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Objective The study aimed to discuss the association between sensorineural hearing loss (SNHL) and inflammatory bowel disease (IBD). Methods We reviewed cases of patients with known IBD seen in an otolaryngology practice with documentation of all otologic data including age of onset, family history of otologic problems, exposure to noise, audiometric findings, and so on. Results Of 38 patients with a history of IBD, 22 had documented SNHL. Nineteen of these had no other identifiable etiology for their inner ear dysfunction. Fourteen of these patients had a diagnosis of ulcerative colitis and 5 had Crohn disease. Sixteen patients had bilateral SNHL, and 3 patients had unilateral SNHL. Only one patient had a lasting response of SNHL to medical treatment. Conclusion This review suggests that SNHL is an extraintestinal association of IBD. As IBD is considered to be a local or systemic immunopathy, the associated SNHL might also be an expression of systemic immune dysfunction.

Schwannomas are benign peripheral nerve sheath tumors that occur throughout the body. They may present as either solitary or multiple masses. They rarely occur in the nasal cavity. The diagnosis is often made only after histologic examination. Because these lesions are radioresistant, the preferred treatment is complete surgical excision. We present a new case of a schwannoma of the nasal cavity.

Objective Relapsing polychondritis (RP) is an episodic disease most likely of autoimmune etiology, characterized by recurrent inflammation of cartilaginous structures. Methods Retrospective case study at two tertiary referral centers with presentation of nine patients with otologic involvement of RP, review of the spectrum of otologic disorders seen, and treatment. Results The clinical course of otologic manifestations of RP was highly variable and ranged from mild to moderate. In 6/9 patients there was an association with other autoimmune disorders. In addition to recurrent auricular chondritis, which was present in 8/9 patients, our patients had otitis externa, chronic myringitis, Eustachian tube dysfunction, conductive hearing loss, sensorineural hearing loss, and tinnitus. All patients had their diagnosis of RP made on the basis of their otologic involvement and the response to systemic corticosteroids. Conclusion The diagnosis of RP is primarily clinical, but laboratory studies and biopsy may contribute as well. Once the diagnosis is suspected, the otolaryngologist should consider consultation with a rheumatologist to assist in the management of additional systemic manifestations.

Objective To clarify the anatomic characteristics, cause, and surgical outcomes relating to fixation of the stapes footplate in children. Study design Retrospective case review and four-center histopathologic study of temporal bones. Setting Tertiary referral center. Patients We reviewed charts and histologic specimens from 12 children, aged 7 to 13 years, who underwent surgery for footplate fixation. We also studied stapes footplates in 288 temporal bones from 181 children ranging from newborn (20-44 weeks of gestation) to 13 years of age. Main outcome measure Anomalies of the stapes footplate in children. Results The average age of diagnosis of hearing loss was 6.6 years. Criteria for a diagnosis of otosclerosis were progression of a conductive hearing loss and an intraoperative finding of fixation of the anterior stapediovestibular joint in five patients. In contrast, a nonhomogeneous, thickened, fixed footplate and the absence of an annular ligament were indicators of congenital fixation in six children. In one child, there was neither new bone from the otic capsule nor any obvious otosclerotic foci. In the temporal bone study, 17 of 181 (9.4%) children had anomalies of the stapes footplates, with ankylosis in 4 (2.2%). In two subjects (1.1%), there was an otosclerotic focus not in contact with the stapes footplate. Conclusion Children younger than 6 years with various congenital anomalies are more likely to have congenital footplate fixation, which will present intraoperatively as a thickened footplate with a partial or absent annular ligament. Children older than 6 years with progressive conductive hearing loss are more likely to have otosclerosis, which presents as fixation of the anterior stapediovestibular joint. The difference in surgical outcomes is probably related to different degrees of footplate abnormality.

