Electrophysiologic effects of placing cochlear implant electrodes in a perimodiolar position in young children.

OBJECTIVE: The purpose of this study was to intraoperatively record the electrically evoked auditory brainstem response (EABR) before and after placement of the electrode positioning system (EPS) (CII Bionic Ear with HiFocus I cochlear implant electrode array) as well as before and after stylet removal (Nucleus Contour cochlear implant electrode array). It was hypothesized that physiologic changes would occur after perimodiolar positioning of the electrode array and these changes would be evident from the EABR recordings. STUDY DESIGN: Consecutive young (11-36 month old) pediatric cochlear implant recipients (n = 17) had intraoperative EABRs recorded from three intracochlear electrodes that represented apical, medial, and basal locations. Wave V amplitudes and thresholds were studied relative to electrode location and pre- versus postperimodiolar positioning. These evoked potential measures were analyzed for statistical significance. SETTING: Tertiary referral children's hospital/medical college. RESULTS: Wave V thresholds of the EABR were lower, and amplitudes were larger after perimodiolar positioning, although the changes were dependent on electrode location and implant design. Statistically significant decreases in EABR wave V threshold and increases in suprathreshold wave V amplitude were found for the basal electrode for the CII Bionic Ear HiFocus I and for the apical electrode for the Nucleus Contour. CONCLUSIONS: Placement of either the CII Bionic Ear HiFocus I or Nucleus Contour cochlear implant electrode array in the perimodiolar position in young children resulted in less electrical current necessary to stimulate the auditory system. Changes in electrophysiologic thresholds and amplitudes, measured with EABR, indicate that the electrode array is placed closer to the modiolus with both electrode designs.

Meningitis due to Enterobacter aerogenes subsequent to resection of acoustic neuroma and percutaneous endoscopic gastrostomy tube placement: a rare nosocomial event.

We present a case of meningitis after percutaneous endoscopic gastrostomy (PEG) tube placement subsequent to acoustic neuroma resection and cranioplasty. Four days following PEG tube placement, the patient developed Enterobacter aerogenes meningitis, requiring explantation of infected cranioplasty material. His condition subsequently improved. Etiology and future intervention strategies are discussed.

Progressive late-onset sensorineural hearing loss and vestibular impairment with vertigo (DFNA9/COCH): longitudinal analyses in a belgian family.

OBJECTIVE: To evaluate audiometric and vestibular signs and symptoms in a new DFNA9 family. SETTING: Tertiary referral centers. METHODS: A multigeneration Belgian family with late-onset progressive sensorineural hearing loss and concomitant ves-tibular impairment with an autosomal dominant pattern of inheritance underwent clinical and genetic evaluation. Medical history was recorded. Blood samples were taken for genetic linkage and mutation analyses. Pure-tone audiometry, speech audiometry and vestibular examinations were performed. Onset and progression in hearing impairment were evaluated with linear regression analysis of longitudinal threshold-on-age data. RESULTS: Linkage to DFNA9 was confirmed and mutation analysis revealed a P51S mutation in the COCH gene. Several patients had a Ménière's-like presentation. All patients developed late-onset progressive sensorineural hearing loss eventually leading to severe deafness and vestibular failure.

External auditory canal eccrine spiradenocarcinoma: a case report and review of literature.

BACKGROUND: Eccrine spiradenocarcinoma is a rare dermal appendage carcinoma believed to arise from transformation of a long-standing benign spiradenoma. This tumor demonstrates highly malignant biologic behavior with a high recurrence rate, frequent lymph node metastases, and overall poor survival. METHODS: We report the first case of eccrine spiradenocarcinoma arising in the external auditory canal. The management of this tumor, its histopathologic characteristics, and a review of literature are presented. RESULTS: A literature review identified 17 cases of eccrine spiradenocarcinoma in the head and neck region. Local recurrence occurred in 58.8% of patients, with an average of 23 months from diagnosis. Lymph node metastasis occurred in 35.3%, with an average of 31 months from diagnosis. Other metastatic sites included skin, bone, and lung. Disease-specific mortality was 22.2%. CONCLUSIONS: Eccrine spiradenocarcinoma is an aggressive tumor with a poor prognosis. Primary treatment should include wide local excision with or without regional lymphadenectomy. Isolated successful treatments have been documented with adjuvant hormonal manipulation, chemotherapy, and radiation therapy.

Electrically evoked auditory brain stem responses for lateral and medial placement of the Clarion HiFocus electrode.

