Faculty Bibliography

HYPOTHESIS: Real-time intraoperative fluoroscopy is a useful adjunct to cochlear implantation in selected cases. The advantages include the avoidance of complications such as extracochlear array placement, intrameatal array insertion, and avoidance of significant bending or kinking. This is particularly useful when implanting a severely abnormal cochlea. BACKGROUND: The technique was initially developed for laboratory study of electrode prototypes in cadaver temporal bones to evaluate insertion dynamics and mechanisms of intracochlear trauma. The technique was subsequently adapted for use in live surgeries. METHODS: Live surgeries were performed using fluoroscopic guidance on nine patients. RESULTS: Five patients were implanted with the Nucleus 24 RCS during preclinical trials. Two patients with severe cochlear malformations were implanted with a Nucleus CI24M straight array. Two patients with severe cochlear ossification were implanted with the Nucleus CI24 double array. Appropriate insertions were achieved without electrode damage in all cases. CONCLUSIONS: Intraoperative fluoroscopy is a useful adjunct to cochlear implantation, which can be performed with minimum risk to the patient and operating room staff if the outlined precautions are taken. Intraoperative fluoroscopy is indicated in cases where the intracochlear behavior of the electrode array cannot be predicted, a condition encountered when implanting new electrode designs, cases with severely malformed inner ears, or cases of severe intraluminal obstruction requiring a double-array insertion

BACKGROUND: Increasingly, deaf students are educated in mainstream school environments. This poses a question of how deaf children negotiate the demands of forming friendships with their hearing peers. The present study was designed to examine coping strategies of 35 deaf children attending full or partial mainstream school settings. METHOD: Using a qualitative analysis design, a range of coping strategies used by the children was described based on parents' reports. The relative success of these coping strategies in establishing relationships with hearing peers was assessed by examining strategies that increased the social success of deaf girls and boys. RESULTS: The results showed differences by gender in the effectiveness of coping strategies used by the participants. Deaf girls who were confident and frequently asked for clarifications or who were comfortable playing alone tended to achieve good relationships with hearing peers. In contrast, deaf boys' relationships with hearing peers benefited mostly from the boys' ability to perform well in sports. CONCLUSIONS: These findings are discussed in light of existing research on coping and current debates on the benefits of mainstreaming for deaf children

OBJECTIVE: Anomalies of the fourth branchial arch complex are exceedingly rare, with approximately forty cases reported in the literature since 1972. The authors report experience with six fourth arch anomalies. METHODS: Retrospective chart review of six consecutive patients presenting to the pediatric otolaryngology service at a tertiary care center with anomalies referable to the fourth branchial arch. RESULTS: All six patients presented within the first or second decade of life. All six had left-sided disease. Four patients presented with recurrent neck infection, one with asymptomatic cervical masses, and one with a neck mass and respiratory compromise. One patient had prior surgery presented with a recurrence. Diagnosis of fourth arch anomalies was suggested or confirmed by computed tomography and flexible laryngoscopy. Treatment was surgical in five patients; one patient is awaiting surgery. Surgical procedures included resection of the mass and endoscopic cauterization of the inner opening of the cyst. CONCLUSIONS: The presentation of a cervical mass, especially with recurrent infections and especially on the left side, in a child in the first or second decade of life heightens suspicion for an anomaly of the fourth branchial arch. Diagnosis can be difficult, but is aided by the use of flexible laryngoscopy, Computed tomography (CT) scanning and ultrasonography. Surgical resection of the cyst and cauterization of its pyriform sinus opening should be undertaken to minimize recurrence

The nasolabial flap is a straightforward and time-tested reconstructive option suitable for a variety of facial and oral defects. The superiorly and inferiorly based variants are well described in the literature and offer a rapid and reliable alternative to time-consuming microvascular free flaps and less esthetic skin grafts. Despite newer and more complex alternatives, the nasolabial flap maintains its prominent position in the reconstructive armamentarium of the oral and maxillofacial surgeon

OBJECTIVES/HYPOTHESIS: Vascular malformations involving the mandible and maxilla are uncommon, and no uniform treatment of these lesions has been defined. The purpose of the study was to evaluate the effectiveness of treating vascular malformations with a multidisciplinary approach and emphasis on endovascular therapy. STUDY DESIGN: Retrospective chart review of patients. METHODS: The treatment of 31 patients (13 male and 18 female patients) with mandibular and/or maxillary vascular malformations presenting between 1979 to 2001 was reviewed. RESULTS: Thirteen patients (42%) presented with mandibular vascular malformations, and an equal number of patients had maxillary vascular malformations. Five patients had involvement of both the mandible and maxilla. Twenty-six patients (84%) had adjacent soft tissue extension, whereas five patients had a vascular malformation isolated either to the mandible (four cases) or maxilla (one case). Twenty-six cases consisted of arterial vascular malformations, and five patients had venous and capillary types. Twenty-five patients (81%) were treated with embolization only, whereas six patients (19%) underwent combined embolization and surgical resection. "Cure" was defined as the complete eradication of disease or permanent resolution of symptoms with complete devascularization by embolization. The cure rates were 70% for mandibular malformations and 46% for maxillary lesions. None of the combined maxillary/mandibular lesions were cured, but all achieved improvement or stabilization of symptoms. The follow-up range was 1 to 22 years with an average follow-up of 6.7 years. CONCLUSION: The location and extent of vascular malformations dictate the treatment and resulting success. Endovascular therapy alone can effectively "cure" most mandibular and maxillary vascular malformations with limited soft tissue involvement. Extensive vascular malformations can be stabilized with control of symptoms, but eradication of the vascular malformation is unlikely even with combined surgery and embolization.

