Mid-frequency sensorineural hearing loss: aetiology and prognosis.

OBJECTIVES: An audiometric finding of mid-frequency sensorineural hearing loss (MFSNHL), or a U-shaped pattern, is uncommon. The objective of this study is to investigate the aetiology and prognostic significance of MFSNHL. DESIGN: Tertiary academic referral centre-based retrospective case review and review of audiograms to determine the prevalence of this audiometric finding. METHODS: Patients with a pure tone threshold average at 1, 2, and 4 kHz at least 10 dB greater than the average at 0.5 and 8 kHz were included in this study; 35 patients met these criteria. The mean age of the patients was 34.6 years old (range 4-71 years). Twelve patients (33 per cent) were under 18 years of age. Serial audiograms were obtained for 14 patients. The notes were reviewed for any pertinent otologic history, subsequent diagnoses, management and disease course. RESULTS: The prevalence of MFSNHL in this practice setting is less than 1 per cent. The average hearing threshold in the mid-frequencies was 44 dB, which was 17 dB and 20 dB lower than at 0.5 Hz and 8 kHz, respectively. The pure tone average (0.5, 1, 2 kHz) was 40 dB. Sixteen patients (44 per cent) required amplification. Of all patients, 22 had hereditary hearing loss, eight had idiopathic hearing loss, and five adults had vestibular schwannomas. CONCLUSIONS: MFSNHL is an infrequent audiometric finding. The great majority of these cases are of presumed hereditary or idiopathic aetiology, although 22 per cent of adults had vestibular schwannomas. This series presents the causes and prognosis of this audiometric pattern.

Accelerated superfractionated irradiation for advanced carcinoma of the head and neck: concomitant boost technique.

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.

Viral labyrinthitis: early pathology in the human.

The histologic findings in the temporal bones of three patients who died from viral encephalopathy are presented. Pathology was restricted to the scala media, vestibular labyrinth, and internal auditory canal and was considered to be expressions of viral labyrinthitis. The changes were different degrees of degeneration of the organ of Corti, early encapsulation of the tectorial membrane, degeneration of the stria vascularis, and round cell infiltration of the modiolus and contents of the internal auditory canal. A new finding in the organ of Corti and early stages of cystic degeneration of the stria vascularis are documented. In all cases, the saccule was degenerated with sloughing of the otolithic membrane and vestibular labyrinth was involved in varying degrees.

Letterer-Siwe disease. Presentation as an otologic problem.

Letterer-Siwe disease is one of the three clinical syndromes that are collectively known as histiocytosis X. This is the most aggressive form of histiocytosis and is frequently fatal. Recently, however, early treatment with chemotherapeutic agents and steroids has prolonged survival and has even resulted in remission of a number of cases. This paper draws attention to a persistent external otitis as an early symptom of Letterer-Siwe disease and identifies, histologically, involvement of the external auditory canal.

Sclerosing lesions of the temporal bone.

Sclerosis of the mastoid portion of the temporal bone is often seen on radiographs of patients who have or have had chronic otitis media. Occasionally lesions are seen that cause sclerosis of the entire temporal bone. The otolaryngologist must be alert to the fact that these findings may signify important pathology beyond that of chronic otitis media and indeed may be indicative of life threatening problems in adjacent or distant areas. In this paper the differential diagnoses of sclerotic lesions of the temporal bone will be discussed. These include fibrous dysplasia, osteopetrosis, meningioma, ossifying fibroma and metastatic lesions to the temporal bone. Three representative cases with sclerotic temporal bones are presented. The first is a patient with a conductive hearing loss secondary to stenosis of the external auditory canal. He was found to have a fibrous dysplasia of the temporal bone. The second case is an elderly male with a draining ear and a facial nerve paresis. He was found to have adenocarcinoma of the prostate gland metastatic to the temporal bone. The third case is that of a woman with a meningioma who demonstrated a sclerotic temporal bone on routine radiographs of the skull.

The first branchial cleft carcinoma.

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.

Distraction osteogenesis for reconstruction of mandibular symphyseal defects.

OBJECTIVE: We investigated the feasibility of arched 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.

Self-induced pneumoparotitis.

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.

Chronic myringitis: prevalence, presentation, and natural history.