OBJECTIVE: The purpose of this study was to compare the electrically evoked auditory brain stem response (EABR) for lateral and medial placement of the Clarion HiFocus cochlear implant electrode array via the electrode positioning system (EPS). DESIGN: Twenty-five adult and pediatric cochlear implant recipients participated in the study. Intraoperatively recorded EABRs were evoked by stimuli via three intracochlear electrodes representing apical, medial, and basal locations, and responses were elicited before and after positioner insertion. Evoked potential measures of wave V amplitude and threshold were examined for statistical significance using ANOVA for repeated measures and Chi-Square methods. RESULTS: For a given supra-threshold stimulus level, the increase in EABR wave V amplitude was significantly larger after EPS placement compared to before EPS placement for electrodes 1 (apical) and 13 (basal). Likewise, when the stimulus was decreased to obtain a minimal amplitude, the wave V threshold was significantly lower after EPS placement for electrodes 7 (medial) and 13. The number of measurements that showed decreased wave V threshold after EPS insertion was significantly dependent on intracochlear electrode location. CONCLUSIONS: Placement of the Clarion Electrode Positioning System following HiFocus electrode insertion resulted in a reduction in the electrical current required to activate the auditory system. The effect of the EPS was greatest for the basal location, demonstrated by lower wave V thresholds and a larger percentage increase in wave V amplitude. The EABR reflected electrophysiologic changes relative to lateral-to-medial changes in intracochlear electrode position due to the EPS.

Gene discovery using a human vestibular schwannoma cDNA library constructed from a patient with neurofibromatosis type 2 (NF2).

BACKGROUND: Despite a strong association of schwannomin/merlin gene mutations with vestibular schwannoma formation, the regulatory mechanisms and biologic pathways involved are still largely unknown. The hypothesis of this study is that the genesis and growth characteristics of neurofibromatosis type 2 (NF2)-associated vestibular schwannomas are determined by genetic alterations that vary in gene transcript expression; this transcript expression includes oncogenic gene products that may be identified by construction and sequencing of a cDNA library from NF2-associated vestibular schwannoma. METHODS: Approximately 3 mL of fresh tumor was obtained during resection of a 4-cm vestibular schwannoma from a patient with NF2. Poly(A)(+) mRNA was isolated, synthesized into double-stranded cDNA, and unidirectionally inserted into Uni-Zap XR (Stratagene, La Jolla, CA) bacteriophage vectors. Bacteriophage vectors containing cDNA inserts were processed into phagemids according to Uni-Zap XR protocol, and inserted vectors were sequenced and analyzed using BLAST software (National Institutes of Health, Bethesda, MD) with GenBank, EMBL, DDBJ, and PBD databases. RESULTS: The cDNA library contained 2.4 million primary plaques. Inserts averaged 1.8 kilobases (kb) in length, with a range of 0.8 to 3.0 kb. BLAST multidatabase comparison of the sequence data obtained from 50 randomly selected clones yielded identification of 13 sequences representing known human genes and 17 sequences representing cloned sequences with unknown function. Three clones represented sequences not previously described in vestibular schwannomas but strongly implicated in oncogenesis within other tissues. CONCLUSIONS: These data have implications for understanding the molecular mechanisms of vestibular schwannoma tumor biology. Identified genes may provide future diagnostic/prognostic markers and therapeutic targets.

Expression of G-protein alpha subunit genes in the vestibular periphery of Rattus norvegicus and their chromosomal mapping.

OBJECTIVE: Heterotrimeric G-proteins play an important role in mediating signals transduced across the cell membrane by membrane-bound receptors. The precise role of G-proteins and their coupled receptors in the physiology of the vestibular neuroepithelium is not well understood. The purpose of this study was to better define the role of these proteins by examining their expression in the rat vestibular periphery and characterizing their chromosomal location. MATERIAL AND METHODS: To characterize G-protein alpha subunit gene expression in the target tissue of interest, we performed polymerase chain reaction (PCR) using degenerate G-protein primers corresponding to conserved regions in the G-protein alpha subunit coding sequence on a normalized rat vestibular cDNA library. PCR amplicons were cloned and 50 clones were randomly selected and sequenced. Radiation hybrid (RH) mapping was used to determine the chromosomal location of G alpha(olf) and two previously identified G-protein alpha subunits--G alpha(i2) and G alpha(i2(vest))--in the rat genome. RESULTS: The following G-protein alpha subunits were identified in the normalized cDNA library: G alpha(olf), G alpha(s), G alpha(o) and G alpha(s2). G alpha(olf) maps to chromosome 18 between markers D18Mit17b and D18Mgh2. G alpha(i2) maps to chromosome 8 between markers D8Rat65 and D8Mgh2. G alpha(i2(vest)) maps to chromosome 1 between markers D1Rat132 and D1Rat202. These chromosomal locations in the rat genome are syntenic to chromosomal regions in which the homologous G-protein alpha subunit genes have been localized in the human and mouse genomes, further validating RH mapping as an effective and accurate tool. We were unable to RH map the location of G alpha(o) due to its extensive homology with the hamster gene. CONCLUSION: The characterization of G-protein alpha subunit gene expression in the vestibular periphery and the chromosomal localization of these genes in the rat revealed that a diverse group of these second messengers are expressed.

