Biomedical subjects
Publications and source records attributed to M M Paparella.
Early and aggressive treatment of allergic, inflammatory, and infectious diseases of the nose and paranasal sinuses should reduce the potential for chronic and irreversible disease of the sinus mucosa. Identification of underlying allergies, reactive mucosa, impaired mucosal transport, anatomic obstruction, or narrowing are key elements in establishing an effective treatment plan and the ultimate resolution of these disease processes.
Fabry's disease is a rare progressive X-linked recessive disorder of glycosphingolipid metabolism. The accumulation of glycosphingolipids occurs in virtually all areas of the body, including the endothelial, perithelial, and smooth-muscle cells of blood vessels, the ganglion cells of the autonomic nervous system, and the glomeruli and tubules of the kidney. Although otologic symptoms have been described in these patients, to our knowledge there have been no temporal bone histopathologic reports. We describe the clinical histories, audiometric results, and temporal bone findings of two patients with this rare disorder. Both patients demonstrated a bilateral sloping sensorineural hearing loss audiometrically. Middle ear findings of seropurulent effusions and hyperplastic mucosa were seen in all four temporal bones. Strial and spiral ligament atrophy in all turns, and hair cell loss mainly in the basal turns, were also common findings. The number of spiral ganglion cells was reduced in all temporal bones; however, evidence of glycosphingolipid accumulation was not observed in the spiral ganglia.
Systemic vasculitis includes a broad spectrum of disorders that may involve blood vessels of any size in any organ system. Systemic vasculitis is associated with immunopathogenic mechanisms. Sixteen temporal bones from eight persons were studied to determine histopathologic changes that occur in systemic vasculitis. Three persons had Wegener's granulomatosis, two had polyarteritis nodosa, and three had systemic lupus erythematosus. Otitis media was seen in 15 ears, with ten ears showing chronic middle ear changes and two showing fibrotic inner ear changes. In Wegener's granulomatosis, granulation tissue was observed around the eustachian tube and protympanum, and in polyarteritis nodosa, inflammatory cell infiltrate and thickened blood vessels were observed around the facial nerve. Although sensorineural hearing loss has been described clinically in systemic lupus erythematosus, the present report describes findings in temporal bones, including severe fibrosis and new bone formation throughout the inner ear.
Diseases with interaction between the middle ear and inner ear include 1) congenital anomalies, 2) trauma, 3) infection/inflammation, 4) tumors, 5) granulomas, 6) ototoxic eardrops, 7) cochlear implants, 8) otosclerosis, 9) Meniere's disease-decompensated, and Meniere's disease with perilymphatic fistula, and 10) perilymphatic hypertension. Clinical and pathological characteristics are briefly categorized in this survey. The clinical utility of exploratory tympanotomy in diagnosis and treatment of middle ear pathology and middle ear/inner ear interactions is commented upon.
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Sensorineural hearing loss can arise from a multitude of etiologic factors. In evaluating hearing loss in a child, it is important to determine if the loss is congenital or delayed and if it is genetic or nongenetic in origin. Examples of etiologies based on this classification are given, including the symptoms and extent of impairment. Determining the extent and etiology of hearing impairment is only the first step; the aim is to maximize the speech and language development of the hearing-impaired child.
The socioeconomic changes and technological advances that have occurred in the field of hearing aid dispensing are chronicled. The various instruments available to aid the hearing-impaired person, including digital electronics and programmable hearing aids, are described. The qualifications required of those who deliver hearing aids are also discussed.
A combination of otologic techniques has been presented in the step-wise surgical rationale that we call the flexible approach. The sequence of steps involved in the flexible approach allows the surgeon to adjust his or her surgery to the individual patient's needs. Very often, in patients with chronic otitis media, a middle ear reconstructive procedure (ossiculoplasty including lateralization of the malleus, removal of pathological tissue, insertion of an implant of silicone rubber sheets, and use of ventilation tubes with or without tympanic grafts) will obviate mastoidectomy. When a mastoidectomy is indicated, the IBM is a suitable procedure that encompasses the advantages of both closed and open mastoidectomy techniques.
Histopathological studies were done in 22 deaf dogs consisting of 10 Dalmatians, 5 English setters, 2 Great Danes, 2 foxhounds, 1 shepherd, 1 bulldog and 1 Australian sheep dog. Hypoplasia or aplasia of the sensory cells of the organ of Corti, stria vascularis and macula sacculi (pars inferior) was noted in all deaf dogs examined, indicating the pathology of inner ear malformation in these dogs to be of Scheibe's type. A solidified and calcified tectorial membrane was noted in 19 out of the 22 deaf dogs. A calcified sclerosis of the tectorial membrane is thought to be a characteristic finding of genetically inherited anomaly of the inner ear in deaf dogs. Distortion of the tectorial membrane, absence of the sensory cells in the organ of Corti, agenesis of the stria vascularis and abnormalities of the saccule described Scheibe's dysgenesis of the pars inferior as the pathological correlate for deafness in these dogs as assessed functionally.