Objectives: The Pierre Robin triad (PRT) consists of micrognathia-retrognathia, glossoptosis, and an oval or cleft palate. The goal of this study was to identify patterns of similarity to and differences from the two previous temporal bone studies of the PRT. Methods: Seven children with the PRT (ages, 45 minutes to 2 years; gestational ages, 41 to 43 weeks) were studied. Thirteen temporal bones were decalcified, sectioned at a thickness of 20 μm, and studied by light microscopy. Results: Our study demonstrated multiple architectural anomalies involving the entire ear, including abnormal auricles, and anomalies of the ossicles, including abnormal stapes footplates (6/13). All children showed signs of middle ear infection (12/13). Anomalies of the inner ear included aplasia of the lateral semicircular canals (5/13), a large vestibular aqueduct (2/13), and unusually large otoconia (1/13). In the mastoid process there were islands of cartilage in the expected position of Reichert's cartilage (9/13) and dehiscence of the fallopian canal (11/13). Loss of cochlear hair cells was seen in children who had antemortem hypoxia. Conclusions: Although the PRT is caused by various genes, most anomalies can be traced to the development of the first and second branchial arches.

To determine the difference in hearing between a group of steelband musicians (pannists) and a control group.We conducted a controlled cross-sectional pilot study consisting of 29 steelpan players and 30 control subjects from a steelpan orchestra (steelband) in the Otolaryngology Department of the Eric Williams Medical Sciences Complex.Significantly more steelpan players had a hearing loss than the control subjects at 3000 Hz, 4000 Hz, and 6000 Hz ( P0.01). chi 2 testing for trend demonstrated that the longer a steelpan player played his instrument the greater the hearing loss.These results indicate that pannists suffer permanent auditory damage from high-intensity sound levels and that preventative measures are required to prevent this hearing loss, particularly in younger players.

An increasing number of patients are seeking nontraditional approaches to treat their health conditions. This article provides an overview of the more frequently used alternative therapies based on a review of the scientific literature. Acupuncture, biofeedback-relaxation, manipulation, physical therapy/massage, herbal substances, and hypnosis are among the most popular forms of alternative therapies.

It is important for us as otolaryngologists to identify our critical role in diagnosing and treating the subset of patients that suffers from headaches. It is equally important for the layperson and the medical community to recognize the importance of a multidisciplinary team approach in the management of headache and facial pain. Despite its limitations, classification provides us with a starting point from which scientists, clinicians, and patients can begin to determine the success of diagnostic and therapeutic strategies. It also indicates future directions for research.

We undertook this study to determine whether or not ante mortem ruptures of the endolymphatic membranes occurred naturally in the infantile labyrinth, what were the most frequent locations and to assess the statistical correlation of an earlier finding of bulging of Reissner’s membrane in pediatric temporal bones. We examined 128 temporal bones from 80 children between the ages of newborn to 12 years with an average age of 15.4 months. All temporal bones were prepared by the celloidin technique and studied by light microscopy. The endolymphatic membranes of the cochlear duct, saccule, utricle and semicircular ducts were examined for ruptures. A rupture of the saccule and utricle was considered to be an ante mortem event if its edges were rolled and bound with fine adhesions and could be identified in adjacent sections. Ante mortem ruptures were seen in 11.1% of the specimens and occurred in 2.4% in Reissner’s membrane, in 6.3% in the saccular membrane and in 2.4% in the utricular membrane. In these specimens, the histopathological changes included atrophy of the stria vascularis and collapse of the organ of Corti, which provided further evidence of an ante mortem event. Infants with extralabyrinthine congenital anomalies or children who were treated with chemotherapy were more likely to develop ruptures of the endolymphatic membranes if they also received aminoglycosides.

Vascular anomalies of the inner ear have been documented in only a few isolated case reports. The goal of our study was to describe, qualify and quantify vascular variations of the inner ear in 122 temporal bones from 64 pediatric subjects aged between 0 and 10 years. The average age was 11.6 months. Horizontal sections of the temporal bone, examined by light microscopy, revealed vessels coursing freely through the perilymphatic space of the cochlea, especially in the apical turn. Other findings included abnormally wide vessels in the stria vascularis as well as a vascular malformation of the internal auditory canal. Our study demonstrated more atypical vessels in the cochlea than in the vestibular labyrinth. We found a statistically significant positive correlation between vascular variations of the inner ear and concomitant cardiac anomalies or endolymphatic hydrops. We also discuss the possible etiology and potential significance of these findings in terms of disturbances of the function of the inner ear.

