Donald A. Shea

Endolymphatic hydrops and otitis media.

Clinical observation of patients with fluctuant sensorineural hearing loss following or occurring with chronic otitis media led to the hypothesis that endolymphatic hydrops can result from chronic otitis media. Illustrative case reports are described. This hypothesis resulted in a temporal bone study of 560 cases in which 109 temporal bones demonstrated the presence of hydrops and 194 evidenced otitis media. Seventy‐five cases demonstrated both otitis media and hydrops, of which 20 cases were selected for more detailed histo‐pathological study. An interesting finding was the presence of apical hydrops in every case of the latter group. Statistical interpretation of this data helped rule out a coincidental or chance occurrence. A discussion of this clinical relationship included the significance of subclinical (silent) otitis media as a possible cause of endolymphatic hydrops.

Otologic Histopathology of Fabry's Disease

Fabrys 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.

Tympanostomy tubes and otic drops

Otic drops have been proposed as a form of prophylaxis against the otitis media which follows middle ear contamination by water in patients with tympanostomy tubes. The potential adverse effects of this form of therapy were studied in chinchillas with tympanostomy tubes; 31 chinchillas underwent bilateral tympanostomy tube insertion. Seven animals had a mixture of green dye and Cortisporinotic suspension placed in both external auditory canals 24 hours following the placement of tympanostomy tubes and were sacrificed 30 minutes later for gross examination; 3 of these animals had previous eustachian tube obstruction with Silastic sponge. Twenty‐one animals had Cortisporinotic suspension placed in the right external auditory canal on postoperative days 3, 4, 5, 6 and 7. No otic drops were placed in the left ear. Ten of these 20 animals had VIIIth nerve action potentials measured on postoperative day 17 and the other 11 animals had VIIIth nerve action potentials measured on postoperative day 42 followed by immediate sacrifice for histological examination and scanning electronmicroscopy. The remaining 3 animals had VIIIth nerve action potentials measured 21 days following tympanostomy tube insertion and served as electrophysiological controls.

Ménière's disease in children.

Approximately 3% of all patients with Menieres disease are in the pediatric age group. These children require extensive evaluation. A history of physical or acoustic trauma should be sought and an allergic work‐up should be obtained. A search for metabolic disturbances and identification of inflammatory disorders is also necessary. If a treatable etiology is identified, specific therapy should be directed toward its control. If, after careful evaluation, the etiology remains obscure, non‐specific therapy should be instituted in an effort to alleviate the symptomatology of Menieres disease. Surgical decompression of the endolymphatic sac and drainage into the mastoid cavity results in relieving the symptoms, particularly vertigo, and appears to be efficacious in patients who have failed diligent attempts at medical therapy.

Passage of albumin from the middle ear to the inner ear in otitis media in the chinchilla

A study of the permeability of the middle ear-inner ear interface for macromolecules was carried out in chinchillas with open and obstructed eustachian tubes utilizing tritiated human serum albumin and immunoelectrophoresis. Tritiated albumin was placed in the round window niche area or normal animals and animals in which the eustachian tubes had been obstructed for 24 hours or 14 days. The tritiated albumin was allowed to remain in the middle ear cavity for 24 hours, Samples of middle ear effusion, perilymph, blood and cerebrospinal fluid were collected and measured for radioactivity. Radioactivity was demonstrated in the perilymph. Samples of middle ear effusions and perilymph were also studied by immunoelectrophoresis with goat antihuman albumin. Albumin placed in the round window niche of an experimental animal could be recovered unchanged in the perilymph. The results suggest a pathophysiologic explanation for the association of otitis media and sensorineural hearing loss or endolymphatic hydrops.

Mucopolysaccharidosis I-H (Hurler's Syndrome) and Human Temporal Bone Histopathology

Six temporal bones from three patients with mucopolysaccharidosis I-H are described. All three patients were diagnosed as having mucopolysaccharidosis I-H by enzyme analysis, and all fit the phenotypic criteria of this disease. Family histories of the three cases described were negative for mycopolysaccharide-storage diseases. All three of the patients suffered chronic recurrent otitis media from infancy through death. Common histopathologic findings include otitis media, residual mesenchyme in the round window niche, partial occlusion of the middle ear cavity, and basophilic concretions within the stria vascularis. The common severe histopathologic changes observed in this study and similar findings in the temporal bones described in other studies lead the authors to believe that ear involvement in this disease may be more common than was previously suspected.

Pneumococcal otitis media following middle ear deflation.

Temporal bone histology has been studied in chinchillas following middle ear deflation in the presence of nasopharyngeal colonization with S pneumoniae. All animals sacrificed within one hour following deflation had similar middle ear hsitology regardless of whether or not the animals had been colonized with S pneumoniae. All animals with negative nasopharyngeal culture for S pneumoniae sacrificed from 3–24 days following − 30 mm Hg negative middle ear pressure had negative middle ear cultures and normal morphology. During the same time period, however, nearly 60% of animals colonized with S pneumoniae developed acute otitis media with positive middle ear cultures for S pneumoniae. Histologic changes in the middle ear included the presence of effusion, leukocytes, granulation tissue, osteoneogenesis, mucosal metaplasia, and hemorrhage. Changes in the inner ear were much less frequent and included some leukocyte infiltration with hyperemia in the scala tympani and endolymphatic hydrops.

Silent Otitis Media: An Animal Study

Recent clinical and laboratory observations suggest that otitis media can occur in the absence of visible tympanic membrane changes (silent otitis media). Temporal bone histopathology was studied in 38 chinchillas that had been subjected to conditions known to predispose these animals to otitis media. Morphological changes were compared to a control group of 25 nonmanipulated animals. The tympanic membranes were examined at intervals and during the immediate premortem period with the operating microscope. Thirty-one tympanic membranes in the experimental group and all tympanic membranes in the control group were judged to be normal (gray and translucent) throughout the study period. The middle ears from the experimental group histologically manifested varying amounts of effusion, mucosal edema, granulation tissue, hyperemia of the subepithelial space, hemorrhage in the middle ear space, and abundant polymorphonuclear leukocytes in the middle ear cleft. These findings were not present in the control group.

Sac Decompression for Refractory Luetic Vertigo

Luetic hydrops can occur in both congenital and acquired lues and otologic manifestations may be indistinguishable from Menieres disease. Eight endolymphatic sac decompression procedures were performed on 6 patients with refractory luetic vertigo. Of those, 3 had congenital and 3 acquired lues. All cases had received extensive anti-luetic medical therapy without any discernible improvement in the vertiginous attacks. In our 6 patients with luetic hydrops the main complaint was severe refractory vertigo, indeed, disabling in most cases. Postoperatively, all cases remained free from these severe attacks and the vertigo was controlled or eliminated in all cases. Although hearing was improved in one patient, hearing seemed not to improve, generally speaking, as a result of the long-term postoperative follow-up. We describe our method of sac decompression and drainage and suggest that this procedure might be considered for patients with uncontrollable luetic vertigo who have not benefited from adequate medical therapy.

Otitis media, cleft palate, and middle ear ventilation.

Chinchillas with unilateral tympanostomy tubes in place underwent palate-clefting in an effort to determine the histologic and bacteriologic effects of using tympanostomy tubes in the treatment of otitis media. The tympanostomy tube appeared to almost totally eliminate the occurrence of middle ear effusion but had much less, if any, effect on eliminating the middle ear inflammation which occurs in the clefted chinchilla.