Perineal hypospadias repair with preservation of a coincidental vagina or perineal utricle in boys with disorders of sex development.

INTRODUCTION: An enlarged utricle in patients with proximal hypospadias or disorders of sex development (DSD) is common. The utricle orifice is usually in the posterior urethra near the verumontanum, but in exceptional cases located on the perineum. Concurrence of a perineal hypospadias and perineal utricle or vagina is also known as male vagina, pseudovagina, or blind vaginal pouch. The utricle in such cases is usually excised either before or concomitant with hypospadias repair. The authors developed an alternative approach in which the vagina or perineal utricle is retained during hypospadias repair and report on the results in four patients. OBJECTIVE: To report a novel technique for perineal hypospadias repair while retaining a concurrent vagina or perineal utricle. PATIENTS AND METHODS: Between 1999 and 2014, four neonates presented with perineal hypospadias. In all patients, a second perineal opening providing access to either an enlarged utricle or a vagina was identified. Karyotype in peripheral blood was in two patients 46,XY and in the other two 45,X/46,XY of which one showed a complex mosaicism in gonadal tissue. No genetic cause was identified on DNA evaluation in the two patients with 46,XY DSD. All patients were raised as boys. Hypospadias repair was performed in two stages at prepubertal age. During the second stage of surgery, performed between the age of 1.5 and 5 years, the vagina or utricle orifice was incorporated into the neo-urethra, resulting in a retained 'built-in' vagina or utricle. RESULTS: Surgical procedures were uneventful, and patients remained asymptomatic during a mean postoperative follow-up of 8.5 (range 2-13.5) years. One patient was lost to follow-up after the age of 8 years. At their last visit, the remaining patients, at the age of 4, 15, and 17 years, were able to void in standing position without dribbling. Both adolescent patients reported erections without ejaculations and identified themselves as males without signs of gender dysphoria. CONCLUSION: Hypospadias repair in boys with perineal hypospadias while leaving a male vagina or perineal utricle in situ has not been reported previously, and the study's preliminary results are favorable. One of the benefits of this approach is that inadvertent injury to adjacent anatomic structures such as urethral sphincter, neurovascular bundles, ureters, vas deferens, and rectum is avoided. The main rationale for adopting this conservative approach however is to minimize genital tissue removal in children with a not yet definite gender identity, which will certainly facilitate unforeseen future gender reassignment surgery.

[Recent surgical options for vestibular vertigo]. / Aktuelle operative Therapie des vestibulären Schwindels.

Vertigo is not a well defined symptom but a heterogenous entity diagnosed and treated mainly by otolaryngologists, neurologists, internal medicine and primary care physicians. Most vertigo syndroms have a good prognosis and management is predominantly conservative, whereas the need for surgical therapy is rare, but for a subset of patients often the only remaining option. In this paper, we describe the development of surgical therapy for hydropic inner ear diseases, Menière disease, dehiscence syndroms, perilymphatic fistulas, and benign paroxysmal vertigo. At the end, we shortly introduce the most recent development of vestibular implants. Surgical vestibular therapy is still indicated for selected patients nowadays when conservative options did not reduce symptoms and patients are still suffering. Success depends on the correct diagnosis and indication for the different procedures going along with an adequate patient selection. In regard to the invasiveness and the possible risks due to the surgery, in depth individual counselling is necessary. Ablative and destructive surgical procedures usually achieve a successful vertigo control, but go along with a high risk for hearing loss. Therefore, residual hearing has to be included in the decission making process for a surgical therapy.

Otopathology in Osteogenesis Imperfecta.

BACKGROUND: Osteogenesis Imperfecta (OI) is a genetic disorder of connective tissue matrix. OI is caused by mutations that affect type I collagen. The hearing loss in OI is characterized by onset in early adulthood and can be conductive, sensorineural, or mixed. OBJECTIVES: To describe the temporal bone histopathology in 9 individuals with OI. MATERIALS AND METHODS: Four adult, 1 pediatric, and 4 infant specimens were identified. Temporal bones were removed at autopsy and studied using light microscopy. RESULTS: All adults and 1 pediatric specimen showed otosclerotic lesions. The findings included examples of clinical, histologic, and cochlear otosclerosis. The temporal bones of infants showed delayed ossification of the endochondral layer of bone and of the ossicles. There were no infant specimens with otosclerotic lesions. CONCLUSION: Hearing loss in OI may be the result of clinical or cochlear otosclerosis. Fracture or atrophy of the ossicles may also be present in OI. A third unidentified mechanism of hearing loss may lead to cochlear degeneration. The described findings of otosclerotic lesions have implications for the observed heterogeneity of hearing loss patterns and for the surgical management of hearing loss in OI.