The aim of this study was to examine the clinical presentation and natural history of chronic myringitis (CM).Retrospective case review.Tertiary referral center.Chronic myringitis is defined as a loss of tympanic membrane epithelium for1 month without disease within the tympanic cavity. Seven hundred fifty patient records were reviewed to determine the prevalence of CM in an academic otology practice. The records of 40 patients (45 ears) with CM seen between 1995 and 1999 inclusive were reviewed.The series was reviewed with attention to previous medical and otologic history, the nature and duration of symptoms, the physical findings, and management.The prevalence of CM was found to be -1% (approximately one fourth as common as cholesteatoma). Symptoms were often present for many years before the diagnosis of CM, with CM often mistaken for chronic otitis media. Sixty percent of patients had undergone previous otologic procedures. There did not appear to be an association between CM and systemic disease. Physical findings were varied, with granulation tissue and tympanic membrane perforations often occurring transiently. The clinical course of CM is typified by recurrent episodes of symptoms, often interspersed with long asymptomatic periods. A subset of CM can result in an acquired atresia. The most effective treatment appeared to be prolonged topical medications, surgery being reserved for only the most refractory cases.Chronic myringitis is often mistaken for chronic otitis media. Such confusion prolongs the initiation of appropriate management and sometimes leads to needless tympanomastoid surgery. The otologist should be aware of this clinical entity and its varied presentation.

The cochlear aqueduct is a bony channel which contains the fibrous periotic duct and connects the perilymphatic space of the basal turn of the cochlea with the subarachnoid space of the posterior cranial cavity. Previous histological studies suggested that patency depended on age, whereas a more recent study showed no statistical correlation between age and patency. To clarify patency in pediatric cochlear aqueducts, we selected 21 temporal bones from 12 infants and children, varying in age from birth to 9 years, in which the cochlear aqueduct was fully visible on one histological section. Photographs were taken for documentation and the length and width of the orifice of the external aperture of the aqueduct at the scala tympani were measured and followed to the internal aperture at the subarachnoid space. The lumen of the duct was examined for mononucleated cells, blood cells and fibrous tissue. Measurements revealed that the mean length of the cochlear aqueduct was 4.6 mm (range, 2.4-10.7 mm), mean width of the external aperture was 484 microm (range, 225-869 microm), and mean width of the internal aperture was 1293 microm (range, 699-2344 microm). The mean diameter of the narrowest part (isthmus) was 151 microm (range, 75-244 microm). In all temporal bones the cochlear aqueduct was patent, with one exception. This latter temporal bone was from a 2-month-old girl with multiple intralabyrinthine anomalies, with the missing cochlear aqueduct believed to be due to an aplasia. Our results support prior measurements of the cochlear aqueduct and demonstrate a short and patent cochlear aqueduct in newborns. With growth, a significant increasing length of the duct was found.

Hypothesis Anomaly in the incudostapedial articulation (IS) may be responsible for persistent conductive hearing loss (HL) in Down's syndrome (DS) patients. Our goal was to perform a detailed histopathologic assessment of the role of the IS joint in the conductive HL. Background In addition to other disabilities, 38% to 78% of patients with Down's syndrome (Trisomy 21) have CHL, some of which are persistent despite maximum treatment. The cause of the persistent conductive HL remains unclear. Methods Retrospective analysis of temporal bone histopathology. Analyses of the midsection through the IS joint of 21 temporal bones (13 subjects) patients with DS and 31 temporal bones (18 subjects) from healthy subjects, performed by 2 blinded authors. The length of 7 different parameters of the width of the IS joint was measured and compared between the 2 groups. Results The IS joint of patients with DS was significantly wider than the normal control. As opposed to the control, it did not decrease because of age. Conclusion The persistent conductive HL in some patients with DS might be caused by anomaly of the IS joint. In severe cases, corrective surgery should be considered.

