The endoscopic transcanal approach to the internal auditory canal: an anatomic study.

PURPOSE: The internal auditory canal (IAC) plays a key role in lateral skull base surgery. Although several approaches to the IAC have been proposed, endoscope-assisted transcanal corridors to the IAC have rarely been studied. We sought to provide a step-by-step description of the transcanal transpromontorial approach to the IAC and analyze anatomic relationships that might enhance predictability and safety of this approach. METHODS: Ten cadaveric specimens were dissected and the extended transcanal transpromontorial approach to the IAC was established. Various morphometric measurements and anatomic landmarks were reviewed and analyzed. RESULTS: The proposed technique proved feasible and safe in all specimens. There was no inadvertent injury to the jugular bulb or internal carotid artery. The chorda tympani, a key landmark for the mastoid segment of the facial nerve, was identified in all dissections. The spherical recess of the vestibule and middle turn of cochlea are important landmarks for identification of the labyrinthine segment of the facial nerve. Identification of all boundaries of the working area is also essential for safe access. Among various morphometric measurements, the modiolus-IAC angle (≈ 150°) proved particularly consistent; given its ease of use and low variability, we believe it could serve as a landmark for identification and subsequent dissection of the IAC. CONCLUSIONS: The extended transcanal transpromontorial approach to the IAC is feasible and safe. Relying on anatomic landmarks to ensure preservation of the involved neurovascular structures is essential for a successful approach. The modiolus-IAC angle is a consistent, reproducible landmark for IAC identification and dissection.

The variations of osseous structure of the internal acoustic canal: an anatomical study.

OBJECTIVES: The internal acoustic meatus is an osseous canal that connects the inner ear to the posterior cranial fossa. It is located in the petrous portion of the temporal bone. A thin cribriform osseous plate known as the fundus is situated at the lateral end of the canal. This study assesses the structural and numerical variations of the fundus formations. METHODS: Fifty-four temporal bones of unknown gender and age were examined with the surgical microscope. RESULTS: The temporal bones analyzed were 46.2% right-sided and 53.7% left-sided. Only one temporal bone had two parallel transverse crests, while three had a single anterior crest that split into two branches posteriorly. The number of foramina at the transverse crest varied, with 29.6% having none, 48.1% having a single foramen, and 22.2% having several foramina. An anterior crest structure was seen in 53.7% of the temporal bones, with 5% having a slightly constricted entry to the facial canal. In cases with a single nerve foramen, 48.1% had one, while 51.8% had more than one, including examples with three or four foramina. A crest was found between the foramina of the single nerve in 7% of patients. Furthermore, a crest between the saccular nerve foramen and the high fiber foramina was seen in 25.9% of cases, and 5% had two saccular nerve foramina. CONCLUSION: We think that revealing the anatomical, structural and numerical variations in the fundus will be useful in explaining the disease-symptom relationship. LEVEL OF EVIDENCE: Level 4.

Evaluation of inner ear damage by mastoid drilling with measurement of serum prestin (SLC26A5) levels.

OBJECTIVE: The objective of this study is to demonstrate any inner ear injury caused by drilling in mastoid surgery with prestin, outer hair cell motor protein specific to the cochlea. METHODS: The patients with chronic otitis media requiring mastoidectomy (n = 21) and myringoplasty (n = 21) were included. Serum prestin level obtained from blood samples was measured before surgery and on postoperative days 0, 3, and 7 using Human Prestin (SLC26A5) ELISA Kit. All patients underwent the Pure Tone Audiometry (PTA) test before surgery and on the postoperative 7th day. The drilling time was also recorded for all patients who underwent mastoidectomy. RESULTS: In both mastoidectomy and myringoplasty groups, the postoperative serum prestin levels increased on days 0 and 7 (pday-0 = 0.002, pday-7 = 0.001 and pday-0 = 0.005, pday-7 = 0.001, respectively). There was no significant difference in the serum prestin levels between the two groups, postoperatively. The PTA thresholds at day 7 did not change in either group. A significant decline at 2000 Hz of bone conduction hearing threshold in both groups and a decline at 4000 Hz in the myringoplasty group were found. There was no correlation between the drilling time and the increase of prestin levels in the postoperative day 0, 3, and 7. CONCLUSION: Our results showed that mastoid drilling is not related to a significant inner ear injury. Although the myringoplasty group was not exposed to drill trauma, there was a similar increase in serum prestin levels as the mastoidectomy group. Also, a significant decline at 2000 Hz of bone conduction hearing threshold in both groups and a decline at 4000 Hz in the myringoplasty group were found. These findings suggest that suction and ossicular manipulation trauma can lead to an increase in serum prestin levels and postoperative temporary or permanent SNHL at 2000 and 4000 Hz. LEVEL OF EVIDENCE: Level-4.

Intracanalicular vestibular schwannomas: facial and hearing outcomes according to the location of the tumor in relation to the nerves of the internal auditory canal.

