Monitoring Cochlear Health With Intracochlear Electrocochleography During Cochlear Implantation: Findings From an International Clinical Investigation.

OBJECTIVES: Electrocochleography (ECochG) is emerging as a tool for monitoring cochlear function during cochlear implant (CI) surgery. ECochG may be recorded directly from electrodes on the implant array intraoperatively. For low-frequency stimulation, its amplitude tends to rise or may plateau as the electrode is inserted. The aim of this study was to explore whether compromise of the ECochG signal, defined as a fall in its amplitude of 30% or more during insertion, whether transient or permanent, is associated with poorer postoperative acoustic hearing, and to examine how preoperative hearing levels may influence the ability to record ECochG. The specific hypotheses tested were threefold: (a) deterioration in the pure-tone average of low-frequency hearing at the first postoperative follow-up interval (follow-up visit 1 [FUV1], 4 to 6 weeks) will be associated with compromise of the cochlear microphonic (CM) amplitude during electrode insertion (primary hypothesis); (b) an association is observed at the second postoperative follow-up interval (FUV2, 3 months) (secondary hypothesis 1); and (c) the CM response will be recorded earlier during electrode array insertion when the preoperative high-frequency hearing is better (secondary hypothesis 2). DESIGN: International, multi-site prospective, observational, between groups design, targeting 41 adult participants in each of two groups, (compromised CM versus preserved CM). Adult CI candidates who were scheduled to receive a Cochlear Nucleus CI with a Slim Straight or a Slim Modiolar electrode array and had a preoperative audiometric low-frequency average thresholds of ≤80 dB HL at 500, 750, and 1000 Hz in the ear to be implanted, were recruited from eight international implant sites. Pure tone audiometry was measured preoperatively and at postoperative visits (FUV1 and follow-up visit 2 [FUV2]). ECochG was measured during and immediately after the implantation of the array. RESULTS: From a total of 78 enrolled individuals (80 ears), 77 participants (79 ears) underwent surgery. Due to protocol deviations, 18 ears (23%) were excluded. Of the 61 ears with ECochG responses, amplitudes were < 1 µV throughout implantation for 18 ears (23%) and deemed "unclear" for classification. EcochG responses >1 µV in 43 ears (55%) were stable throughout implantation for 8 ears and compromised in 35 ears. For the primary endpoint at FUV1, 7/41 ears (17%) with preserved CM had a median hearing loss of 12.6 dB versus 34/41 ears (83%) with compromised CM and a median hearing loss of 26.9 dB ( p < 0.014). In assessing the practicalities of measuring intraoperative ECochG, the presence of a measurable CM (>1 µV) during implantation was dependent on preoperative, low-frequency thresholds, particularly at the stimulus frequency (0.5 kHz). High-frequency, preoperative thresholds were also associated with a measurable CM > 1 µV during surgery. CONCLUSIONS: Our data shows that CM drops occurring during electrode insertion were correlated with significantly poorer hearing preservation postoperatively compared to CMs that remained stable throughout the electrode insertion. The practicality of measuring ECochG in a large cohort is discussed, regarding the suggested optimal preoperative low-frequency hearing levels ( < 80 dB HL) considered necessary to obtain a CM signal >1 µV.

Extracorporeal septoplasty with internal nasal valve stabilisation.

Among various septoplasty techniques, the extracorporeal one is used for severe deformities of the caudal septum and consists essentially in removal of the nasal septum followed by correction of deformities. Reconstruction of the neo-septum is carried out by repositioning the septal fragments in a straight position. The disadvantages of this surgical technique are the septal haematoma, oedema of the mucosa in the valve area and some types of abnormalities of the middle third of the nose such as saddling of the dorsum. All of these conditions can be associated with various degrees of functional disorders. To prevent these possible complications, we developed a suture technique to fix the caudal portion of the neo-septum and avoid alterations or narrowing of the internal nasal valve. The purpose of this study is to describe extracorporeal septoplasty results with this suture technique in stabilising the internal nasal valve. From January 2011 to December 2013, a retrospective review of adult patients treated with extracorporeal septoplasty was performed at the ENT department of Imola Hospital. Pre- and post-operative evaluations were carried out by rhinomanometry and acoustic rhinometry. Statistical analysis was performed with commercially available software (IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp). 133 cases fulfilled inclusion criteria and were enrolled. A significant improvement was evident after surgery based on the results of rhinomanometry and acoustic rhinology. Extracorporeal septoplasty with stabilisation of the internal nasal valve is an effective and reproducible surgical technique that yields optimal functional results.