A group of central auditory neurons residing in the lateral superior olivary nucleus (LSO) responds selectively to interaural level differences and may contribute to sound localization. In this simple circuit, ipsilateral sound increases firing of LSO neurons, whereas contralateral sound inhibits the firing rate via activation of the medial nucleus of the trapezoid body (MNTB). During development, individual MNTB fibers arborize within the LSO, but they undergo a restriction of their boutons that ultimately leads to mature topography. A critical issue is whether a distinct form of inhibitory synaptic plasticity contributes to MNTB synapse elimination within LSO. Whole-cell recording from LSO neurons in brain slices from developing gerbils show robust long-term depression (LTD) of the MNTB-evoked IPSP/Cs when the MNTB was activated at a low frequency (1 Hz). These inhibitory synapses also display mixed GABA/glycinergic transmission during development, as assessed physiologically and immunohistochemically (Kotak et al. 1998). While either glycine or GABA(A) receptors could independently display inhibitory LTD, focal delivery of GABA, but not glycine, at the postsynaptic-locus induces depression. Furthermore, the GABA(B) receptor antagonist, SCH-50911, prevents GABA or synaptically induced depression. Preliminary evidence also indicated strengthening of inhibitory transmission (LTP) by a distinct pattern of inhibitory activity. These data support the idea that GABA is crucial for the expression inhibitory LTD and that this plasticity may underlie the early refinement of inhibitory synaptic connections in the LSO

Botulinum toxin (Botox) has firmly established itself as one of the premier nonsurgical therapies. Recently, Botox is also being used as an adjuvant to many aesthetic procedures, both surgical and nonsurgical. This combined therapeutic use for Botox may not only create an added benefit toward facial rejuvenation but may create a synergistic one as well. This article examines such uses for Botox and reviews the current medical literature.

Massive sinonasal polyposis associated with skull base dehiscence and intracranial extension is a difficult disease to treat. Conventional transnasal or transfacial techniques can result in dural injury, cerebrospinal fluid (CSF) leak and infection. We describe our experience with a combined neurosurgical-endoscopic technique that protects the meninges. Five patients with massive sinonasal polyposis extending intracranially through skull base dehiscence were reviewed retrospectively. The minimum follow-up was 2 years. A frontal craniotomy was performed through a bicoronal approach. The dura was carefully separated from all infectious material at the floor of the anterior cranial fossa. The frontal lobe with the intact meninges was elevated off the anterior cranial floor. A sheet of blue plastic material was inserted under the frontal lobe from the craniotomy site to the planum sphenoidale. Next, a transnasal endoscopic ethmoidectomy, sphenoidectomy, and frontal sinusotomy were performed to remove the inflammatory processes. The blue plastic material was visible through any existing or potential skull base dehiscence, thus providing visual protection for the dura and brain. All gross disease was removed from the frontal, ethmoid, and sphenoid skull base regions in the 5 patients without dural injury. None of the patients developed a CSF leak or meningitis. Two patients developed recurrent polyposis limited to the sinuses without intracranial extension. The endoscopic craniofacial approach with the 'Blue-Sky' protective technique offers a safe method for completely removing massive sinonasal polyposis associated with an intracranial extension

ACF7 is a member of the spectraplakin family of cytoskeletal crosslinking proteins possessing actin and microtubule binding domains. Here, we show that ACF7 is an essential integrator of MT-actin dynamics. In endodermal cells, ACF7 binds along microtubules but concentrates at their distal ends and at cell borders when polarized. In ACF7's absence, microtubules still bind EB1 and CLIP170, but they no longer grow along polarized actin bundles, nor do they pause and tether to actin-rich cortical sites. The consequences are less stable, long microtubules with skewed cytoplasmic trajectories and altered dynamic instability. In response to wounding, ACF7 null cultures activate polarizing signals, but fail to maintain them and coordinate migration. Rescue of these defects requires ACF7's actin and microtubule binding domains. Thus, spectraplakins are important for controlling microtubule dynamics and reinforcing links between microtubules and polarized F-actin, so that cellular polarization and coordinated cell movements can be sustained.