The tangential nucleus controls a gravito-inertial vestibulo-ocular reflex.

BACKGROUND: Although adult vertebrates sense changes in head position by using two classes of accelerometer, at larval stages zebrafish lack functional semicircular canals and rely exclusively on their otolithic organs to transduce vestibular information. RESULTS: Despite this limitation, we find that larval zebrafish perform an effective vestibulo-ocular reflex (VOR) that serves to stabilize gaze in response to pitch and roll tilts. By using single-cell electroporations and targeted laser ablations, we identified a specific class of central vestibular neurons, located in the tangential nucleus, that are essential for the utricle-dependent VOR. Tangential nucleus neurons project contralaterally to extraocular motoneurons and in addition to multiple sites within the reticulospinal complex. CONCLUSIONS: We propose that tangential neurons function as a broadband inertial accelerometer, processing utricular acceleration signals to control the activity of extraocular and postural neurons, thus completing a fundamental three-neuron circuit responsible for gaze stabilization.

Assessing cochlear evoked potentials after electrocautery-induced trauma to the utricle.

UNLABELLED: Utriculostomy is a new surgical alternative for Ménière's disease. The basis of this procedure is that the outcome of an electrocautery-induced utricular trauma does not affect cochlear function. However, a demonstration of the hypothesis that this approach to the utricle would preserve hearing is still pending. OBJECTIVE: To determine whether any changes would occur in the electrical potentials evoked in the cochlea and auditory nerve before, during, and 1 month after a surgical procedure in the utricule in an animal model. MATERIALS AND METHODS: An experimental study. Eight sheep underwent electrocautery-induced utricular trauma, and their cochlear function was assessed by electrocochleography--recording of electrical evoked potentials, in the preoperative, immediate postoperative and medium-term postoperative periods. The results were analyzed statistically. RESULTS: There were no statistically significant variations in amplitude (p = 0.099) and latency (p = 0.591) before and 1 month after the surgical procedure. There was a statistically significant change in the summation of the potential/action potential area ratio (p = 0.0122), a calculated loss of 11.8 dB. CONCLUSION: The intervention performed in this study enabled us to conclude that, taking into account the impaired electrophysiological responses observed during and 1 month after the surgical procedure, hearing was preserved in the operated sheep.

Dissection of adult mouse utricle and adenovirus-mediated supporting-cell infection.

Hearing loss and balance disturbances are often caused by death of mechanosensory hair cells, which are the receptor cells of the inner ear. Since there is no cell line that satisfactorily represents mammalian hair cells, research on hair cells relies on primary organ cultures. The best-characterized in vitro model system of mature mammalian hair cells utilizes organ cultures of utricles from adult mice (Figure 1). The utricle is a vestibular organ, and the hair cells of the utricle are similar in both structure and function to the hair cells in the auditory organ, the organ of Corti. The adult mouse utricle preparation represents a mature sensory epithelium for studies of the molecular signals that regulate the survival, homeostasis, and death of these cells. Mammalian cochlear hair cells are terminally differentiated and are not regenerated when they are lost. In non-mammalian vertebrates, auditory or vestibular hair cell death is followed by robust regeneration which restores hearing and balance functions. Hair cell regeneration is mediated by glia-like supporting cells, which contact the basolateral surfaces of hair cells in the sensory epithelium. Supporting cells are also important mediators of hair cell survival and death. We have recently developed a technique for infection of supporting cells in cultured utricles using adenovirus. Using adenovirus type 5 (dE1/E3) to deliver a transgene containing GFP under the control of the CMV promoter, we find that adenovirus specifically and efficiently infects supporting cells. Supporting cell infection efficiency is approximately 25-50%, and hair cells are not infected (Figure 2). Importantly, we find that adenoviral infection of supporting cells does not result in toxicity to hair cells or supporting cells, as cell counts in Ad-GFP infected utricles are equivalent to those in non-infected utricles (Figure 3). Thus adenovirus-mediated gene expression in supporting cells of cultured utricles provides a powerful tool to study the roles of supporting cells as mediators of hair cell survival, death, and regeneration.

