to controls of both species. Chinchilla model additionally exhibited pathology extending to the lower mid turn. A negative correlation was found between the mean strial area and the severity of EH in both the animal model and human samples. CONCLUSIONS: COM associated with significant changes in the stria vascularis that may lead to significant increase in the degree of EH. The presented animal model exhibited parallel findings with human samples, suggesting its viability as a valuable model for future studies. LEVEL OF EVIDENCE: N/A Laryngoscope, 2024.

Task force of the Brazilian Society of Otology - evaluation and management of peripheral facial palsy.

OBJECTIVE: To review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. CONCLUSIONS: Peripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Bilateral Sudden Sensorineural Hearing Loss: A Distinct Phenotype Entity.

OBJECTIVE: To investigate the clinical phenotype and hearing prognosis of patients with unilateral and bilateral (simultaneous and nonsimultaneous) sudden sensorineural hearing loss (SSNHL). STUDY DESIGN: Retrospective cohort. SETTING: Otology outpatient clinic of a tertiary university hospital. PATIENTS: Three hundred eighty-five medical records from patients with SSNHL. MAIN OUTCOME MEASURES: Comparison of demographic, clinical, and audiometric (pretreatment and posttreatment) data of patients with unilateral and bilateral SSNHL. RESULTS: Our final analysis comprised of 239 medical records of patients with SSNHL. Most patients had unilateral SSNHL (186, 77.82%). We identified a potential underlying cause for the SSNHL in 105 (43.9%) of the cases, being more frequent in cases of bilateral simultaneous SSNHL (100%) as compared with unilateral (45.6%) and nonsimultaneous bilateral SSNHL (22.7%). Cases of simultaneous bilateral SSNHL presented more frequently with severe or profound hearing loss (89%; unilateral SSNHL 50.5%; nonsimultaneous bilateral SSNL, 59.1%) and had a significantly worse hearing recovery prognosis as compared with unilateral or nonsimultaneous bilateral SSNHL (p = 0.002). CONCLUSION: We observed a high heterogeneity of clinical presentations of SSNHL, the most common being unilateral SSNHL. Our results demonstrate that the clinical and audiological prognosis of simultaneous bilateral SSNHL differed significantly from unilateral and nonsimultaneous bilateral SSNHL, suggesting that simultaneous cases of bilateral SSNHL may be a part of a different disease process. The presence of a simultaneous, bilateral SSNHL indicates the need to investigate the presence of an underlying systemic disease.

Evaluation of the vestibulo-ocular reflex in patients with chronic otitis media.

INTRODUCTION: In recent years, scientific evidence has shown that chronic otitis media may cause balance and vestibular dysfunction. OBJECTIVE: To compare the results of the video head impulse test (gain and symmetry of the vestibulo-ocular reflex and presence of covert and overt saccades) in patients with chronic otitis media and controls. METHODS: Cross-sectional study of patients with chronic otitis media (study group), aged between 18 and 60 years. The patients in the study group were further divided according to the chronic otitis media type as (1) non-suppurative, (2) suppurative, and (3) cholesteatomatous. For the comparative analysis, we selected volunteers with no history of ear and vestibular diseases (control group), who met the same inclusion and exclusion criteria as the study group. Patients in both groups underwent a video head impulse test. RESULTS: The study group consisted of 96 volunteers, and the control group of 61 individuals. The prevalence of vestibular symptoms was 66% in the study group and 3.2% in the control group (p <  0.001). The results show a higher prevalence of changes in the vestibulo-ocular reflex gain (22.9%) and corrective saccades (12.6%) in the chronic otitis media group compared to the control group (p <  0.001). Despite the higher prevalence of changes in gain, the average vestibulo-ocular reflex gains in the chronic otitis media groups were within the pre-defined values ​​of normality; however, the mean vestibulo-ocular reflex gain in the anterior semicircular canal was statistically worse in the cholesteatomatous chronic otitis media group compared to controls (p <  0.001). Regarding the corrective saccades, the prevalence of saccades was statistically higher in the suppurative and cholesteatomatous chronic otitis media subgroups compared to the non-suppurative and control groups (p =  0.004). CONCLUSION: The present study demonstrated that chronic otitis media is associated with a higher prevalence of vestibular symptoms and also a higher prevalence of changes in gain and corrective saccades when compared to controls.