Radiation hybrid mapping of five muscarinic acetylcholine receptor subtype genes in Rattus norvegicus.

Acetylcholine is the main neurotransmitter of the vestibular efferent system and a wide variety of muscarinic and nicotinic acetylcholine receptors are expressed in the vestibular periphery. The role of these receptors and in particular the role of muscarinic acetylcholine receptors in the physiology of the vestibular neuroepithelium is not understood. Congenic and consomic rats are a convenient way to investigate the involvement of candidate genes in the manifestation of defined traits. To use congenic or consomic rats to elucidate the roles of these receptors in vestibular physiology or pathology the chromosomal location of the genes encoding these receptors has to be determined. Using radiation hybrid (RH) mapping and a rat RH map server (www.rgd.mcw.edu/RHMAP SERVER/), we determined the chromosomal locations of the muscarinic acetylcholine receptor genes in the rat (Rattus norvegicus). The m1-m5 muscarinic subtypes mapped to the following chromosomes: Chrm1, chromosome 1; Chrm2, chromosome 4; Chrm3, chromosome 17; Chrm4, chromosome 3; and Chrm5, chromosome 3. With the chromosomal location for each of these muscarinic subtypes known, it is now possible to develop congenic and consomic strains of rats that can be used to study the functions of each of these subtypes.

Endoscopy in neuro-otologic surgery.

Endoscopy offers several distinct advantages over the operating microscope during neuro-otologic surgery that make it an excellent adjunctive tool to the microscope or independent modality during cranial base surgery. The high magnification gives excellent definition of perforating blood vessels, cranial nerves, and neural structures, which in many cases is superior to that achieved with the microscope. Furthermore, the use of angled or flexible endoscopes allows one to look around corners and behind anatomic structures blocking the view seen via a 0 degree microscope. Endoscopy also has the theoretical advantage that a less invasive operative procedure is required, which should reduce the operative morbidity. Several notable disadvantages of endoscopy include the problems associated with blood soiling the endoscope, making visualization difficult or impossible, the lack of readily available instrumentation designed specifically for endoscopic neuro-otology, and the poor overview of the operative field. This last point is an important one because the endoscope is placed adjacent to the lesion and does not allow one to look backward to prevent [figure: see text] injury to structures next to the shaft of the telescope. Furthermore, the surgeon must be cognizant of potential thermal injury to structures caused by the heat generated by the light source. The present endoscopic technology limits the image that the surgeon sees to two dimensions, which results in certain unique problems when operating in a three-dimensional milieu. Because of this, there is a steep learning curve to acquire endoscopic dexterity and three-dimensional orientation. Finally, bimanual operation requires the use of an articulated endoscope holder or the commitment of the co-surgeon to hold the endoscope. One of the limitations of the operative microscope is that the angle of view is determined by the distance of the lens to the skull, retractor, or obstructing tissue, which is a function of the lens focal length; the longer the focal length, the narrower the viewing angle. During most microsurgical procedures, the focal distance varies between 200 and 400 mm. Using a previous analogy, if one looks through a door's keyhole at close range, nearly the entire room on the opposite side of the door can be seen, although nothing can be seen when the hole is viewed from a long distance. This is similar to what happens when using the endoscope with focal lengths ranging from 5 to 20 mm: a wider angle of view can be achieved. Based on their, experience the authors believe that endoscopes can be used safely during neuro-otologic surgery. As an adjunct to or substitution for the operative microscope, this modality does improve visualization of bony, neural, and vascular structures while minimizing cerebellar retraction.

Biophysics of cochlear implant/MRI interactions emphasizing bone biomechanical properties.

OBJECTIVE/HYPOTHESIS: The forces exerted during a 1.5-Tesla MRI evaluation on the internal magnet of a cochlear implant (CI) raise concern about the safety for CI recipients. This study determines the magnitude of force required to fracture the floor of a CI receiver bed. METHOD: Recessed CI beds were drilled to maximum uniform thinness into formalin-fixed and fresh-frozen human calvaria specimens. A Med-El stainless steel CI template mounted to the piston of an electrohydraulic testing device was used to fracture the floor of the implant beds. Force and displacement were measured as a function of time using a digital data acquisition system. RESULTS: Mean force to first failure, displacement to first failure, and minimum thickness, respectively, were: group 1 (formalin-fixed, 0.3-0.4-mm thick [n = 22]), 34.08 N (8.21-59.64 N, standard deviation [SD] 15.41 N), 1.09 mm (0.40-2.16 mm, SD 0.51 mm), 0.36 mm (0.3-0.4 mm, SD 0.05 mm); group 2 (formalin-fixed, 0.5-0.9 mm thick [n = 21]), 52.82 N (20.28-135.53 N, SD 25.29 N), 1.08 mm (0.50-2.28 mm, SD 0.47 mm), 0.58 mm (0.5-0.9 mm, SD 0.12 mm); group 3 (fresh-frozen [n = 9]), 134.13 N (86.44-190.70 N, SD 34.92 N), 1.96 mm (1.47-2.46 mm, SD 0.35 mm), 0.42 mm (0.3-0.6 mm, SD 0.11 mm). CONCLUSIONS: The mean magnitude of force required to fracture the floor of a CI bed is significantly greater than those that are generated when a Med-El Combi 40+, CII Bionic Ear CI, or Nucleus Contour CI is placed into a 1.5-Tesla MRI unit.