Pneumoparotitis is a rare cause of enlargement of the parotid gland; it is often misdiagnosed and therefore incorrectly treated. We report three pediatric cases of self-induced pneumoparotitis and detail the clinical presentation, pathogenesis, radiographic findings, and treatment options. We also review the literature on the subject. In children, inflammatory swelling of the parotid gland is usually due to acute viral or bacterial infection, juvenile recurrent parotitis, or allergic, autoimmune, or systemic disease. Infrequently, swelling may result from air being forced through Stensen's duct, resulting in pneumoparotitis. This may occur as a transient or recurrent phenomenon. Recurrent parotid insufflation is not entirely benign and may predispose to sialectasias, recurrent parotitis, and even subcutaneous emphysema. (Arch Otolaryngol Head Neck Surg. 1995;121:1426-1429)

Malformations of the auricle are not uncommon and occur in 1 out of 12500 births. They can occur alone or can be associated with genetically determined syndromes. Most congenital malformations in the human occur during the third to twelfth weeks of embryonic life. During this period, the external ear is undergoing development and can be affected in many ways. There are three parts to the external ear: the auricle, the cartilaginous external auditory canal, and the bony external canal. The auricle (pinna) and the cartilaginous canal are closely related and probably develop from the same anlage. The bony canal is derived from the tympanic ring which is an incomplete cylinder of membranous bone. It must be clearly understood that, although this article is primarily concerned with the morphogenesis and dysmorphogenesis of the auricle, the rest of the external ear, specifically the external auditory canal, is developing simultaneously. Therefore, maldevelopment of the external canal and the auricle will frequently occur together.

Apart from clinical observations of Ménière's disease in children, there have only been a few histological descriptions of endolymphatic hydrops (EH) as an incidental finding in children. In this paper we report on the incidence of EH in temporal bones of children. One hundred eighteen temporal bones from 70 infants between newborn and 10 years (average age 12.9 months) were analyzed by light microscopy for EH of the cochlear duct, which was defined as a bulging of Reissner's membrane into the scala vestibuli in more than one turn of the cochlea. All endolymph spaces were carefully examined for factors such as an obliterated ductus reuniens which may have contributed to the development of EH. In 64 (54.2%) of the 118 temporal bones, a bulging of Reissner's membrane in the cochlear duct was found. In 65.6%, the bulging was confined to the apical turn and since the significance of isolated apical hydrops is controversial, we elected not to consider this group as having hydrops. Twenty (16.9%) bones met our criteria for a diagnosis of EH. Compared to the high incidence of bulging in the cochlear duct, fewer saccules and utricles were dilated. In approximately half of the temporal bones (49.2%), the ductus reuniens was collapsed. The ductus reuniens seems to be closed in its normal position and might open when pressure occurs due to an increasing volume of endolymph. If there is a permanent closure, EH may result. Our investigation does not indicate that EH is associated with a specific disease; however, where there were congenital anomalies the incidence of EH was higher.(ABSTRACT TRUNCATED AT 250 WORDS)

Objective: We investigated the feasibility of arced trifocal distraction osteogenesis for reconstruction of mandibular symphyseal defects. Design: Four adult canine subjects were used. An external apparatus was designed to allow trifocal distraction around an arc. An anterior mandibulectomy was performed between the third premolars bilaterally. The bony defect was regenerated using trifocal distraction osteogenesis at a rate of 1 mm a day bilaterally. New arced bone was produced over a period of 20 to 35 days. The apparatus functioned as an external fixator for the ensuing 6 weeks. The apparatus was then removed, the dogs were returned to their preoperative diet, and were killed after 2 weeks of observation. Main Outcome Measures: Grossly a smooth arc of bone was formed measuring 4.5 to 5.5 cm in length, 2.2 to 3.0 cm in height, and 1.3 to 1.5 cm in width. Histologically all specimens demonstrated direct membranous bone growth. Radiographically opaque columns were seen streaming in the direction of distraction. Conclusions: Symphyseal bony defects resulting from oncologic resection, trauma, and congenital deformities create tremendous cosmetic and functional handicaps and have remained extremely challenging to surgically reconstruct. This study demonstrates the ability to use arced trifocal distraction osteogenesis to reconstruct anterior mandibulectomy defects in a canine model. This method may provide a satisfactory solution to a difficult problem in human mandibular reconstruction. (Arch Otolaryngol Head Neck Surg. 1994;120:911-916)