OBJECTIVE: The objective was to evaluate whether the position of the nerves within the internal auditory canal (IAC) has any effect on preoperative and postoperative cochlear and facial nerve function in patients with intracanalicular vestibular schwannoma (VS) resected through the retrosigmoid transmeatal approach. METHODS: Forty-four patients with sporadic intracanalicular VS, from a series of 710 patients with VS who underwent operations from January 1993 to April 2022, were retrospectively reviewed. The pattern of displacement of the cranial nerves and tumor within the IAC was recorded. Tumors were divided into 2 types: type T1A lesions had only anteriorly displaced nerves, and type T1B had posteriorly displaced vestibular nerves and anteriorly displaced facial and cochlear nerves. Differences in surgical outcomes between groups in terms of facial nerve function and hearing preservation were evaluated. RESULTS: Thirty-five cases (79.5%) were T1A tumors and 9 were T1B (20.5%). Gross-total resection and anatomical preservation of the facial and cochlear nerves were achieved in all patients. Postoperatively, all patients with T1A VS maintained normal facial nerve function; however, among T1B VS patients, 6 (67%) retained House-Brackmann grade I, 2 worsened to grade II, and 1 worsened to grade III at 6 months (p = 0.006). The 27 T1A VS patients with serviceable hearing maintained this status, and an additional patient with nonserviceable hearing improved to serviceable hearing; among T1B VS patients, only 2 of the 5 patients with serviceable hearing remained as such, 2 evolved to nonserviceable hearing, and 1 lost hearing after surgery (p = 0.0022). T1B VS patients had a 24-fold risk of facial nerve deterioration (relative risk [RR] 25.2, 95% CI 1.42-448.57, p = 0.028) and a 32-fold risk of hearing deterioration (RR 32.7, 95% CI 1.93-553, p = 0.016) after surgery. CONCLUSIONS: In intracanalicular VS, postoperative cochlear and facial nerve function are directly related to the location of the tumor in relation to the nerves, with worse outcomes in cases where the tumor is located between the vestibular and facial-cochlear nerves.

cAMP-Epac1 signaling is activated in DDAVP-induced endolymphatic hydrops of guinea pigs.

OBJECTIVE: To explore whether Cyclic Adenosine Monophosphate (cAMP)-Epac1 signaling is activated in 1-Desamino-8-D-arginine-Vasopressin-induced Endolymphatic Hydrops (DDAVP-induced EH) and to provide new insight for further in-depth study of DDAVP-induced EH. METHODS: Eighteen healthy, red-eyed guinea pigs (36 ears) weighing 200-350 g were randomly divided into three groups: the control group, which received intraperitoneal injection of sterile saline (same volume as that in the other two groups) for 7 consecutive days; the DDAVP-7d group, which received intraperitoneal injection of 10 mg/mL/kg DDAVP for 7 consecutive days; and the DDAVP-14d group, which received intraperitoneal injection of 10 µg/mL/kg DDAVP for 14 consecutive days. After successful modeling, all animals were sacrificed, and cochlea tissues were collected to detect the mRNA and protein expression of the exchange protein directly activated by cAMP-1 and 2 (Epac1, Epac2), and Repressor Activator Protein-1 (Rap1) by Reverse Transcription (RT)-PCR and western blotting, respectively. RESULTS: Compared to the control group, the relative mRNA expression of Epac1, Epac2, Rap1A, and Rap1B in the cochlea tissue of the DDAVP-7d group was significantly higher (p <  0.05), while no significant difference in Rap1 GTPase activating protein (Rap1gap) mRNA expression was found between the two groups. The relative mRNA expression of Epac1, Rap1A, Rap1B, and Rap1gap in the cochlea tissue of the DDAVP-14d group was significantly higher than that of the control group (p <  0.05), while no significant difference in Epac2 mRNA expression was found between the DDAVP-14d and control groups. Comparison between the DDAVP-14d and DDAVP-7d groups showed that the DDAVP-14d group had significantly lower Epac2 and Rap1A (p <  0.05) and higher Rap1gap (p < 0.05) mRNA expression in the cochlea tissue than that of the DDAVP-7d group, while no significant differences in Epac1 and Rap1B mRNA expression were found between the two groups. Western blotting showed that Epac1 protein expression in the cochlea tissue was the highest in the DDAVP-14d group, followed by that in the DDAVP-7d group, and was the lowest in the control group, showing significant differences between groups (p <  0.05); Rap1 protein expression in the cochlea tissue was the highest in the DDAVP-7d group, followed by the DDAVP-14d group, and was the lowest in the control group, showing significant differences between groups (p <  0.05); no significant differences in Epac2 protein expression in the cochlea tissue were found among the three groups. CONCLUSION: DDAVP upregulated Epac1 protein expression in the guinea pig cochlea, leading to activation of the inner ear cAMP-Epac1 signaling pathway. This may be an important mechanism by which DDAVP regulates endolymphatic metabolism to induce EH and affect inner ear function. OXFORD CENTRE FOR EVIDENCE-BASED MEDICINE 2011 LEVELS OF EVIDENCE: Level 5.