Calcitonin gene-related Peptide and choline acetyltransferase colocalization in the human vestibular periphery.

Within the vestibular system, calcitonin gene-related peptide (CGRP) has been localized in the efferent terminals and their brainstem neuronal cell bodies in several animal models. Presently, very few studies have verified these findings in the vestibular system in adult primates or humans. CGRP immunoreactivity (CGRPi) and its colocalization with choline acetyltransferase immunoreactivity (ChATi) in human vestibular end organs and Scarpa's ganglion were studied using polyclonal antibodies against CGRP and ChAT, at the light-microscopic level. The CGRPi axons ramified to produce numerous CGRPi terminals throughout the neurosensory epithelium of the maculae and cristae, primarily in the basal and midbasal areas. Numerous CGRPi efferent terminals made contact with both type II vestibular hair cells and the afferent chalices surrounding type I vestibular hair cells. All CGRP immunoreactive fibers also exhibited ChATi. As in the animal models, no CGRPi was found within Scarpa's ganglion. This study provides evidence for CGRPi in the human vestibular periphery and validates the biomedical relevance of the current animal models.

Granular cell tumor of the pituitary fossa.

Although granular cell tumors are relatively common in the head and neck, symptomatic granular cell tumors of the neurohypophysis are extremely rare. Ophthalmologic symptoms are most common, followed by endocrinologic manifestations. We report a case of a granular cell tumor of the pituitary fossa that was surgically treated. The clinical manifestations, radiographic appearance, and surgical management of granular cell tumors of the pituitary fossa are reviewed, as well as the unique histopathology and electron microscopy of this uncommon neoplasm.

The ascending pharyngeal artery: branches, anastomoses, and clinical significance.

Neuroradiologists generally do not fully appreciate the importance of the territory of the ascending pharyngeal artery. The ascending pharyngeal artery is a small but important artery that supplies multiple cranial nerves and anastomotic channels to the anterior and posterior cerebral circulations. Several disease processes in the head and neck involve the ascending pharyngeal artery. To evaluate and treat such diseases, it is necessary for neuroradiologists not only to know selective angiography and embolization techniques, but also the territory of the ascending pharyngeal artery, anastomoses, and vascular supply to the vasa nervorum of lower cranial nerves. Herein, the normal angiographic anatomy of the ascending pharyngeal artery, its relationship with neighboring territories, its importance in clinical situations, and research models are reviewed.

A novel connexin 26 compound heterozygous mutation results in deafness.

OBJECTIVE: Mutations of the gap junction beta 2 (GJB2) gene coding for the protein connexin 26 account for up to 50% of nonsyndromic sensorineural hearing loss (NSHL), with specific mutations associated with distinct ethnic groups. A biracial family with nonsyndromic sensorineural deafness consistent with autosomal recessive inheritance was examined for connexin 26 (Cx26) mutations. STUDY DESIGN: Prospective observational study. METHODS: A family consisting of a Caucasian mother and a Chinese father with two of six children affected by NSHL was examined for Cx26 mutations. Peripheral blood lymphocyte DNA was used to amplify by polymerase chain reaction the Cx26 coding region, followed by mutation detection enhancement gel screening and complete sequencing. Phenotypic characterization using audiometric testing was completed for all children and both parents. RESULTS: The two affected children were found to be compound heterozygotes for Cx26 mutations, displaying a previously unreported combination of 35delG and 235delC. The parents were each unaffected heterozygotes consistent with their ethnic heritage, specifically, the Caucasian mother a 35delG heterozygote and the Chinese father a 235delC heterozygote. CONCLUSIONS: Connexin 26 mutations account for a significant proportion of NSHL worldwide, with specific mutations linked to distinct ethnic groups. Genetic analysis of a biracial family with NSHL revealed a novel 35delG/235delC compound heterozygous state in phenotypically affected children. These results highlight the usefulness of Cx26 mutation screening for genetic counseling and suggest that the 235delC mutation is present in China as it is in Japan and Korea.