That acquired cholesteatoma of the human middle ear and mastoid process can be caused by migration of squamous epithelium from the tympanic membrane.To provide histologic evidence in humans of the potential for medial epithelial migration toward the middle ear.The origin of cholesteatomas of the middle ear complex of humans is still not clearly understood. The most popular theory, the medial migration of squamous epithelium from the external auditory canal/tympanic membrane through a perforation of the tympanic membrane to form a cholesteatoma, was first proposed over a century ago independently by Bezold (1899) and Habermann (1899) based on their observations during surgery. Neither author had histologic proof. Despite much experimental efforts in animals, histologic evidence in the human continues to be the missing link.We examined the histologic sections of the temporal bones of 60 children aged 1 day to 12 years with evidence of acute otitis media. We present our findings in the temporal bones of 3 infants.We find evidence of the propensity of the squamous epithelium of the tympanic membrane particularly in the pars flaccida to become very active and to migrate medially toward the middle ear.Our concept is that this material supports the migration theory that cholesteatomas are formed by the medial migration of the stimulated squamous epithelium of the tympanic membrane.

HYPOTHESIS To study the detailed anatomy of the incudostapedial joint (ISJ). BACKGROUND Detailed study of the anatomy of the ISJ has been surprisingly neglected and continues to be controversial. METHODS We studied the joint in 86 temporal bones from 51 subjects, aged 12 to 104 years, by light microscopy and three-dimensional images of computer-based reconstructions. RESULTS In the course of this study, we found that the lenticular process is extremely delicate, consisting of thin trabeculae and spaces filled with areolar tissue. The ISJ is at least partially a bichambered diarthrodial joint divided by a fibrous articular disk into unequal joint spaces. This disk could be identified in all 86 specimens. In addition, we identified for the first time a concavity on the anterior face of the inferior end of the long process of the incus. The asymmetric joint capsule of the ISJ, which completely envelops the lenticular process, extends to this incudal concavity, thereby incorporating part of the long process into the joint complex. We also found that the attachment of the tendon of the stapedius muscle to be more complex than previously described with contributions to the posterior and probably the inferior joint capsule. CONCLUSION On the basis of its finely cohesive anatomic structure, we believe that the incudostapedial articulation should be considered to be a single complex and to be a single entity physiologically.

The authors report a case of asymmetric development of the mastoid antrum in a neonate. The lack of mastoid development most likely represents a congenital maldevelopment. Agenesis of the mastoid antrum has surgical implications, as failure to recognize its absence can result in disorientation during surgery, with potential to injure adjacent structures.

Objective: To analyze and discuss the deafness of Ludwig van Beethoven (1770-1827) and to offer a logical theory for its etiology. Method: The study will carefully review the composer's symptoms as described in his letters to friends and acquaintances and also will review a large body of source material, particularly publications by his contemporaries, some of which were generously loaned by Beethoven-haus, Bonn, Germany, where necessary translations were made directly from the original German. We will also study publications on Inflammatory Bowel Disease (IBD) and its associated extraintestinal manifestations and personal discussions with experienced gastroenterologists. Results: Beethoven's abdominal symptoms that began in his teens are highly suggestive of IBD, which we believe to be a correct diagnosis. IBD is an umbrella term that includes a number of named entities such as ulcerative colitis and Crohn's Disease. IBD is now considered to be a problem of immune regulation with extra intestinal manifestations that include sensorineural hearing loss and primary sclerosing cholangitis (PSC). PSC eventually causes cirrhosis and failure of the liver. A diagnosis of IBD therefore provides a single entity that explains most of the composer's symptoms and was finally the cause of his death. Our conclusion is that Beethoven's sensorineural hearing loss was an immunopathy associated with IBD.

(Editorial Comment: This article highlights the previously unreported relationship between relapsing polychondritis and Crohn’s disease, and parallel improvement of both on treatment. We are reminded of the differential diagnosis of cellulitis of the auricle.) Relapsing polychondritis is a well described rare autoimmune disease of unknown etiology. It is probably underdiagnosed, because in many cases, the disease has a mild or subtle presentation. Fulminant or progressively severe cases leading to death do occur, and a high index of suspicion is necessary when one is confronted with the management of an inflamed ear. In approximately 30% of cases, relapsing polychondritis is associated with another autoimmune disease. In this report, we describe the occurrence of polychondritis in a patient with Crohn’s disease. This association has not been reported before. CASE PRESENTATION

