Faculty Bibliography

Adenomatous lesions of the temporal bone are seldom encountered in clinical practice. Four cases of primary adenocarcinoma of the middle ear are reported. All four patients presented with a mass behind an intact tympanic membrane. Two of the four patients had received radiation to the head and neck area 10 or more years prior to their developing adenocarcinoma of the middle ear space. Although this tumor is locally invasive, it does not appear to be highly aggressive and can be treated by local excision, i.e., mastoidectomy with tympanoplasty.

Abnormal mineral deposits from the membranous labyrinths of deaf Dalmatian puppies were examined microscopically. Most such deposits were located on the saccular otoconial membrane and were associated with collapse of the saccule and of Reissner's membrane. In both ears of one pup, crystals resembling otoconia were found on the surface of the stria vascularis. In two others, grossly abnormal crystals were seen on the utricular otoconial membrane, and in one of these crystals were also attached to the cupula of the left horizontal canal. In four animals the tectorial membrane was calcified. Most deposits consisted of apatite spherulites, but large multiple crystals (probably of calcite) were occasionally present. Some of the abnormal mineral deposits in the Dalmatian closely resemble those found in man, and they may originate in the same way.

Four variations and degrees of severity of the Mondini malformation were found in the temporal bones from two neonates, one with congenital heart disease and the other with trisomy D, and from one teenager with leukemia: 1) short cochlea and normal vestibular organs; 2) short cochlea and persistent horizontal canal anlage; 3) markedly shortened cochlea with no modiolus, wide internal auditory meatus, and persistent horizontal canal anlage; 4) same as variation 3, but with persistent anlagen in all semicircular canals. Variations 3 and 4 were from the case of trisomy D, in which the left cochlea had a normal hair cell population but few nerve fibers, and the intraganglionic spiral bundle was displaced from Rosenthal's canal to the osseous spiral lamina. The right ear had no cochlear nerve fibers; the organ of Corti was present, but hair cells were unusually small. In the case of trisomy D, both ears showed subtotal loss of vestibular nerve fibers. Although the rudimentary cristae of the right ear had numerous hair cells, the macular hair cells were fewer and malformed. No hydrops was present.

Electrocochleograms (ECochG) in response to tone bursts and electronystagmograms (ENG) during sinusoidal rotational stimulation were monitored in guinea pigs equipped with chronically implanted round-window and periocular electrodes before and for as long as 90 days after uni- or bilateral surgical obliteration of the endolymphatic duct. The presence of cochlear and saccular endolymphatic hydrops was verified during microdissection of the inner ear, and cochlear hair cell counts were performed in some of the animals. The results indicate that in surgically-induced endolymphatic hydrops, cochlear and vestibular changes occur similar to those observed clinically in Meniere's disease: fluctuant threshold shifts, threshold elevation at low frequencies, sensorineural loss at all frequencies with recruitment, increase in the ratio of summating potential to compound action potential (SP/CAP), asymmetry and recruitment in the ENG, and increased vestibular excitability, with occasional post-rotational and spontaneous nystagmus. Functional changes could not be clearly correlated with those seen by light microscopy, since hair cell loss was confined mainly to the apical coil. The observations indicate that the guinea pig with surgically-induced endolymphatic hydrops can be a useful model of Meniere's disease, not only on morphological grounds, but also on the basis of functional changes, which over the relatively short term of observation represent a dysfunction rather than a loss of sensorineural structures.

A 57-year-old patient became totally deaf two days after receiving excessive doses of the aminoglycosidic antibiotic lividomycin parenterally for 14 days; she died four and a half months later. Her temporal bones were examined by microdissection, surface preparation, and serial sectioning of the modiolus. Loss of inner and outer cochlear hair cells was virtually complete. Refractile concretions were scattered along the atrophic stria vascularis, especially in the middle turn. The distal half of the radial cochlear nerve fibers in the osseous spiral lamina had degenerated, but closer to the modiolus they appeared to be intact. The spiral ganglion in the basal turn showed partial loss of neurons. Scanning electron microscopy revealed hair cell loss from the vestibular end-organs, more severe in the ampullar cristae than in the utricular macula. The dark cells of the utricular wall appeared to be altered.