A syndrome termed perilymphatic hypertension is described as being seen in a small subset of patients with sudden sensorineural hearing loss. A patent or semipatent cochlear aqueduct or modiolus are considered precursors to this condition. Perilymphatic hypertension is believed to predispose to perilymphatic fistula, which may be part of the process of resolution. The pathogenesis for perilymphatic hypertension and fistula are hypothesized and discussed. Treatment for this subset of patients consisted of paracentesis of the round window membrane followed by grafting, with improvement of hearing in certain patients. In no instance did hearing subsequently decrease.
A subset of patients with intractable Menière's disease had a good result for months or years following endolymphatic sac enhancement (ESE), only to develop recurrent symptoms of Menière's disease, including vertigo and deafness. Revision procedures (ESR) revealed extrasaccular fibrosis or granulation tissue, osteoneogenesis, and aditus block syndrome resulting in saccular obstruction and a tight contiguous dura in 26 patients (4% of total ESE patients to date). Following ESR, symptoms and pathologic findings in the main were diminished; in one patient, useful hearing was restored from complete deafness. ESR provides another option for managing these recurrent problems, and most of our patients have continued to do well. This option of therapy, plus a unique opportunity to assess the pathogenesis of Menière's disease in humans will be discussed.
Studies of the permeability of the round window membrane (RWM) during otitis media are important because toxins and cellular components that occur in otitis media as well as pharmacologic agents used in treating the disease have the potential to cross the RWM and pass into the inner ear. Twenty-five cats were evaluated electron microscopically as to the passage of a tracer, horseradish peroxidase, through normal RWMs and RWMs three days, one week, and two weeks following eustachian tube obstruction. Passage at three days following obstruction was similar to passage through the normal RWM. Following one to two weeks of obstruction, the permeability of the membrane was drastically reduced. The reduction in permeability was probably due to the presence of residual effusion overlying the membrane, the presence of granulation tissue within the niche, and a thickening of the RWM.
The thickness and morphologic characteristics of the round window membrane were evaluated in temporal bones from normal subjects as well as those with serous otitis media, purulent otitis media, and chronic otitis media. Temporal bones were studied in chronological order in six age ranges to determine the possibility of age-related differences. No significant difference in the mean thickness of the round window membrane was observed in terms of age groups in normal temporal bones or temporal bones from patients with otitis media; however, a significant difference in the mean thickness was observed in the various forms of otitis media compared with the normal round window membrane in all age groups. The membrane was thickest in patients with chronic otitis media when compared with that in normal subjects or those with serous or purulent otitis media. The epithelial layer (including the subepithelial space) and the fibrous layer were measured individually to determine in which layer the change in mean thickness occurred. These measurements showed an involvement of all layers of the round window membrane in those groups with otitis media, with maximal involvement of the combined epithelial layer and subepithelial space.
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The round window niche and membrane can be involved in clinical problems including perilymphatic fistulas, sensorineural hearing loss in otitis media, and a variety of others. The background of these problems is documented by a review of the literature and recent experimental evidence on round window grafting and tracer studies. Clinical pathological correlates including recent clinical experience are discussed for perilymphatic fistulas (sudden deafness, chronic progressive fluctuating sensorineural hearing loss, and association with Meniere's disease), sensorineural hearing loss in acute and chronic otitis media, and findings in round window membrane including absent round window reflex in cases of exploratory tympanotomy and otosclerosis. In addition, a new syndrome termed perilymphatic hypertension is described as seen in a subset of patients with sudden sensorineural hearing loss.
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Labyrinthectomy is indicated for a patient who has unilateral peripheral labyrinthine disease with unserviceable hearing loss. For most patients, labyrinthectomy provides complete relief from intractable vertigo. Some patients continue to have persistent vertigo, however, even after a complete destructive labyrinthectomy. Only after cochleovestibular nerve sections were these patients relieved of intractable vertigo. Representative cases of cochleovestibular nerve sections in labyrinthectomized patients are presented. The reason why the cochleovestibular nerve section relieves the symptom of vertigo in labyrinthectomized patients is not clear. Labyrinthectomy destroys vestibular sense organs, while cochleovestibular nerve section eliminates spontaneous activity and prevents the possibility of nerve regeneration by excision of the ganglion. For those patients who required vestibular nerve sections, there may have been abnormal excitation of vestibular nerve fibers. This might be explained by incomplete labyrinthectomy, regeneration of vestibular nerve fibers, contribution from vestibular ganglia or nonfunctioning cochlea, and vascular loop syndrome.