Temporal Bone Pathology Secondary to Head Trauma-A Human Temporal Bone Study.

HYPOTHESIS/BACKGROUND: We hypothesize that following head trauma there is a difference in temporal bone (TB) pathology in cases with and without skull fracture. Although conductive, sensorineural, mixed hearing loss, and TB pathology following head trauma have been reported, to our knowledge, there are no studies that have compared the pathology of the TB in cases with and without skull fracture. METHODS: We analyzed 34 TBs from donors who had a history of head trauma (20 with skull fracture and 14 without fracture), and 25 age-matched controls without clinical or histological evidence of otologic disorders. We documented the presence and location of TB fracture, ossicular injury, and cochlear hemorrhage and evaluated the loss of spiral ganglion cells and sensory hair cells, damage to the stria vascularis, and the presence of endolymphatic hydrops. RESULTS: We found a significant loss of outer hair cells in the upper basal, lower, and upper middle turns of the cochlea (p = 0.009, =0.019, =0.040, respectively), a significant loss of spiral ganglion cells (p = 0.023), and cochlear hemorrhage predominantly in the basal turns secondary to head trauma. Interestingly, these findings were significantly observed in TBs from donors with a history of head trauma without skull fracture. CONCLUSION: The greatest damage was to the cochlear basal turn. Our findings suggest that head trauma may result in tonotopic high frequency sensorineural hearing loss. TBs from donors with skull fracture have less pathologic changes than those without.

Evaluation of Oxidative-Stress Pathway and Recovery of Sudden Sensorineural Hearing Loss.

Introduction Although the pathogenesis of sudden sensorineural hearing loss (SSNHL) has been discussed in the literature, many unclear points remain. Several authors have hypothesized that oxidative stress plays a role in the pathogenesis of noise-related hearing loss, as well as in drug- and aging-related hearing loss. Reactive oxygen species (ROS) may contribute to the pathogenesis of SSNHL in a similar way as in cases of ototoxicity, noise-induced hearing loss and presbyacusis. Objective The aim of the present study was to find potential peripheral biomarkers to show the levels of oxidative stress in samples of peripheral blood collected from SSNHL patients with and withouth metabolic disease. Methods In total, 80 consecutive patients with SSNHL were evaluated in the otolaryngology emergency room and outpatient clinic of a tertiary hospital between May 2017 and May 2019. All patients underwent detailed anamnesis, physical examination, audiometry, magnetic resonance imaging (MRI) of the inner ears, and blood tests for serum lipids and plasma activity of thiobarbituric acid reactive species (TBARS). Results No significant effect of malondialdehyde (MDA) activity was observed regarding the hearing recovery of patients who developed SSNHL. Conclusion We did not observe a significant correlation between the concentration of TBARs in the peripheral blood or the presence of arterial hypertension and the severity of the initial hearing loss or the prognosis of hearing recovery in patients with SSNHL. The concentration of TBARs in the peripheral blood may not adequately represent the abnormalities that occur in the intracoclear environment.

Evaluation of vestibular symptoms and postural balance control in patients with chronic otitis media.