Transmastoid exposure of the labyrinthine segment of the facial nerve: an anatomical study.

INTRODUCTION: Compression of the labyrinthine segment of the facial nerve by edema has been considered as an important pathology in the majority of the cases of idiopathic facial nerve paralysis. Hence, it is suggested that total decompression of the facial nerve should also include the labyrinthine segment by a middle fossa approach. However, the middle fossa approach requires craniotomy and temporal lobe retraction, which increases the morbidity. The labyrinthine segment of the facial nerve can also be reached through mastoidectomy. However, many ear surgeons are not familiar with this approach due to the lack of anatomical data on this surgical area. OBJECTIVE: To study the anatomical limitations of decompression of the labyrinthine segment via transmastoid approach. METHODS: Complete mastoidectomy was performed in six adult cadavers heads. Dissection was extended in the zygomatic root and posterior bony wall of the external auditory canal to visualize the incudomallear joint completely. The bone between tympanic segment, lateral and superior semicircular canal's ampullas and middle fossa dural plate was removed. Fine dissection was carried out over tympanic segment of the facial nerve in an anterosuperomedial direction the labyrinthine segment was reached. RESULTS: All the mastoids were well pneumatized. Distances between the labyrinthine segment and middle fossa dura, and between the labyrinthine segment and superior semicircular canal, were 2.5 and 4.5 mm on average, respectively. In addition, distances between the middle fossa dura and dome of the lateral semicircular canal, and between the middle fossa dura and tympanic segment were 4.6 mm and 4.3 mm on average, respectively. CONCLUSION: It is possible to expose the labyrinthine segment of the facial nerve through mastoidectomy by dissecting the bone in the area between the tympanic segment of the facial nerve, middle fossa dural plate and ampullary ends of the lateral and superior semicircular canals.

Radiological parameters and audiometric findings in otosclerosis: is there any relationship?

OBJECTIVE: The role of high-resolution computed tomography scans in otosclerosis remains uncertain. There is a debate over the relationship between radiological and audiometric findings among patients. METHOD: Pre-operative audiometry and high-resolution computed tomography findings from 40 ears with surgically confirmed otosclerosis were compared. High-resolution computed tomography scan data regarding the characteristics of the disease foci, the endosteal extension and the occurrence of internal auditory canal diverticula were obtained. The influence of each radiological variable on the simple pure tone average, the high-frequency pure tone average and the bone-conduction pure tone average were investigated. RESULTS: Cases with endosteal extension (p = 0.047) and a higher number of affected sites within the otic capsule had a worse bone-conduction pure tone average, although it was only significant for the latter (p = 0.006). Those without concomitant retrofenestral disease (p = 0.019) had better simple pure tone average. CONCLUSION: The number of sites of involvement and concomitant retrofenestral disease seem to significantly impact audiometric findings in otosclerosis.

Osteoporosis bilateral del yunque, hallazgos otopatológicos / Case report: Bilateral incus osteoporosis, otopathological findings / Relato de caso: Osteoporose bilateral da bigorna, achados otopatológicos

La osteoporosis es una enfermedad sistémica esquelética, cuyas manifestaciones más comunes son las fracturas vertebrales y de cadera. En relación con el oído, se han realizado algunos estudios controversiales que sugieren el aumento de riesgo de pérdida auditiva en pacientes con osteoporosis, mientras otros indican que no hay relación alguna con esta enfermedad. Se realizó un reporte de caso donde se describen los hallazgos en el oído medio y oído interno, tras la valoración bajo microscopía de luz, en un espécimen de hueso temporal con antecedente de osteoporosis. Se evidencia desmineralización, porosidad y disminución cualitativa del tejido óseo, así como disminución del espacio incudomaleolar.

Osteoporosis is a skeletal systemic disease, commonly known for its affection on hips and spine. In relation to the ear, several controversial studies have documented an increased risk for hearing loss in patients with osteoporosis, while others do not find any association with these disorders. A case report was carried out which describes the findings in the middle ear and inner ear, after evaluation under light microscopy, in a temporary bone specimen with a history of osteoporosis. Demineralization, porosity and qualitative diminished bone tissue are found, as well as a decrease in the incudomallear joint.

A osteoporose é uma doença esquelética sistêmica, cujas manifestações mais comuns são as fraturas vertebrais e de quadril. Em relação ao ouvido, foram realizados alguns estudos controversos que sugerem um risco aumentado de perda auditiva em pacientes com osteoporose, enquanto outros indicam que não há relação com essa doença. Foi feito um relato de caso descrevendo os achados em ouvido médio e ouvido interno, após avaliação sob microscopia de luz, em espécime de osso temporal com histórico de osteoporose. Há evidências de desmineralização, porosidade e diminuição qualitativa do tecido ósseo, bem como diminuição do espaço incudomaleolar.

