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.
There is a traditional view that chronic otitis media and chronic mastoiditis must exist in the presence of a tympanic membrane perforation. Based on a human histopathological study of 123 temporal bones with chronic otitis media out of 333 temporal bones with all forms of otitis media pathology, only 24 patients (36 ears) had symptoms of otological disease recorded on their charts and only 19.5% of these had an associated tympanic membrane perforation. Unsuspected findings of chronic otitis media (active or inactive) are occasionally confirmed at exploratory tympanotomy. Such quiet chronic pathological findings in the middle ear have occurred in association with endolymphatic hydrops and cochlear end organ lesions suggesting the possibility that silent chronic otitis media may help explain sensorineural hearing loss, vertigo, and tinnitus for certain patients. We put forth the hypothesis that clinically undetectable silent otitis media in neonates and infants might help explain why certain children are otitis media prone. Our otopathology laboratory includes 111 ears harvested from neonates and infants (average age 8 months). Histological analysis revealed normal findings in some, whereas significant numbers of others show all forms of otitis media, including purulent otitis media, chronic otitis media, serous otitis media, and mucoid otitis media, with occasional overlap of types. Clinical implications will be discussed with emphasis on silent otitis media and its relationship to the pathogenesis of otitis media and silent chronic otitis media and its possible role in causing insidious labyrinthine changes. A significant outcome of this study was the marked degree of unresolved mesenchyme in the clinically manifest otitis media group or silent otitis media group as compared to a control group. This association and role of mesenchyme is discussed relative to the pathogenesis of infantile otitis media.
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 Cortisporin otic 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 Cortisporin otic 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. The 8 ears receiving Cortisporin otic drops mixed with green dye from animals with normal eustachian tubes showed staining of the round window membrane at sacrifice, while the 6 ears receiving Cortisporin otic suspension and green dye from animals with eustachian tube obstruction demonstrated no dye in the middle ear. All animals receiving Cortisporin otic drops in the right ear showed an intra-aural difference in action potentials with the right ear being attenuated by an average of 10.3 dB at 2,000 Hz, 12 dB at 4,000 Hz, 21 dB and 8,000 Hz, and 26 dB at 12,000 Hz. Morphological study revealed hair cell loss in the hook portion of the cochlea in those animals receiving Cortisporin otic drops. It was concluded from this study that, in patients with patent tympanostomy tubes in place, potentially ototoxic topical agents should be used with caution.
\s=b\Sensorineural hearing loss (SNHL) has been described clinically following chronic otitis media with effusion, but to the best of our knowledge, no studies have demonstrated SNHL in an animal model of otitis media. Using the chinchilla model of pneumococcal otitis media, significant SNHL was demonstrated after purulent otitis media, especially at higher frequencies. Animals with otitis media received penicillin G procaine treatment for five days after otitis media with effusion (OME) was first documented; resolution of middle ear infection was confirmed by middle ear effusion culture in all animals. Both the inoculated and uninoculated ears were examined by tone burst\p=m-\ elicited compound action potential at threshold. The inoculated ear showed a marked hearing loss of 13 to 36 dB three to four days after OME was first documented; a hearing loss up to 24 dB persisted two to five weeks after inoculation.The change in the compound action potential was highly significant at all frequencies studied. Conductive losses were largely ruled out because there was no middle ear effusion at death and the tympanogram was normal. Purulent labyrinthitis was ruled out by histopathological study. These results indicate that purulent pneumococcal otitis media in the chinchilla model causes significant SNHL and suggest that the pathogenesis of SNHL associated with chronic OME in humans may be studied in this model. (Arch Otolaryngol 1985;111:794-798) Sensori neural hearing loss (SNHL) has been described in patients with chronic otitis media15; in these studies, deterioration in bone conduc¬ tion has been used as an indication of SNHL. However, presbycusis is also associated with deteriorating bone conduction and must be taken into consideration when interpreting the cochlear losses in chronic otitis media.Paparella et al6 compared bone con¬ duction loss by decade in diseased and normal ears and found an increased incidence of SNHL in patients with chronic otitis media in all decades, suggesting that the SNHL was not due to presbycusis. Walby et al5 also noted an abnor¬ mality in bone conduction in their retrospective study of 87 patients with unilateral uncomplicated chronic sup¬ purative otitis media. However, their study of cochlear histopathology revealed no greater loss of specialized sensorineural structures in infected ears than in normal control ears; hence, they concluded that alteration in the mechanics of sound transmis¬ sion was a more plausible explanation for the hearing loss in chronic otitis media.Chinchillas have been used widely to study otitis media and cochlear function; longitudinal studies are pos¬ sible in this species, with periodic sampling of middle ear effusion, which cannot be easily performed in humans with otitis media. The present study was designed to exam¬ ine relationships between acute puru¬ lent otitis media and cochlear func¬ tion in the chinchilla model. Basic bactériologie and pathologic studies in the chinchilla model of otitis media7"9 and basic neurophysiologic studies in the ch...
Approximately 3% of all patients with Meniere's 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 Meniere's 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.
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