BACKGROUND: Evidence to support potential links between chronic otitis media (COM) and vestibular impairment/postural balance control issues is lacking. OBJECTIVE: To investigate whether COM associates with vestibular symptoms, balance problems, and abnormalities in vestibular function tests. METHODS: We selected 126 patients with COM and excluded patients with any identifiable underlying causes for vestibular dysfunction. Fifty-two healthy volunteers were included as controls. All subjects underwent anamnesis, physical examination, posturography, and video-head impulse tests. RESULTS: We found a high prevalence of vestibular symptoms (58.4%) among patients with COM, while only 2% of the controls had vestibular symptoms. There was a positive correlation between COM activity with the presence of tinnitus and vestibular symptoms (P < 0.05). Clinical vestibular tests were abnormal in 63% of patients with COM, and those positively associated with presence of vestibular symptoms. Posturography results shown worse postural balance control in patients with COM as compared with controls, especially in the limit of stability (LOS) (Mean LOS, COM = 157.56 cm2; controls = 228.98 cm2; p < 0.001) and worse results in the test with eyes closed while standing on a foam mattress (sway area, COM = 10.91 cm2; controls = 5.90 cm2; p < 0.001) in patients with COM as compared with controls. We did not observe differences in the average vestibuloocular reflex gains in the video-head impulse test between our COM and control groups. CONCLUSIONS: Our results show that COM associates with higher prevalence of vestibular symptoms and abnormalities in clinical vestibular function tests, and worse postural control as compared with controls. Among patients with COM, the activity of the middle-ear inflammation seemed to positively associate with the severity of hearing and balance problems.

Sensorineural hearing loss in the acute phase of a single episode of acute otitis media.

INTRODUCTION: Acute otitis media is a disease with high global prevalence, that can lead to several acute complications and auditory sequelae. Data regarding the auditory evaluation in the acute phase of acute otitis media are scarce. OBJECTIVE: To evaluate the main audiometric changes (air and bone conduction thresholds) in the initial phase of an acute otitis media episode. METHODS: A case-control study was performed. Patients diagnosed with acute otitis media with less than 7 days of evolution in relation to the complaint onset were selected, and healthy volunteers were selected as controls. The acute otitis media and control groups were submitted to pure tone and vocal audiometry. RESULTS: The acute otitis media group included a total of 27 patients (30 ears). Hearing loss was present in 90.0% of the ears with acute otitis media, with conductive loss in 14 (46.67%) and mixed loss in 13 (43.33%). Both the air and bone conduction thresholds obtained with the tonal audiometry in the acute otitis media group were significantly worse than the controls at all tested frequencies (p < 0.05). In patients with acute otitis media, we observed that the thresholds for frequency >1 kHz (bone conduction) and 3 kHz (air conduction) were significantly worse in patients with tinnitus compared to patients without tinnitus. CONCLUSION: During the first 7 days of evolution after the onset of an isolated episode of acute otitis media, we observed significant increases in bone and air thresholds at all frequencies, especially >2 kHz, compared to healthy ears.

Histopathology of Inner Ear Malformations: Potential Pitfalls for Cochlear Implantation.

HYPOTHESIS: The presence of bony inner ear malformations may associate with a number of anatomical abnormalities affecting the middle ear structures. Those malformations may create pitfalls and complications for cochlear implantation. BACKGROUND: Inner ear malformations associate with varying degrees of hearing loss, and frequently require cochlear implantation for hearing rehabilitation. Therefore, the abnormalities affecting the middle- and inner-ear structures may increase the risk of surgical complications. METHODS: We examined 38 human temporal bones from donors with bony inner ear malformations. Using light microscopy, we analyzed the presence of abnormalities in the structures of the middle- and inner-ear. RESULTS: Our collection comprises of 38 specimens with inner-ear malformations (cochlear aplasia, n = 3; cochlear hypoplasia, n = 30; incomplete partition, n = 3; isolated vestibular malformation, n = 2). The anatomy of the middle ear was abnormal in most temporal bones with cochlear aplasia, cochlear hypoplasia, and incomplete partition type I (40%-100%). Some of those abnormalities (hypoplastic or obliterated mastoid, 55.2%; aplastic or obliterated round window, 71.0%; aberrant course of the facial nerve, 36.8%) may hinder the access to the round window using the conventional facial recess approach for cochlear implantation. The cochlear nerve and associated bony structures (internal auditory canal and bony canal for cochlear nerve) were normal in 71.0% of all temporal bones with inner ear malformations. CONCLUSION: Each different type of malformation may create specific surgical challenges to surgeons. Comprehensive preoperative imaging is fundamental toward the surgical success of cochlear implants in patients with malformations. Alternatives to circumvent those middle- and inner-ear abnormalities and potential complications are further discussed.