The role of aquaporins in hearing function and dysfunction.

The inner ear is composed by tiny and complex structures that, together with peripheral and central auditory pathways, are responsible for hearing processing. However, not only the anatomy of the cochlea, its compartments and related structures are complex. The mechanisms involved in the regulation of homeostasis in the inner ear fluid, which determines the ionic gradient necessary for hearing and balancing sensory excitability, is an intricate phenomenon that involves several molecules. Among them, Aquaporins (AQP) play a significant role in this process. AQP are part of a family of small, integral membrane proteins that regulate different processes, including bidirectional water and ionic flow in the inner ear. Changes in the expression of these proteins are essential to auditory physiology and several pathophysiological processes in the inner ear. This review focuses on the role of AQP in health and disease of the auditory system.

El buceo recreativo y la otorrinolaringología: ¿qué sabemos y deberíamos saber? / Recreational diving and otolaryngology: what do we know and should we know?

El buceo recreativo es una práctica cada vez más popular en la población mundial, sin embargo, no está exenta de riesgos. A medida que transcurre una inmersión, el buzo es susceptible a una serie de cambios de presión que afectan las distintas cavidades que contienen aire dentro del cuerpo humano, tales como el oído, cavidades paranasales y los pulmones. Existe un gran espectro de patologías asociadas al buceo, explicándose la mayoría de ellas por el barotrauma asociado, cuya gravedad depende de la magnitud del daño asociado, pudiendo presentar desde manifestaciones a nivel local, así como también a nivel sistémico. Las patologías otológicas suelen ser las más frecuentes y el principal motivo de consulta en este tipo de pacientes. Sin embargo, las afecciones otoneurológicas, rinosinusales, de vía aérea y sistémicas pueden ser comunes dependiendo de cada perfil de buceo. Actualmente no existen recomendaciones locales sobre esta práctica, por lo que el conocimiento de la fisiología, fisiopatología y el tratamiento de las patologías otorrinolaringológicas asociadas deben ser conocidas a medida que este deporte se vuelve cada vez más popular. Se realizó una revisión de la literatura sobre las distintas afecciones otorrinolaringológicas con el fin de sistematizarlas y elaborar recomendaciones para establecer una práctica segura.

Recreational diving is an increasingly popular practice in the world; however, it is not without risks. As a dive progresses, the diver is susceptible to a series of pressure changes that affect the air-containing cavities, such as the ear, paranasal cavities, and lungs. There is a large spectrum of pathologies associated with diving, most of them being explained by associated barotrauma, the severity of which depends on the magnitude of the associated damage, could present local manifestations, as well as at systemic level. Otological pathologies are usually the most frequent and the main reason for consultation in this type of patients, however, otoneurological, rhinosinusal, airway and systemic conditions can be common depending on each diving profile. Currently there are no local recommendations on this practice, therefore, knowledge of the physiology, pathophysiology and treatment of associated otorhinolaryngological pathologies should be known as this sport becomes increasingly popular. A review of the literature on the different ear, nose and throat conditions was carried out in order to systematize them and develop recommendations to establish a safe practice.

Analysis of wideband tympanometry in Ménière's disease / Análise da timpanometria de banda larga na doença de Ménière

Abstract Introduction: Endolymphatic hydrops is the pathophysiological substrate of Ménière's disease. The changes in the inner ear, transmitted to the middle ear through changes in the ossicular chain mobility, can be quantified by wideband tympanometry, through the measurement of the acoustic absorbance at multiple frequencies, represented by the sound energy absorbed by the middle ear, even at its early stages. Studying the behavior of the middle ear through the absorbance in patients with endolymphatic hydrops under ambient pressure and under peak pressure can be useful for detecting Ménière's disease. Objective: To characterize acoustic absorbance behavior in subjects with symptomatic and asymptomatic Ménière's disease compared to controls, in order to verify the ability of wideband tympanometry to detect Ménière's disease. Methods: We carried out a cross-sectional study with a diagnostic approach comparing the findings of wideband tympanometry at ambient pressure and peak pressure between the ears of the control group (n = 30), the asymptomatic group (n = 21) and the symptomatic group (n = 9). Results: Different peak pressure values were found between the ears of the control group (0daPa), the asymptomatic group (−11 daPa) and the symptomatic group (−192 daPa), with p < 0.05 by the Kruskal-Wallis test, Mann Whitney test and Bonferroni correction. Different absorbance values were found between the ears of the symptomatic group and the asymptomatic group compared to the control group for low frequencies at ambient pressure and peak pressure, with p < 0.05 by the Kruskal-Wallis test, Mann Whitney test and Bonferroni correction. Conclusions: The Wideband Tympanometry test was capable of identifying the presence of Ménières disease, and to differentiate between asymptomatic and symptomatic patients, when comparing them with healthy individuals.