We investigated the vestibular endorgans in three children using 3-D reconstructions from histological sections. The right temporal bone of a newborn child without peripheral vestibular pathology was used as reference model and the temporal bones from a child with Goldenhar syndrome and a child with Pierre Robin sequence with known peripheral vestibular pathology were studied. All five temporal bones were prepared by the celloidin technique and sectioned at 20 microm. Each available section was digitized with a slide scanner. The imaging data were layered anatomically correctly and rendered in a 3-D software. With this technique all vestibular endorgans were reconstructed and measured. The standard deviations in distances ranged between 0.5 and 1.2% and in angles between 0.1 and 2.9 degrees. Both maculae were curved in the longitudinal and transverse axes which described a curve of approximately 35 degrees. The angles between the semicircular ducts varied between 97 and 110 degrees. The pathological models demonstrated a distorted configuration of the semicircular canals and differed substantially from the reference model in most of the measured distances and angles. The method presented is capable of generating 3-D models of the vestibular system from histological sections with an acceptable precision without previously inserted reference marks. Archival celloidin sections are widely available and will be an important resource in understanding the detailed 3-D geometry of the vestibular system which has not yet been accomplished.

Maldevelopment of the first branchial cleft can produce a broad spectrum of anomalies in its derivative structure, the external auditory canal (EAC). Failure of the cleft to develop normally can result in either the absence of a normally patent EAC (atresia, or stenosis) or a duplication anomaly (cyst, sinus, or fistula). Despite their common origins, the coexistence of these anatomical abnormalities is quite unusual. We present four patients with both aural atresia and duplication anomalies of the EAC. Three patients had non-syndromic unilateral aural atresia and presented with periauricular lesions originating from the first branchial cleft. The other patient had a variant of Treacher Collins syndrome and presented with draining infra-auricular fistulae.The classification and management of first branchial cleft anomalies is reviewed in light of these cases. An understanding of the embryogenesis of the external ear is necessary to successfully recognize and treat this spectrum of deformities. A classification system is presented that encompasses the full spectrum of first cleft anomalies.

Objectives Deposits of basophilic material on the cupulae of the semicircular ducts have been described in adult human temporal bones, and such deposits have been implicated in the clinical phenomenon of benign paroxysmal positional vertigo (BPPV). Although relatively rare, BPPV has been reported to occur in children. The goal of this study was to evaluate the occurrence of cupular deposits in pediatric material. Study Design Basic science, two-center histopathologic temporal bone study. Methods We examined 186 temporal bones from 121 individuals between the ages of newborn and 10 years. The average age was 14.7 months. All temporal bones were acquired at autopsy, embedded in celloidin, and sectioned for light microscopic study. The cupulae of the semicircular ducts were identified and any deposits found were graded according to their size and position on the cupula. Results A total of 276 cupulae were identified, and in 35 (12.7%) of these a basophilic deposit was found. In many cases the deposits contained recognizable otoconial crystals. Most deposits were small and they were almost equally distributed between the three semicircular ducts. However, the lateral and posterior cupulae were somewhat more involved than the superior cupula. Our data showed that cupular deposits are less frequently found in the pediatric labyrinth, compared with adults as reported in previous studies. Conclusions The lower occurrence in children versus adults suggests that the deposits may be a phenomenon of the aging vestibular labyrinth.

The temporal bones of a 26-month-old white female with a paralytic syndrome clinically and pathologically identical to poliomyelitis were examined. The aetiological agent was unknown although a non-poliomyelitis enterovirus infection seemed likely. There was a complete absence of the cochlear neurons and substantially reduced peripheral and central axons with loss of some inner hair cells but preservation of outer hair cells. Scarpa’s ganglion, and the geniculate ganglion were partially atrophied. The saccule and utricle were mildely dilated and Reissner’s membrane of the apical turn was bulging. In two previous audiological studies a 10-20 dB bilateral sensorineural hearing loss was found in poliomyelitis patients and a neuronal lesion was postulated which is now supported by our findings. This is a rare example of an almost pure neural hearing loss.

Primary branchiogenic carcinoma was previously a debated entity. Initially it was a popular diagnosis for many cystic carcinomas of the neck. Then, in 1950, stringent criteria were defined for establishing the diagnosis of primary branchiogenic carcinoma. Only a limited number of cases have successfully fulfilled these criteria. We describe a patient who meets the criteria and who we believe represents the first reported case of a carcinoma arising from the vestigium of the first branchial cleft. We also review the literature on branchial cleft carcinomas and discuss the diagnosis, histopathologic features, and therapeutic options.