Otopathology in the United States: History, Current Situation, and Future Perspectives.

: Human temporal bone studies have documented the pathophysiologic basis of many pathologic conditions and diseases affecting the ear, contributing to the development of specific clinical knowledge and pathology-oriented treatments. Researchers dedicated to the study of anatomy and histology of the temporal bone emanated from Europe to the United States during the first part of the 20th Century. The first otopathology laboratory was founded in the United States in 1924, at Johns Hopkins University; over time, the otopathology laboratories-considered by some authors as "gold mines" for studying ear diseases-became numerous and very prolific. However, today, only three of the temporal bone laboratories are still running and producing scientific knowledge to the Otology/Neurotology field: the ones at Harvard Medical School, University of Minnesota, and University of California. Molecular biologic assay techniques and new microscopy and computer equipment broadened the possibilities for temporal bone studies; however, the current funding for those laboratories are insufficient to cover the costs for processing and studying human temporal bones. The main objective of this study is to briefly describe the history, current situation, and future perspectives of the otopathology laboratories in the United States.

Current evidence of peripheral vestibular symptoms secondary to otitis media.

BACKGROUND: The association between otitis media and vestibular symptoms has been hypothesized in the past. Thus, in this study, we aimed to critically analyze (based in a systematic review of the literature) whether patients who have otitis media are at greater risk of developing vestibular impairment or not. METHODS: We performed a systematic review of the literature and identified potentially relevant articles reporting vestibular symptoms and results of vestibular function tests in patients with otitis media through searches of the PubMED, Web of Science, Scopus, and Google Scholar databases. The quality of the final set of records was assessed using the "Newcaste-Ottawa Scale". RESULTS: Of the 2334 records searched, 43 met our inclusion and exclusion criteria, and those included 2250 patients. The records comprised 20 longitudinal studies, 21 cross-sectional studies, and 2 case reports. Regarding the type of otitis media studied, 25 examined vestibular impairment in otitis media with effusion, 6 acute otitis media, and 12 chronic otitis media. Results of anamnesis, clinical exams, and several vestibular function tests are reported and critically discussed. CONCLUSION: Most studies evaluating the association between otitis media and vestibular symptoms have potential methodological flaws. Clinical evidence suggests that patients with otitis media have increased chances for having vestibular symptoms, delayed acquisition of developmental milestones, and abnormalities in several vestibular function tests as compared with controls. Future studies with rigorous methodology aiming to assess the clinical significance (and prognostic factors) of the association between otitis media and vestibular impairment are warranted. Key message Several studies demonstrated long-term sequelae secondary to otitis media. However, the evidence supporting those assumptions are based in low-quality evidence. Thus, better structured studies are warranted to better understand the clinical relevance of such association.

Progression of changes in the sensorial elements of the cochlear and peripheral vestibular systems: The otitis media continuum.

Our study aimed to evaluate pathologic changes in the cochlear (inner and outer hair cells and stria vascularis) and vestibular (vestibular hair cells, dark, and transitional cells) sensorial elements in temporal bones from donors who had otitis media. We studied 40 temporal bones from such donors, which were categorized in serous otitis media (SOM), serous-purulent otitis media (SPOM), mucoid/mucoid-purulent otitis media (MOM/MPOM), and chronic otitis media (COM); control group comprised 10 nondiseased temporal bones. We found significant loss of inner and outer cochlear hair cells in the basal turn of the SPOM, MOM/MPOM and COM groups; significant loss of vestibular hair cells was observed in the MOM/MPOM and COM groups. All otitis media groups had smaller mean area of the stria vascularis in the basal turn of the cochlea when compared to controls. In conclusion, our study demonstrated more severe pathologic changes in the later stages of the continuum of otitis media (MOM/MPOM and COM). Those changes seem to progress from the basal turn of the cochlea (stria vascularis, then inner and outer hair cells) to the middle turn of the cochlea and to the saccule and utricle in the MOM/MPOM and COM stages.