Resumo Introdução: A hidropsia endolinfática é o substrato fisiopatológico da doença de Ménière. As alterações desencadeadas na orelha interna, transmitidas à orelha média pelas modificações na mobilidade da cadeia ossicular, podem ser quantificadas pela timpanometria de banda larga, através da medida da absorvância acústica sob múltiplas frequências, representadas pela energia sonora absorvida pela orelha média, mesmo em estágios iniciais de sua instalação. Estudar o comportamento da orelha média através da absorvância em pacientes com hidropisia endolinfática sob pressão ambiente e sob o pico de pressão pode ser útil na detecção da doença de Ménière. Objetivo: Caracterizar o comportamento da absorvância em indivíduos com diagnóstico da doença de Ménière sintomáticos e assintomáticos, comparados com controles, a fim de verificar a capacidade da timpanometria de banda larga em detectar variações clínicas relacionadas a possível hidropisia endolinfática. Método: Foi realizado um estudo transversal, com enfoque diagnóstico, que comparou os achados da timpanometria de banda larga na pressão ambiente e no pico de pressão entre orelhas do grupo controle (n = 30), grupo assintomático (n = 21) e grupo sintomático (n = 9). Resultados: Foram encontrados valores do pico de pressão diferentes entre orelhas do grupo controle (0 daPa), do grupo assintomático (-11 daPa) e do grupo sintomático (-192 daPa), com p <0,05 pelos testes de Kruskal-Wallis, Mann Whitney e correção de Bonferroni. Foram encontrados valores de absorvância diferentes entre orelhas do grupo sintomático e do grupo assintomático em relação ao grupo controle para as frequências baixas na pressão ambiente e na pressão de pico, com p < 0,05 pelos testes de Kruskal-Wallis, Mann Whitney e correção de Bonferroni. Conclusão: A timpanometria de banda larga foi um teste capaz de identificar a presença da doença de Ménière e de diferenciar os pacientes assintomáticos e sintomáticos, comparando-os com indivíduos hígidos.

The neuroanatomy of Zulmasuchus querejazus (Crocodylomorpha, Sebecidae) and its implications for the paleoecology of sebecosuchians.

The endocranial structures of the sebecid crocodylomorph Zulmasuchus querejazus (MHNC 6672) from the Lower Paleocene of Bolivia are described in this article. Using computed tomography scanning, the cranial endocast, associated nerves and arteries, endosseous labyrinths, and cranial pneumatization are reconstructed and compared with those of extant and fossil crocodylomorphs, representative of different ecomorphological adaptations. Z. querejazus exhibits an unusual flexure of the brain, pericerebral spines, semicircular canals with a narrow diameter, as well as enlarged pharyngotympanic sinuses. First, those structures allow to estimate the alert head posture and hearing capabilities of Zulmasuchus. Then, functional comparisons are proposed between this purportedly terrestrial taxon, semi-aquatic, and aquatic forms (extant crocodylians, thalattosuchians, and dyrosaurids). The narrow diameter of the semicircular canals but expanded morphology of the endosseous labyrinths and the enlarged pneumatization of the skull compared to other forms indeed tend to indicate a terrestrial lifestyle for Zulmasuchus. Our results highlight the need to gather new data, especially from altirostral forms in order to further our understanding of the evolution of endocranial structures in crocodylomorphs with different ecomorphological adaptations.

New digital braincase endocasts of two species of Desmatosuchus and neurocranial diversity within Aetosauria (Archosauria: Pseudosuchia).

In the present contribution we revise, figure, and redescribe several isolated braincases of the iconic aetosaur Desmatosuchus from the Placerias Quarry locality, Chinle Formation, Arizona, United States. The detailed study of the isolated braincases from the UCMP collection allowed us to assign them at the species-level and recognize two species of Desmatosuchus for the Placerias Quarry: D. spurensis and D. smalli. The former can be distinguished from the latter by the presence of a transverse sulcus on the parietals, deep median pharyngeal recess on the basisphenoid, almost no gap between the basal tubera and the basipterygoid processes, and the exoccipitals meeting at the midline. The presence of D. smalli at the Placerias Quarry has not been previously reported. Based on the braincases UCMP 27408, 27410, 27407, three new brain endocasts were developed through CT scan images, reconstructing the most complete endocranial casts known for an aetosaur, including the encephalon, cranial nerves, inner ear, and endocranial vasculature. The cranial endocasts also exhibited some differences between both species of Desmatosuchus, with D. spurensis having a distinguishable dural expansion and markedly asymmetric anterior and posterior semicircular canals of the labyrinth. Additionally, the combination of osteological features and the endocranial casts allowed us to identify and discuss the presence of an ossified orbitosphenoid on the anteriormost region of the braincase among aetosaurs. Furthermore, we were able to reinterpret some of the observations made by previous authors on the endocast of the holotype of Desmatosuchus spurensis (UMMP VP 7476) and provide some insight into their neurosensory capabilities.