OBJECTIVE: The aim of this study was to examine the clinical presentation and natural history of chronic myringitis (CM). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Chronic myringitis is defined as a loss of tympanic membrane epithelium for >1 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. MAIN OUTCOME MEASURES: The series was reviewed with attention to previous medical and otologic history, the nature and duration of symptoms, the physical findings, and management. RESULTS: 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. CONCLUSIONS: 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.

Waardenburg's syndrome: variations in expressivity.

Twenty-seven subjects with a family history of Waardenburg's syndrome were examined with respect to 18 specific characteristics of the syndrome, with particular emphasis in identifying the spectrum of the phenotypic expression of affected persons. Our results indicate that patients with the syndrome may have a variety of clinical signs, and an accurate diagnosis will therefore depend on a thorough and pertinent family history and physical examination.

Lymphangiomas of the head and neck in adults.

Lymphangiomas are considered to be congenital malformations of lymphatic channels. Fifty percent to 60% of these lesions are present at birth, with 80% to 90% detected by the second year of life. These lesions are rare in adults but may occur as late as the fifth decade of life. Primary lymphangiomas of the hypopharynx and upper aerodigestive tract are exceedingly rare in adults. The clinical courses of three adults with lymphangiomas of the head and neck are presented. In one case the lesion was confined to the hypopharynx. The literature contains considerable discussion about congenital lymphangiomas; few studies, however, have addressed this problem specifically in the adult population. The clinical appearance, difficulties of diagnosis, and guidelines of treatment are discussed.

Presentation of Wegener's granulomatosis in young patients.

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.

Association of adhesive macromolecules with terminal sprouts at the neuromuscular junction after botulinum treatment.

Small quantities of botulinum toxin (BTX) are useful in the treatment of certain movement disorders, such as laryngeal spasmodic dysphonia, blepharospasm, and cervical dystonia. However, the corrective paralytic effects of BTX are only temporary, in part because of the formation of remodeled neuromuscular junctions. Here, we questioned whether various factors within and near the neuromuscular junction could contribute to the remodeling seen after BTX treatment. BTX was injected subcutaneously in the region of the levator auris longus muscle. At 1-week intervals, levator auris longus muscles were removed and examined histochemically. As previously described, BTX treatment results in a progressive elongation of end plates. The neural cell adhesion molecule was not associated with the elongated end plates but was associated with the BTX-induced nerve sprouts after long intervals (3 to 4 weeks). Similarly, after BTX, laminin-1 (composed of alpha 1, beta 1, and gamma 1 chains) reactivity was associated with the nerve sprouts, but not with the end plates. Laminin beta 2 reactivity at the end plate dispersed somewhat within 1 week but remained diffusely associated with the elongating end plates for up to 5 weeks. Together these results suggest that neural cell adhesion molecule and laminins may participate in the sprouting observed after BTX treatment and that alterations in laminin beta 2 expression may participate in initial loss of contacts.

Viral labyrinthitis -- an experimental study.

Summary--An attempt was made to produce viral labyrinthitis in the rhesus monkey. Rhesus monkeys are susceptible to the mumps virus. Nine animals were used. After removal of the stapes, the left oval windows were plugged with Gelfoam soaked in a culture of live mumps virus; right oval windows were plugged with Gelfoam soaked in killed cultures. Animals were sacrificed at different time intervals, postinfection. In eight of the nine animals there was seroconversion from negative to positive; however, none of the animals developed the histologic changes of viral labyrinthitis.

Congenital laryngeal atresia. Histopathologic study of two cases.

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 embryologic events.

Reversible sensorineural hearing loss with intravenous erythromycin lactobionate.

Three patients are presented who developed reversible sensorineural hearing losses during treatment with intravenous erythromycin lactobionate. A fourth patient treated with erythromycin gluceptate did not develop hearing loss. Ototoxicity with erythromycin lactobionate has been previously reported in only three patients, one of whom had medication orally. Withdrawal of the antibiotic resulted in prompt improvement in every case with return of hearing to pretreatment levels.

The pathology of the temporal bones of a child with acquired cytomegalovirus infection: studies by light microscopy, immunohistochemistry and polymerase-chain reaction.

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.

Vascular variations of the inner ear.

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.

Neural hearing loss in a child with poliomyelitis: a histopathological study.

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 mildly 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.