Evaluation of the vestibulo-ocular reflex in patients with chronic otitis media / Avaliação do reflexo vestíbulo-ocular em pacientes com otite média crônica

Abstract Introduction In recent years, scientific evidence has shown that chronic otitis media may cause balance and vestibular dysfunction. Objective To compare the results of the video head impulse test (gain and symmetry of the vestibulo-ocular reflex and presence of covert and overt saccades) in patients with chronic otitis media and controls. Methods Cross-sectional study of patients with chronic otitis media (study group), aged between 18 and 60 years. The patients in the study group were further divided according to the chronic otitis media type as (1) non-suppurative, (2) suppurative, and (3) cholesteatomatous. For the comparative analysis, we selected volunteers with no history of ear and vestibular diseases (control group), who met the same inclusion and exclusion criteria as the study group. Patients in both groups underwent a video head impulse test. Results The study group consisted of 96 volunteers, and the control group of 61 individuals. The prevalence of vestibular symptoms was 66% in the study group and 3.2% in the control group (p< 0.001). The results show a higher prevalence of changes in the vestibulo-ocular reflex gain (22.9%) and corrective saccades (12.6%) in the chronic otitis media group compared to the control group (p< 0.001). Despite the higher prevalence of changes in gain, the average vestibulo-ocular reflex gains in the chronic otitis media groups were within the pre-defined values ​​of normality; however, the mean vestibulo-ocular reflex gain in the anterior semicircular canal was statistically worse in the cholesteatomatous chronic otitis media group compared to controls (p< 0.001). Regarding the corrective saccades, the prevalence of saccades was statistically higher in the suppurative and cholesteatomatous chronic otitis media subgroups compared to the non-suppurative and control groups (p= 0.004). Conclusion The present study demonstrated that chronic otitis media is associated with a higher prevalence of vestibular symptoms and also a higher prevalence of changes in gain and corrective saccades when compared to controls.

Resumo Introdução Nos últimos anos, evidências científicas demonstraram que a otite média crônica se associa a alterações de equilíbrio e disfunção vestibular. Objetivo Comparar os resultados do teste do impulso cefálico por vídeo (ganho e simetria do reflexo vestíbulo-ocular e presença de sacadas cobertas e descobertas) em pacientes com otite média crônica e controles. Método Estudo transversal que envolveu pacientes com otite média crônica (grupo de estudo), entre 18 a 60 anos. Os pacientes no grupo estudo ainda foram divididos de acordo com o tipo de otite média crônica em (1) não supurativa, (2) supurativa e (3) colesteatomatosa. Para análise comparativa, selecionamos voluntários sem história de doenças otológicas e vestibulares (grupo controle), que obedeceram aos mesmos critérios de inclusão e exclusão do grupo de estudo. Os pacientes de ambos grupos foram submetidos ao teste de impulso cefálico por vídeo. Resultados O grupo estudo foi composto por 96 voluntários e o grupo controle por 61 indivíduos. A prevalência de sintomas vestibulares foi de 66% no grupo de estudo e 3,2% no grupo controle (p < 0,001). Os resultados mostram maior prevalência de alterações do ganho do reflexo vestíbulo-ocular (22,9%) e de sacadas corretivas (12,6%) no grupo otite média crônica em comparação ao grupo controle (p < 0,001). Apesar da maior prevalência de alterações de ganho, a média dos ganhos do reflexo vestíbulo-ocular dos grupos de otite média crônica estava dentro dos valores pré-definidos de normalidade; porém, a média do ganho do reflexo vestíbulo-ocular no canal semicircular anterior foi estatisticamente pior no grupo otite média crônica colesteatomatosa em comparação aos controles (p< 0,001). Em relação às sacadas corretivas, a prevalência de sacadas foi estatisticamente maior nos subgrupos otite média crônica supurativa e colesteatomatosa em comparação aos grupos não supurativa e controle (p = 0,004). Conclusão A otite média crônica se associa à maior prevalência de sintomas vestibulares e também maior prevalência de alterações no ganho e de sacadas corretivas em comparação a controles.