Otopathologic Abnormalities in CHARGE Syndrome.

OBJECTIVE: To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. STUDY DESIGN: Otopathologic study of human TB specimens. SETTING: Otopathology laboratories. METHODS: From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. RESULTS: Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data). CONCLUSIONS: In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

Analysis of wideband tympanometry in Ménière's disease.

INTRODUCTION: Endolymphatic hydrops is the pathophysiological substrate of Ménière's disease. The changes in the inner ear, transmitted to the middle ear through changes in the ossicular chain mobility, can be quantified by wideband tympanometry, through the measurement of the acoustic absorbance at multiple frequencies, represented by the sound energy absorbed by the middle ear, even at its early stages. Studying the behavior of the middle ear through the absorbance in patients with endolymphatic hydrops under ambient pressure and under peak pressure can be useful for detecting Ménière's disease. OBJECTIVE: To characterize acoustic absorbance behavior in subjects with symptomatic and asymptomatic Ménière's disease compared to controls, in order to verify the ability of wideband tympanometry to detect Ménière's disease. METHODS: We carried out a cross-sectional study with a diagnostic approach comparing the findings of wideband tympanometry at ambient pressure and peak pressure between the ears of the control group (n = 30), the asymptomatic group (n = 21) and the symptomatic group (n = 9). RESULTS: Different peak pressure values were found between the ears of the control group (0 daPa), the asymptomatic group (-11 daPa) and the symptomatic group (-192 daPa), with p < 0.05 by the Kruskal-Wallis test, Mann Whitney test and Bonferroni correction. Different absorbance values were found between the ears of the symptomatic group and the asymptomatic group compared to the control group for low frequencies at ambient pressure and peak pressure, with p < 0.05 by the Kruskal-Wallis test, Mann Whitney test and Bonferroni correction. CONCLUSIONS: The Wideband Tympanometry test was capable of identifying the presence of Ménière´s disease, and to differentiate between asymptomatic and symptomatic patients, when comparing them with healthy individuals.

Sordera neurosensorial congénita con malformación de oído interno ligada al X en una familia mexicana / Congenital sensorineural deafness X-linked, with inner ear malformation in a Mexican family

Introducción. Las sorderas o hipoacusias prelinguales son de etiología genética entre el 60 y el 68% de los casos; de estos, del 20 al 40% son malformaciones del oído interno. De los casos de hipoacusia no sindrómica ligada al X se han descrito siete tipos. De las malformaciones de oído interno, la partición coclear incompleta tipo III es la menos frecuente.Objetivo. Presentar el reporte clínico-genético de una familia mexicana, con indi-viduos varones afectados por sordera neurosensorial congénita con malformación de oído interno. Material y Métodos. Se realizó estudio de una familia en la que nueve miembros presentaban sordera. Se estudiaron cuatro de ellos y una madre sin manifestaciones, a través del estudio clínico general por médico genetista, el estudio audiológico (otos-copía y audiometría) por médico audiólogo y el estudio de tomografía computada (TC) por médico radiólogo.Resultados. Los pacientes estudiados presentaron sordera neurosensorial congéni-ta, de severa a profunda bilateral. A través de la TC, se evidenció malformación de oído interno. Tres pacientes presentaron partición coclear incompleta tipo III y un paciente partición incompleta tipo I. Debido al estudio clínico y al árbol genealógico, se definió diagnóstico de hipoacusia neurosensorial no sindrómica ligada al X. La TC de la madre sin manifestaciones no presentó evidencia de malformaciones en oído interno (MOI).Conclusión. El estudio de imagen es fundamental para definir presencia o no de MOI en todos los pacientes con hipoacusia y así poder guiar la terapéutica y el aseso-ramiento genético, así como realizar los estudios moleculares más adecuados

Introduction. The pre-lingual deafness or hearing loss are of genetic cause in be-tween 60% and 68% of cases, among these, between 20% and 40% are malforma-tion of the inner ear. From the non-syndromic hearing loss cases that are linked to the X chromosome, seven types have been described. Among these inner ear malforma-tions, incomplete cochlear partition type III is the less frequent.Objective. Present the clinical genetical report of a Mexican family, with male in-dividuals affected by congenital neurosensory deafness with inner ear malformation.Materials and methodology. A study on a family in which nine members were affected by deafness was done. Four of them, plus a mother without manifestation, were studied through a general clinical study by a geneticist, an audiological study (otoscopy and audiometry) by an audiologist, and a computed tomography (CT) scan by a radiologist.Results. The studied patients presented congenital neurosensory deafness, from se-vere to deep bilateral. Via the CT, the inner ear malformation was made clear. Three of the patients presented incomplete cochlear partition type III and one patient in-complete cochlear partition type I. Due to the clinical study and the family tree, it was diagnosed non-syndromic neurosensory deafness linked to X. The CT of the mother without manifestation did not show evidence of inner ear malformations.Conclusion. The study by image is fundamental to define whether there is or not a presence of inner ear malformations in any patient with heading loss to be able to guide the therapeutics and the genetic counseling, as well as to make more accurate molecular studies