Obstrucción nasal congénita por estenosis de la apertura piriforme. Serie de casos / Congenital nasal obstruction due to pyriform aperture stenosis. A case series

La obstrucción de las fosas nasales en el neonato es un cuadro potencialmente fatalpor su respiración nasal obligada. La causa principal es inflamatoria o infecciosa, y con menor frecuencia puede ser de origen congénito, neoplásico, traumático o iatrogénico. La atresia de coanas es la anomalía congénita nasal más común. Una etiología menos frecuente de obstrucción nasal congénita es la estenosis de la apertura piriforme. Debe pensarse en esta última en todo recién nacido con cornaje y dificultad respiratoria de grado variable, asociado a la dificultad de pasar una sonda a través de la región anterior de las fosas nasales. El diagnóstico se confirma por tomografía computarizada del macizo craneofacial. La conducta terapéutica dependerá de la gravedad de los síntomas. Describimos nuestra experiencia con 5 pacientes que presentaban esta afección, tratados quirúrgicamente mediante abordaje sublabial y colocación de tutor nasal

Nasal obstruction in neonates is a potentially fatal condition due to their exclusive nasal breathing. The main cause is inflammatory or infectious rhinitis. Congenital, neoplastic, traumatic or iatrogenic causes are less frequent. Choanal atresia is the most common congenital nasal anomaly. A less common etiology of congenital nasal obstruction is pyriform aperture stenosis. Suspicion might arise in any newborn with varying degrees of stridor and respiratory distress, associated with the difficulty of passing a probe through anterior nares. Diagnosis should be confirmed by a computed tomography of the craniofacial massif. The therapeutic approach will depend on the severity of symptoms. We describe our experience with 5 patients with this condition, treated surgically using a sub-labial approach, and followed by nasal stenting

[Congenital nasal obstruction due to pyriform aperture stenosis. A case series]. / Obstrucción nasal congénita por estenosis de la apertura piriforme. Serie de casos.

Nasal obstruction in neonates is a potentially fatal condition due to their exclusive nasal breathing. The main cause is inflammatory or infectious rhinitis. Congenital, neoplastic, traumatic or iatrogenic causes are less frequent. Choanal atresia is the most common congenital nasal anomaly. A less common etiology of congenital nasal obstruction is pyriform aperture stenosis. Suspicion might arise in any newborn with varying degrees of stridor and respiratory distress, associated with the difficulty of passing a probe through anterior nares. Diagnosis should be confirmed by a computed tomography of the craniofacial massif. The therapeutic approach will depend on the severity of symptoms. We describe our experience with 5 patients with this condition, treated surgically using a sub-labial approach, and followed by nasal stenting.

[Angiomyolipoma and Lenk syndrome: case report]. / Triada de Lenk como presentación clínica del angiomiolipoma.

Angiomyolipoma (AML) is a benign clonal neoplasm that consists of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS), an autosomal-dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceous, a distinctive skin lesion. Massive retroperitoneal hemorrhage from AML, also known as Wunderlich's syndrome, has been found in up to 10% of patients and represents the most significant and feared complication. The presence of even a small amount of fat within a renal lesion on CT scan (confirmed by Hounsfield unit's < or = 10) is considered diagnostic of AML. Intervention should be considered for larger tumors, particularly if the patient is symptomatic, taking into account patient age, comorbidities, and other related factors. A nephron-sparing approach, via either partial nephrectomy or selective embolization, is clearly preferred. We present the case of a fifty-nine-year old patient with an angiomyolipoma and massive retroperitoneal hemorrhage with Lenk syndrome.