Otologic manifestations of Larsen syndrome.

OBJECTIVE: To describe and discuss otologic manifestations of Larsen syndrome, based on a case report and a systematic review of the literature. MATERIALS AND METHODS: We performed a PubMED database search, and we selected studies reporting otolaryngologic manifestations secondary to Larsen syndrome. The selected articles were read in full, and three researchers independently extracted data from the studies. In parallel, we report the case of a 14-year-old patient who had hearing loss secondary to Larsen syndrome. RESULTS: Fifteen studies met our selection criteria. Seven studies reported hearing loss in patients with Larsen syndrome (4 had conductive hearing loss and 3 had mixed hearing loss). The conductive hearing loss may be secondary to ossicular malformations and/or middle ear effusions. Other causes for conductive hearing loss are mesenchymal remnants in the middle ear, Eustachian tube dysfunction, and cleft palate. Surgical management of the hearing loss is possible in selected cases, although the surgical and anesthetic risks should be considered. Hearing aids seem to be safe and effective treatment options for the hearing loss secondary to Larsen syndrome. CONCLUSION: Although rare, patients with Larsen syndrome may have hearing loss. The most frequent type of deficit is conductive, either secondary to malformation of the ossicles or middle ear effusion. Possible surgical correction of these abnormalities should be weighed against the anesthetic risks of these patients.

Pathophysiology and Diagnosis of Vertebrobasilar Insufficiency: A Review of the Literature.

Introduction Vertebrobasilar insufficiency is defined as transitory ischemia of the vertebrobasilar circulation. Dizziness, vertigo, headaches, vomit, diplopia, blindness, ataxia, imbalance, and weakness in both sides of the body are the most common symptoms. Objective To review the literature regarding the three available diagnostic testing in patients with dizziness complaints secondary to vertebrobasilar insufficiency (VBI): magnetic resonance angiography; transcranial Doppler ultrasound; and vertebrobasilar deprivation testing. Data Synthesis We selected 28 studies that complied with our selection criteria for appraisal. The most frequent cause of the hemodynamic changes leading to VBI is atherosclerosis. The main clinical symptoms are dizziness, vertigo, headaches, vomit, diplopia, blindness, ataxia, imbalance, and weakness in both sides of the body. Even though arteriography is considered the most important exam to diagnose the disease, the inherent risks of this exam should be taken into consideration. The magnetic resonance angiography has been widely studied and is a good method to identify and localize any occlusions and stenosis in both neck and intracranial great vessels. Conclusion Each patient with a suspected diagnosis of VBI should be individually evaluated and treated, taking in consideration the pros and cons of each diagnostic testing and treatment option.

A three-dimensional analysis of the endolymph drainage system in Ménière disease.