Avaliação dos potenciais evocados cocleares após lesão do utrículo por eletrocauterização / Assessing cochlear evoked potentials after electrocautery-induced trauma to the utricle

A utriculostomia é uma nova alternativa cirúrgica para a doença de Ménière. A hipótese cirúrgica desse procedimento é a de que o desfecho de uma lesão do utrículo por eletrocauterização não interfere na função coclear. Contudo, a hipótese de que essa abordagem do utrículo preserva a audição ainda não foi comprovada. OBJETIVO: Determinar se ocorrem mudanças nos potenciais elétricos evocados na cóclea e no nervo auditivo antes, durante e 1 mês após o procedimento cirúrgico no utrículo em modelo animal. MATERIAIS E MÉTODOS: Estudo experimental: Oito ovelhas foram submetidas à lesão do utrículo por eletrocauterização e sua função coclear foi avaliada por meio de eletrococleografia, registrando-se os potenciais elétricos evocados nos períodos pré-operatório, pós-operatório imediato e pós-operatório de médio prazo. Os resultados foram analisados estatisticamente. RESULTADOS: Não houve variação estatisticamente significativa na amplitude (p = 0,099) e na latência (p = 0,591) antes e 1 mês após o procedimento cirúrgico. Houve alteração estatisticamente significativa na razão entre potencial de somação/potencial de ação (p = 0,0122), representando uma perda calculada de 11,8 dB. CONCLUSÃO: A intervenção realizada no utrículo neste estudo permite concluir que, tendo em conta respostas eletrofisiológicas deficientes observadas durante e 1 mês após o procedimento cirúrgico, houve preservação da audição nas ovelhas operadas.

Utriculostomy is a new surgical alternative for Ménière's disease. The basis of this procedure is that the outcome of an electrocautery-induced utricular trauma does not affect cochlear function. However, a demonstration of the hypothesis that this approach to the utricle would preserve hearing is still pending. OBJECTIVE: To determine whether any changes would occur in the electrical potentials evoked in the cochlea and auditory nerve before, during, and 1 month after a surgical procedure in the utricule in an animal model. MATERIALS AND METHODS: An experimental study. Eight sheep underwent electrocautery-induced utricular trauma, and their cochlear function was assessed by electrocochleography - recording of electrical evoked potentials, in the preoperative, immediate postoperative and medium-term postoperative periods. The results were analyzed statistically. RESULTS: There were no statistically significant variations in amplitude (p = 0.099) and latency (p = 0.591) before and 1 month after the surgical procedure. There was a statistically significant change in the summation of the potential/action potential area ratio (p = 0.0122), a calculated loss of 11.8 dB. CONCLUSION: The intervention performed in this study enabled us to conclude that, taking into account the impaired electrophysiological responses observed during and 1 month after the surgical procedure, hearing was preserved in the operated sheep.

Impact of endolymphatic mastoid shunt surgery on saccule and lateral semicircular canal function.

Endolymphatic mastoid shunt surgery (EMSS) is widely performed in patients with medically intractable Meniere's disease. Although many patients report an improvement of symptoms after surgery, the mechanisms which are responsible for the relief of complaints are not known. To date, only few studies exist which studied the influence of EMSS on vestibular function. The present study examines the effect of EMSS on saccule function by measuring vestibular evoked myogenic potentials and the effect on lateral semicircular canal function by sinusoidal harmonic acceleration (SHA) testing. No changes in vestibulo-collic reflexes were found after surgery compared to before surgery. SHA testing resulted in comparable phase lag and gain pre- and postoperatively. Although central compensation is clinically evident no effect in specific vestibular diagnostic testing is seen. Modulations of canal-otolith interaction might suggest a change of symptoms. The only method so far to evaluate the success of EMSS is the patient's subjective assessment.

Morphology and function of Bast's valve: additional insight in its functioning using 3D-reconstruction.

The utriculo-endolymphatic valve was discovered by Bast in 1928. The function of Bast's valve is still unclear. By means of orthogonal-plane fluorescence optical sectioning (OPFOS) microscopy 3D-reconstructions of the valve and its surrounding region are depicted. The shape of the duct at the utricular side is that of a flattened funnel. In the direction of the endolymphatic duct and sac this funnel runs into a very narrow duct. The valve itself has a rigid 'arch-like' configuration. The opposing thin, one cell-layer thick, utricular membrane is highly compliant. We propose that opening and closure of the valve occurs through movement of the flexible base/utricular membrane away from and toward the relatively rigid valve lip.

Surgical treatment of peripheral vertigo and vertiginous diseases.