Study design: The first case of an acquired cytomegalovirus (CMV) infection of the inner ear is reported in a 3-year-old girl in remission from acute lymphocytic leukemia. Methods: Horizontal sections of the temporal bones were studied by light microscopy and immunohistological staining by avidin-biotin-complex-technique was performed on selected archival sections. Three sections were processed for detection of the virus genome by the polymerase chain reaction (PCR). Results: By light microscopy the epithelium of the endolymphatic sac, the utricle and the semicircular canals showed deeply stained acidophilic inclusions and the stria vascularis had a loose structure especially in the intermediate layer. The changes were limited to the non-sensory parts of the labyrinth and no CMV type cells were observed in the organ of Corti. There was a loss of inner and outer hair cells and loss of cochlear ganglion cells caused by either the virus or treatment with gentamicin. Standard immunohistochemistry failed to demonstrate staining with CMV antibodies, but PCR, demonstrated CMV-DNA in one section. Conclusion: Molecular techniques may be able to detect acquired CMV infections in archival pediatric bones temporal bones. The histologic findings in the labyrinth were milder, however showed some similarity to children with congenital CMV labyrinthitis.

The authors report a case of asymmetric development of the mastoid antrum in a neonate. The lack of mastoid development most likely represents a congenital maldevelopment. Agenesis of the mastoid antrum has surgical implications, as failure to recognize its absence can result in disorientation during surgery, with potential to injure adjacent structures.

Homeostasis of the intralabyrinthine fluids is considered to be essential for normal functioning of the inner ear. Disturbances in the intralabyrinthine fluids can be caused by alterations in composition, or changes in volume secondary to resorption or from exchanges between intra- and extralabyrinthine fluid compartments. Carlborg et al demonstrated, in cats, that increased cerebrospinal fluid pressure can be transferred via the cochlear aqueduct to the perilymphatic compartment (Carlborg et al. 1992). Clinically Moss showed, by the tympanic membrane displacement technique, that hydrocephalus can influence hearing in children (Moss et al. 1989). Transmission of fluid and associated pressures between the perilymphatic and subarachnoid spaces demands a patent cochlear aqueduct, but in histologic studies the patency of the cochlear aqueduct in humans is still controversial. In previous studies (Palva, Dammert 1969; Wlodyka 1978) patency was reported to be age-dependent whereas a recent study did not show statistical correlation between age and patency (Merchant, Gopen, 1995), although in most mammals (Bohmer 1993; Carlborg et al. 1992) and in infants the cochlear aqueduct always seems to be open (Palva 1970). In histologic studies the status of the intralabyrinthine fluids is assumed by the position of the endolymphatic membranes. Bulging of Reissner’s membrane is described as endolymphatic hydrops which suggests an excess of endolymph. The opposite situation of retraction (collapse) of Reissner’s membrane and the saccule has been described in adult patients (Fraysse et al. 1988; Fernandez 1958), however its significance is not known. Hypothetically, it seems possible that increased intracranial pressure would produce retraction of the endolymphatic membranes. We therefore conducted this study to answer the following questions: 1. What is the patency of the intralabyrinthine fluid pathways in the temporal bones of children? 2. Are there histopathologic findings that indicate pressure changes in the inner ear from hydrocephalus?

Educational objectives: To grasp the fundamentals of the embryology of the neck and to recognize and understand the clinical presentation of the associated anomalies and formulate a practical approach to the management of anomalies of the neck, including diagnostic maneuvers and surgical techniques.

Between 1980 and 1988, 94 patients with AJCC Stage III and IV squamous cell carcinoma of all sites of the upper aero-digestive tract were treated with radiotherapy. We report here on 62 patients who are followed for a minimum of 2 years. Of these, 30 patients were treated with conventional once-a-day radiotherapy and 32 patients were irradiated using an accelerated superfractionation regimen during part of the treatment course. The altered fractionation schedule employed a concomitant boost technique with clinically demonstrable disease being irradiated twice-a-day during the first or second half of the treatment course. Daily radiation fractions were 1.8 Gy and the boost field was treated with 1.6 Gy after a 4- to 6-hr interval. No significant differences in acute treatment toxicity were observed in the two treatment groups. Patients treated with conventional and accelerated fractionation regimens experienced 36 months actuarial local tumor control rates of 40% and 67% (p = 0.03), respectively, which translated into an actuarial disease-free survival of 40% and 64% (p = 0.04). The increased locoregional control rates in patients treated with accelerated fractionation were associated with an adjusted and overall survival advantage at the p = 0.05 level. We conclude that our regimen of accelerated superfractionated irradiation with shortening of the treatment course resulted in improved control and survival rates at conventional doses of 68.4 to 73.8 Gy.