Internal acoustic opening: different osseous landmarks and their clinical implications / Poro acústico interno: diferentes hitos óseos y sus implicaciones clínicas

SUMMARY: Internal acoustic opening is a space that opens to facies posterior of the petrous piece of temporal bone that goes inside facial nerve, vestibulocochlear nerve, intermedial nevre and labyrinthine artery. The purpose of this study is the assessment of internal acoustic opening from a morphometric perspective, determination of the shape of the hole and determination of the distance to some important anatomic formations. This study is conducted on 166 temporal bones with unknown sex formation which are part of the skull collection in NEU and KTO Karatay University, Anatomy Department. In this study, the vertical and diameter of internal acoustic meatus, its distance to the bottom and top sides of posterior surface of the petrous part, its distance to groove for superior sagittal sinus and its distance to apex were measured. Moreover, in this study internal acoustic opening spaces are categorized into six groups as round, oval, U-shaped, fissure, irregular and V shape. Digital caliper was used for internal acoustic meatus measurements. While the vertical horizontal diameters and distance to groove for superior sagittal sinus of internal acoustic opening on the right side are 4.12 mm, 6.83 mm and 19.64mm respectively, they are 4.56 mm, 7.10 mm and 21.06 mm on the left side respectively. We have observed in this study, 37.3 % of the internal acoustic opening as round, 34.3 % as oval, 6.6 % as U-shaped, 6.6 % as fissure, 12.7 % as irregular and 2.4 % as V-shaped. We believe that these measurements can provide guidance and help in surgical procedures.

RESUMEN: El poro acústico interno es un espacio que se abre en la cara posterior de la parte petrosa del hueso temporal, donde entran los nervios facial, intermedio y vestibulococlear, además de la arteria laberíntica. El propósito de este estudio fue la evaluación del poro acústico interno desde una perspectiva morfométrica, determinación de la forma del foramen y de la distancia a algunas formaciones anatómicas importantes. Este estudio se realizó en 166 huesos temporales de individuos de sexo desconocido que forman parte de la colección de cráneos del Departamento de Anatomía en NEU y KTO, Universidad de Karatay, Se midió la altura vertical y el diámetro del poro acústico interno, su distancia a los lados inferior y superior de la superficie posterior de la parte petrosa, su distancia al surco del seno sagital superior y su distancia al vértice. Además, el poro acústico interno se clasificó en seis grupos: redondos, ovalados, en forma de U, de fisura, irregulares y en forma de V. Se utilizó un calibrador digital para las mediciones del meato acústico interno. Mientras que los diámetros horizontales, verticales y la distancia al surco para el seno sagital superior del poro acústico interno en el lado derecho fue de 4,12 mm, 6,83 mm y 19,64 mm respectivamente, en el lado izquiedo fue de 4,56 mm, 7,10 mm y 21,06 mm, respectivamente. Hemos observado que en el 37,3 % de los casos el poro acústico interno era redondo, el 34,3 % ovalado, el 6,6 % en forma de U, el 6,6 % de fisura, el 12,7 % irregular y el 2,4 % en forma de V. Estas medidas pueden servir de guía y ayuda en los procedimientos quirúrgicos.

Internal auditory canal hypoplasia associated with bilateral vestibulocochlear nerve aplasia and deviant facial nerve course: A case report and MRI findings.

The evaluation of internal auditory canals and cochlea has gained significant importance due to the increasing number of cochlear implantations worldwide. This region's anatomical study is essential for cochlear implant surgery using magnetic resonance imaging as the method of choice. We report a case of a 6-year-old male patient diagnosed with a rare bilateral malformation of the internal auditory canals associated with an aberrant course of the facial nerve and vestibulocochlear nerve aplasia. This report raises the importance of identifying this rare malformation for appropriate management and reinforces awareness of possible complications.