Although our understanding of the mechanisms of vertigo and pathophysiology of vertiginous disorders has increased, diagnosis and treatment of various vertiginous diseases is challenging. The objective for the treatment of a vertiginous disease is to eliminate the underlying pathology either with maneuvers or drugs. In vertiginous diseases, surgery is performed either to eliminate the underlying pathologic event or to create stability in the incoming vestibular signals. It is not always possible to treat the underlying disease. Therefore, surgery is usually performed for the relief of vertigo. There are various surgical approaches used to treat a variety of vertiginous diseases. Selection of the approach depends on the type of vertiginous disease. This review mainly focuses on the current status and outcome of the surgeries used in the treatment of a variety of vertiginous diseases.

Ossicular fixation caused by bone dust after saccus decompression surgery.

HYPOTHESIS: To highlight the clinical and histologic features of ossicular fixation produced by bone dust during mastoid surgery. BACKGROUND: Bone dust deposition in the middle ear may occur during temporal bone surgery, but the ossicular fixation that may result from it remains underreported. METHODS: A case is reported of delayed conductive hearing loss after saccus decompression surgery resulting from ossicular fixation by bone dust deposition. RESULTS: Six months after the initial surgery, the patient experienced a 45 dB conductive hearing loss. The findings on second-look tympanotomy were stapes fixation and round window niche occlusion by newly formed tissue. Histologic examination of the latter showed fibrosis and new bone formation in reaction to particles of bone dust. New bone formation is a frequent concomitant of fibrosis in the middle ear and is probably not a growth from the bone dust. CONCLUSION: The use of high-speed drilling of bone in temporal bone surgery carries the risk of bone dust particle deposition in the middle ear. Measures are discussed that can be used to prevent this complication.

Effects of saccular otolith removal on hearing sensitivity of the sleeper goby (Dormitator latifrons).

It is not known to what extent the entire saccule contributes to overall hearing sensitivity in any fish species. Here we report directional and frequency sensitivity in a teleost fish (Dormitator latifrons) and effects of unilateral and bilateral removal of saccular otoliths on its hearing sensitivity. The fish had different hearing thresholds in the horizontal (-54.4 to -50.3 dB re: 1 micro m) and mid-sagittal (-58.6 to -53.1 dB) planes. At 100 Hz, unilateral otolith removal did not significantly change hearing sensitivity in the mid-sagittal plane, but caused selective reductions of auditory sensitivity by 3-7 dB in the azimuthal axes that are consistent with the longitudinal axis of the damaged saccule. Along the fish's longitudinal axis, unilateral otolith removal significantly decreased auditory sensitivity at 50 Hz and 400 Hz, but not at 100 Hz, 200 Hz, and 345 Hz. At 100 Hz, bilateral otolith removal resulted in robust hearing loss of 27-35 dB at different axes in both horizontal and mid-sagittal planes. Along the fish's longitudinal axis, the bilateral removal reduced auditory sensitivity by 13-27 dB at the different frequencies. Therefore, these results demonstrate that the saccule plays important roles in directional hearing and frequency responses.

Revision surgery after saccotomy for Meniere's disease: does it make sense?

Saccotomy is an established surgical treatment for Meniere's disease that involves exposing and opening the endolymphatic sac and draining the endolymph. However, in patients experiencing recurrent vertigo after saccotomy, it is questionable whether revision saccotomy should be recommended as opposed to more invasive procedures, such as neurectomy of the vestibular nerve. To determine the utility of revision saccotomy, we conducted a retrospective study of 29 patients undergoing this procedure for recurrent vertigo. Patient charts were reviewed for the re-occurrence of any vertiginous symptoms and outcomes with regard to hearing results and post-operative complications. An attempt was made to correlate vertigo status post-revision with surgical findings at the time of revision. After an average follow-up of 20 months, 18 patients (62%) were free of vertigo, and four patients (14%) had a significant decrease in the frequency and intensity of vertiginous attacks. Two patients (7%) relapsed after a vertigo-free period of 18 months, and five patients (17%) experienced no clinical improvement after revision surgery. In patients with a successful sac revision, a common finding at the time of revision was new bone formation in the endolymphatic sac area (n=11), which may have caused blocking of the endolymphatic drainage. Our results demonstrate that revision saccotomy should be considered for recurrent Meniere disease before other, more invasive, surgical options.

[Follow-up assessment of intratympanic gentamicin therapy and Saccotomia in Menière's Disease]. / Retrospektive Untersuchung zur intratympanalen Gentamicin-Behandlung und Saccotomie bei einseitigem Morbus Menière.