We have reviewed 50 cases of Wegener's granulomatosis, seen at the New England Medical Center Hospital between 1970 and 1984, and were impressed that 10 (20%) of these patients were under 25 years of age, with ages ranging from 13 to 23 years. Closer examination of this younger group revealed striking differences in their presenting symptoms and organ involvement when compared to the older group of patients. The presentation of these young patients was varied, with no single predominant symptom. Patients presented with otalgia and otitis media or hearing loss, fulminant sinusitis, arthralgias, and even corneal ulcers. Only one patient had "typical" rhinitis and nasal congestion. This group also had a disproportionate number of patients with involvement of the oral cavity, skin, and trachea. Biopsy of these sites frequently demonstrated necrotizing vasculitis. Three of our 50 patients had intracranial involvement, leading to transient hemiplegia in the first, permanent hemiplegia in the second, and a seizure disorder in the third. Two of these patients were in the younger age group. The proportion of patients with limited and generalized Wegener's granulomatosis was the same in both the younger and older age groups. All the younger patients, however, had manifestations of the disease in the head and neck, while four of the older patients had no symptoms in the upper respiratory tract. The number of young patients in our study emphasizes the fact that Wegener's granulomatosis, indeed, occurs in the younger patient and with a greater frequency than previously supposed. This study suggests that in the teenager and young adult, with an unusual constellation of symptoms of the head and neck and accompanying systemic problems, a diagnosis of Wegener's granulomatosis should be seriously considered.

Anomolies of the first branchial groove cleft are common and variable. There has been, however, a tendency in the literature to refer to only one or two types as the first branchial cleft syndrome. A classification of first groove anomalies is offered to clarify this topic. Anomalies can be divided into aplasia, atresia, stenosis, and duplication.

In the squirrel monkey noninflammatory mechanical obstruction of the eustachian tube results in the collection of a clear straw-colored, nonpurulent fluid in the tympanum. At the present time it is presumed that the fluid collection is the direct result of mechanical obstruction of the tube, although this was demonstrated histologically in only two out of 13 animals. The alternative possibility is lymphatic obstruction. It is significant that 93% of the animals developed a clear fluid which has the characteristics of a transudate. In clinical medicine one finds different types of middle ear effusions, all of which are labeled generically as serous otitis media. As it has been possible in this experiment to produce, with a simple technique, the same type of noninflammatory fluid in 93% of our animals, it is believed that this represents the creation of a reliable experimental model which can be used for further investigations into the etiology of the other generic forms of serous otitis media.

Angiosarcomas are comparatively rare neoplasms that present more commonly in the head and neck. Treatment of these tumors has been generally unsatisfactory because of a tendency to local recurrence regardless of the modality of therapy used. A case of anfiosarcoma involving the soft tissue of the premaxilla is presented. This was treated with carefully controlled radiation. The results have been highly satisfactory with preservation of facial tissue and with no evidence of recurrence after 40 months. The relevant literature is discussed.

Four congenitally deaf patients who developed episodic vertigo are presented. In two patients exploratory tympanotomy showed no fistulas. The clinical pattern suggests the development of endolymphatic hydrops in association with congenital deafness.

Two specimens of laryngeal atresia were studied. In the first case stenosis was hard due to noncanalization of the anterior cricoid cartilage. In the second case there was a soft stenosis from limited canalization of the endoluminal soft tissue. Additionally, the first case had a posterior cleft with a persistent pharyngotracheal duct. The stenotic soft-tissue elements in both cases contained striated muscle fibers that interdigitated across the midline. The mucosa on the inferior surface of the hard stenosis was grossly thickened and contained large mucous glands. The embryologic significance of these anomalies is discussed. Presumably these cases represent anomalies caused by different enbryologic events.