Tríada de Lenk como presentación clínica del angiomiolipoma / Angiomyolipoma and Lenk syndrome: case report

El angiomiolipoma (AML) es una neoplasia clonal benigna compuesta por cantidades variables de tejido adiposo maduro, músculo liso y vasos sanguíneos de paredes gruesas. Aproximadamente un 20% de todos los AML, se detectan en pacientes con el Síndrome de la Esclerosis Tuberosa, trastorno autonómico dominante que se asocia a retraso mental, epilepsia, adenoma sebáceo. La presencia de hemorragia retroperitoneal masiva, conocida como Síndrome de Wunderlich se ha documentado en hasta un 10% de los pacientes y representa la complicación más importante y temida de estas lesiones. La TAC identifica las zonas del tejido adiposo, correspondiente a una baja atenuación entre 20-100Hounsfield, dicho coeficiente dependerá del contenido y madurez de la grasa intratumoral y de la presencia o no de hemorragias. En el caso de pacientes con AML pequeños que requieren intervención debido a la presencia de síntomas, en pacientes con ET o AML multicéntricos y en pacientes en quienes la preservación de la función renal reviste particular importancia el enfoque preferido es la cirugía ahorradora de nefronas con nefrectomía parcial o embolizaciòn selectiva. Presentamos un caso de una paciente de 59 años con un angiomiolipoma gigante quién debuta con el Síndrome clínico conocido como Tríada de Lenk (AU)

Angiomyolipoma (AML) is a benign clonal neoplasm that consists of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS), an autosomaldominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceous, a distinctive skin lesion. Massive retroperitoneal hemorrhage from AML, also known as Wunderlich's syndrome, has been found in up to 10% of patients and represents the most significant and feared complication. The presence of even a small amount of fat within a renal lesion on CT scan (confirmed by Hounsfieldun it’s <=10) is considered diagnostic of AML. Intervention should be considered for larger tumors, particularly if the patient is symptomatic, taking into account patient age, comorbidities, and other related factors. A nephron-sparing approach, via either partial nephrectomy or selective embolization, is clearly preferred. We present the case of a fifty-nine-year old patient with an angiomyolipoma and massive retroperitoneal hemorrhage with Lenk syndrome (AU)

Asistencia endoscópica en la cirugía de la fístula traqueoesofágica / Assistance endoscopic in the surgery of the tracheoesophageal fistula

La fístula traqueoesofágica congénita, sin atresia de esófago, conocida como fístula en H, es una malformación rara de la vida aérea, que ocurre en sólo en 4 por ciento de las malformaciones traqueoesofágica y su incidencia es de 1:50.000-80.000 nacimientos. El tratamiento de elección es el cierre quirúrgico de la fístula. Para este procedimiento es siempre preferido el abordaje cervical sobre el torácico ya presenta una mejor complejidad y morbilidad. La asistencia endoscópica por broncoscópia esofagoscopía rígidas permite la cateterización del trayecto fistuloso, la individualización de la fístula y la movilización de la misma dentro del cuello, para ofrecer un acceso más fácil por cervicotomía lateral. Se presenta al Servicio de Otorrinolaringología del Hospital Italiano de Buenos Aires una paciente de 2 meses de edad con falta de progreso de peso, tos emetizante y fiebre sin foco. Como antecedente habia padecido una neumonía tratada en los quince días previos.Frente a la sospecha de episodios aspirativos, se realizó Videodeglución donde se detectó Fístula Traqueoesofágica en H. Se realizó también Rinofibrolaringoscópia para evaluación dinámica de laringe. Se decide cierre quirúrgico de la fístula a través de abordaje crevical, para lo que se planeó asistencia endoscópica para cateterización e identificación de la misma.