BACKGROUND: The intratympanic application of ototoxic antibiotics and saccotomia have been well documented in severe cases of one-sided Menière's disease for 30 years now. Both treatment methods are still subject to controversial discussion. PATIENTS AND MATERIAL: This study includes 8 patients suffering from persistent one-sided Menière's disease after treatment with gentamicin application and additional saccotomia (n=2). We performed follow-up visits including the assessment of hearing ability, vestibular function and cranial MRI, 2-10 years after onset of treatment. RESULTS: After therapeutic interventions 7 patients reported to be healed of their symptom vertigo. In 4 patients the gentamicin-treated vestibular organ did not respond to thermic stimulus at the time of follow-up examination. Auditory threshold shift was observed in 3 patients (1 permanent, 2 temporary), whereas hearing threshold improved in 2 patients. Tinnitus was attenuated in 3 patients, while 1 patient suffered from posttherapeutic onset of tinnitus. Morphological and structural changes due to Menière's disease and treatment procedures were ruled out by cranial MRI. CONCLUSION: The intratympanic application of ototoxic medication is a safe and effective treatment method in severe cases of Menière's disease. Saccotomia should be considered as therapeutic regimen for persistent symptoms after repeated application of gentamicin.

Hipospadias sin secretos / Hypospadias uncovered

El hipospadias es una condición que parece estar aumentando su incidencia en los EE.UU. Después de 200 años de experiencia con cirugía correctora, el urólogo pediátrico moderno dispone de múltiples herramientas dentro de su armamento, que incluyen refinados instrumentos de sutura, magnificación óptica, así como múltiples técnicas quirúrgicas para corregir cualquier deformidad hipospádica con mínima complicación y molestia para el paciente. La cirugía se realiza de forma óptima en los primeros 2 años de vida, para minimizar la carga psicológica y para maximizar las posibilidades de normalidad. Teniendo en cuenta que la primera operación es la que da más posibilidades de conseguir una reaparición normal con éxito, los padres deben ser dirigidos hacia cirujanos competentes experimentados. Este artículo intenta ser una guía para identificar y manejar correctamente el niño con hipospadias; y, además, servir de información respecto a esta condición; su reaparición quirúrgica, y el seguimiento a largo plazo. (AU)

Surgical treatment of vertigo by utriculostomy: an experimental study in sheep.

The objective of this study was to develop and test an endolabyrinthic microsurgical procedure for treatment of vertigo, the utriculostomy. This involves the application of local heat for obtaining a fistula in the membranous labyrinth, so as to establish communication between the endolymphatic and perilymphatic spaces at the utricle level. Before the procedure, an experimental model using quail eggs was built for pre-evaluation, and macroscopic and histological studies were performed in the temporal bones of three healthy sheep. Following this, the utriculostomy was performed through the oval window in 12 sheep. A microthermocautery was conceived by the first author and developed at Hospital de Clinicas de Porto Alegre. This equipment allows for control of temperature and duration of exposure to heat. Three months after the surgery, the animals were killed. A histological study of the temporal bones was performed to assess whether communication had been created between the endolymphatic and perilymphatic spaces, or whether a neomembrane had developed in the cauterized region. Histological sections of the vestibule of eight animals (three normal, five surgical) were analysed. All non-surgical cases presented a normal utricle wall. Three surgical cases (60%) presented a neomembrane. The absence of identifiable perforations in the utricle wall and the presence of neomembrane areas in 60% of the operated bones suggest that utriculostomy is a promising procedure for the treatment of Meniere's disease.

Utriculostomia: apresentaçäo de uma nova técnica para tratamento cirúrgico da vertigem / Utriculusstomy: presentation of a new technique for the treatment of vertigo

Objetivo: apresentar uma nova técnica alternativa para o tratamento cirúrgico de vertigem causada pela Doença de Menière, a utriculostomia, desenvolvida no HCPA...

Treatment of ipsilateral delayed endolymphatic hydrops.

Nine patients with ipsilateral delayed endolymphatic hydrops were treated. All cases were initially treated by conservative therapy using isosorbide or other drugs and 4 patients have been doing well without surgical intervention, though they occasionally have slight dizziness. Five patients required surgery to control vertigo. Three patients first underwent cochleosacculotomy to maintain the vestibular function in the diseased ear, but vertigo recurred after 6 months in all 3 cases. These 3 patients and another patient who wanted definitive treatment right from the start underwent transmastoid labyrinthectomy which resulted in complete control of vertigo. One patient received chorda tympani nerve section because he did not want his vestibule destroyed. After the nerve section, he has had no vertigo though sometimes dizziness. Transmastoid labyrinthectomy seems to be the best treatment for ipsilateral delayed endolymphatic hydrops.