Disfunções metabólicas e vertigem: apresentação e revisão dos métodos diagnósticos / Metabolic dysfunctions and vertigo: presentation and review of diagnostic methods

Contexto: A tontura é um sintoma de alta prevalência e as labirintopatias de causas metabólicas destacam-se como uma das mais frequentes em nosso meio. Distúrbios do metabolismo glicêmico, disfunções tireoidianas e dislipidemias são as principais. Objetivos: Os objetivos deste estudo são descrever a porcentagem das principais disfunções metabólicas nos pacientes com vertigem e revisar os métodos diagnósticos. Desenho e local: Estudo observacional de pacientes atendidos no setor de Otoneurologia do Instituto Penido Burnier. Métodos: Avaliação de 506 pacientes com vertigem por meio da análise do hormônio tireoestimulante, glicemia de jejum, hemoglobina glicada, insulina de jejum, curvas glicoinsulinêmica de três horas, colesterol total e suas frações (lipoproteína de alta densidade [HDL] e lipoproteína de baixa densidade [LDL]) e triglicerídeos. Foi feita a comparação entre os sexos e com os dados encontrados na população geral. Resultados: Níveis de HDL, triglicerídeos e glicemia de jejum foram mais alterados no sexo masculino e o colesterol total foi mais elevado no sexo feminino. A elevação do HOMA (homeostatic model assessment) foi a alteração mais significante encontrada no grupo em estudo comparando com a população geral, entretanto as curvas glicoinsulinêmicas mostraram mais alterações em comparação ao HOMA. Discussão: A análise do HOMA e da curva glicoinsulinêmica mostrou que a porcentagem de alteração é diferente entre os métodos, não havendo concordância entre eles. O HOMA não substitui as curvas na investigação dos pacientes com vertigem e suspeita de distúrbios do metabolismo glicêmico. Conclusão: É alta a porcentagem das disfunções metabólicas na população com vertigem, justificando a investigação laboratorial neste grupo de pacientes.

Análise das respostas do VEMP cervical e ocular em indivíduos hígidos / Analysis of cervical and ocular VEMP responses in healthy individuals / Análisis de las respuestas VEMP cervicales y oculares en individuos sanos

Introdução: os potenciais evocados miogênicos vestibulares (VEMP) são respostas eletrofisiológicas que conseguem fornecer informações dos órgãos otolíticos sáculo, utrículo e do nervo vestibular. O VEMP é um exame complementar à avaliação vestibular, consistindo num exame rápido, de fácil aplicação e objetivo. Objetivo: analisar os parâmetros de latência, amplitude, limiar e índice de assimetria das respostas do VEMP cervical (cVEMP) e ocular (oVEMP) de indivíduos sem queixas vestibulares. Métodos: estudo transversal realizado com 53 indivíduos de ambos os sexos, sem queixas auditivas e vestibulares. Resultados: encontrou-se simetria de respostas nas latências, amplitudes e limiares de respostas do exame cVEMP. Entretanto, verificou-se diferença entre orelhas da latência P15 do exame oVEMP, sendo maior à direita no sexo feminino. Conclusão: Encontrou-se simetria nas respostas de todos os parâmetros avaliados do cVEMP. Houve assimetria apenas na latência de P15 do oVEMP no sexo feminino. Os limiares de resposta encontrados nos exames cVEMP e oVEMP foram iguais ou maiores que 75 dBNA.

Introduction: Vestibular evoked myogenic potentials (VEMP) are electrophysiological responses that can provide information on the otolithic organs saccule, utricle and of the vestibular nerve. VEMP is a complementary exam to the vestibular assessment; it is a quick exam, easy to apply and objective. Purpose: to analyze the parameters of latency, amplitude, threshold and asymmetry index of the cervical (cVEMP) and ocular VEMP (oVEMP) responses of individuals without vestibular complaints. Methods: cross-sectional study carried out with 53 individuals of both genders without hearing and vestibular complaints. Results: response symmetry was found in the latencies, amplitudes and thresholds of cVEMP test responses. However, there was a difference between the ears of the P15 latency of the oVEMP exam, and this was greater on the right ear in females. Conclusion: symmetry was found in the responses of all cVEMP evaluated parameters. There was asymmetry in oVEMP P15 latency only in female patients. The response thresholds found in the cVEMP and oVEMP tests were equal or greater than 75 dBHL.

Introducción: los potenciales miogénicos evocados vestibulares (VEMP) son respuestas electrofisiológicas que pueden proporcionar información sobre los órganos otolíticos el sáculo, el utrículo y el nervio vestibular. El VEMP es un examen complementario a la evaluación vestibular; es un examen rápido, fácil de aplicar y objetivo. Objetivo: analizar los parámetros de latencia, amplitud, umbral e índice de asimetría de las respuestas VEMP cervical (cVEMP) y ocular (oVEMP) de individuos sin quejas vestibulares. Métodos: estudio transversal realizado con 53 individuos de ambos sexos, sin quejas auditivas y vestibulares. Resultados: Se encontró simetría de respuestas en las latencias, amplitudes y umbrales de respuestas en el examen cVEMP. Sin embargo, hubo una diferencia entre los oídos de la latencia P15 del examen oVEMP, siendo mayor a la derecha en el sexo femenino . Conclusión: se encontró simetría en las respuestas de todos los parámetros evaluados de cVEMP. Hubo asimetría solo en la latencia P15 de oVEMP en el sexo femenino. Los umbrales de respuesta encontrados en las pruebas cVEMP y oVEMP fueron iguales o superiores a 75 dBHL.