OBJECTIVES/HYPOTHESIS: To measure the volume of the endolymph drainage system in temporal bone specimens with Ménière disease, as compared with specimens with endolymphatic hydrops without vestibular symptoms and with nondiseased specimens STUDY DESIGN: Comparative human temporal bone analysis. METHODS: We generated three-dimensional models of the vestibular aqueduct, endolymphatic sinus and duct, and intratemporal portion of the endolymphatic sac and calculated the volume of those structures. We also measured the internal and external aperture of the vestibular aqueduct, as well as the opening (if present) of the utriculoendolymphatic (Bast's) valve and compared the measurements in our three study groups. RESULTS: The volume of the vestibular aqueduct and of the endolymphatic sinus, duct, and intratemporal endolymphatic sac was significantly lower in the Ménière disease group than in the endolymphatic hydrops group (P <.05). The external aperture of the vestibular aqueduct was also smaller in the Ménière disease group. Bast's valve was open only in some specimens in the Ménière disease group. CONCLUSIONS: In temporal bones with Ménière disease, the volume of the vestibular aqueduct, endolymphatic duct, and intratemporal endolymphatic sac was lower, and the external aperture of the vestibular aqueduct was smaller as compared with bones from donors who had endolymphatic hydrops without vestibular symptoms and with nondiseased bones. The open status of the Bast's valve in the Ménière disease group could be secondary to higher retrograde endolymph pressures caused by smaller drainage systems. These anatomic findings could correlate with the reason that some patients with hydrops develop clinical symptoms, whereas others do not. LEVEL OF EVIDENCE: N/A Laryngoscope, 127:E170-E175, 2017.

Epitympanum volume and tympanic isthmus area in temporal bones with retraction pockets.

OBJECTIVES/HYPOTHESIS: To compare the volume of the epitympanic space, as well as the area of the tympanic isthmus, in human temporal bones with retraction pockets to those with chronic otitis media without retraction pockets and to those with neither condition. STUDY DESIGN: Comparative human temporal bone study. METHODS: We generated a three-dimensional model of the bony epitympanum and measured the epitympanic space. We also compared the area of the tympanic isthmus. RESULTS: The mean total volume of the epitympanum was 40.55 ± 7.14 mm3 in the retraction pocket group, 50.03 ± 8.49 mm3 in the chronic otitis media group, and 48.03 ± 9.16 mm3 in the neither condition group. The mean volume of the anterior, lateral, and medial compartments in temporal bones in the retraction pocket group was significantly smaller than in the two control groups (P < 0.05). Total epitympanic volume was also significantly smaller in the retraction pocket group than in both control groups (P < 0.05). The mean area of the tympanic isthmus was significantly smaller in the retraction pocket group (8.11 ± 2.44 mm2 ) than in the chronic otitis media group (9.82 ± 2.06 mm2 ) or the neither condition group (10.66 ± 1.78 mm2 ) (P < 0.05). CONCLUSION: Our data indicate that temporal bones with retraction pockets have a smaller volume bony epitympanum and a smaller tympanic isthmus area as compared with temporal bones from both control groups. The smaller volume tympanic isthmus in the retraction pocket group may suggest that a blockage in the aeration pathways to the epitympanum could create dysventilation, resulting in negative pressure and ultimately in retraction pockets and cholesteatomas. LEVEL OF EVIDENCE: NA Laryngoscope, 126:E369-E374, 2016.

Treatment and Prognosis of Facial Palsy on Ramsay Hunt Syndrome: Results Based on a Review of the Literature.

Introduction Ramsay Hunt syndrome is the second most common cause of facial palsy. Early and correct treatment should be performed to avoid complications, such as permanent facial nerve dysfunction. Objective The objective of this study is to review the prognosis of the facial palsy on Ramsay Hunt syndrome, considering the different treatments proposed in the literature. Data Synthesis We read the abstract of 78 studies; we selected 31 studies and read them in full. We selected 19 studies for appraisal. Among the 882 selected patients, 621 (70.4%) achieved a House-Brackmann score of I or II; 68% of the patients treated only with steroids achieved HB I or II, versus 70.5% when treated with steroids plus antiviral agents. Among patients with complete facial palsy (grades V or VI), 51.4% recovered to grades I or II. The rate of complete recovery varied considering the steroid associated with acyclovir: 81.3% for methylprednisolone, 69.2% for prednisone; 61.4% for prednisolone; and 76.3% for hydrocortisone. Conclusions Patients with Ramsay-hunt syndrome, when early diagnosed and treated, achieve high rates of complete recovery. The association of steroids and acyclovir is better than steroids used in monotherapy.