Ototoxic and nephrotoxic potentiation with concomitant cis-diamminedichloroplatinum, or cis-platinum II (CSP), and aminoglycoside therapy was investigated in the guinea pig. We evaluated possible potentiation of the toxic effects of CSP and kanamycin compared with CSP alone in the inner ear and kidney and quantitatively localized CSP in the cochlea with gamma emission analysis of 195mPt. Kanamycin-treated animals demonstrated cytocochleograms and ABR waveforms, absolute latencies, and interwave latencies for waves I, II, and III similar to control animals at our maximum level of acoustic stimulation. CSP treatment produced 60% to 70% mean outer hair cell (OHC) loss in the basal turn of the cochlea, a reduction in ABR waveform and amplitude, and an increase in latencies of ABR waves I, II, and III. Combined CSP and kanamycin treatment produced 90% to 100% mean OHC loss in all rows of the basal turn of the cochlea, with no discernible ABR waveform corresponding to the region stimulated by a 4500 to 7000 Hz acoustic click. Combined treatment produced the most significant cortical medullary tubular necrosis and interstitial nephritis. Furthermore, this study reports for the first time localization of platinum in the inner ear.

The temporal bones from a 55-year-old woman with deafness and dizzy spells caused by a right-sided acoustic neurinoma were examined by the techniques of microdissection, surface preparations, and celloidin sections. Sensorineural degeneration was present in the basal end of the right cochlea but was not severe enough to explain the deafness. The compression of the cochlear nerve by the tumor was postulated to have caused deafness by injury to neurons adjacent and central to the tumor. There was profound degeneration of the vestibular nerve and sensory cells. A gelatinous material occluded the scala vestibuli, and the vestibular fluid spaces contained an amorphous proteinaceous substance. Fewer erythrocytes were seen in the vessels of the right cochlea, but there was no atrophy of capillaries to indicate long-term reduction of circulation. Vasculoneogenesis of venous vessels had occurred in the scala tympani, probably as a result of venous stasis.

In the past decade the number of voice laboratories has increased dramatically. Their primary mission is to enhance patient care by the application of knowledge gained from basic research. They also are dedicated to further improvement of diagnostic and therapeutic resources. The strength of the voice laboratory lies in collaboration between the clinician and the scientist.

Auditory evoked potentials are nearly all on-effects and the 'effective stimuli' for them are necessarily brief. Their frequency specificity is therefore limited, especially for the brainstem responses, because of the well-known trade-off between duration and frequency specificity. Brainstem responses are of special interest because they are unchanged in the sleep-like sedation that is required for difficult-to-test children. The middle-latency responses do not meet this requirement. Two patterns of tone burst that are appropriate and promising for the slow cortical potentials and for brainstem potentials, respectively, have rise and fall times of 2 periods of the modulated tone and plateaus of 10 (or 7) periods and 1 period, respectively. Their behavioral thresholds are nearly insensitive to difference in repetition rate between 4 and 40 stimuli/s. Their peak equivalent SPL threshold values at 500, 1 000, 2 000 and 4 000 Hz have been determined for 16 otologically normal ears. Using these reference levels, audiograms have been obtained for subjects with impaired hearing. The audiograms for 'flat' hearing losses do not differ significantly from the corresponding conventional pure-tone audiograms. The slopes for steep high-frequency hearing losses are underestimated, however, particularly with the brief (2-1-2) pattern. Nevertheless, the 2-1-2 pattern appears to be close to the best possible compromise.

Audiologic findings in a child who received a single-channel cochlear implant are presented. The measures used were threshold sensitivity to frequency-specific stimuli and results on various subtests from the Test of Auditory Comprehension (Trammel, 1976), the Monosyllable, Trochee, Spondee Test (Erber & Alencewicz, 1976), and the Minimal Auditory Capabilities Battery (Owens, Kessler, Telleen, & Schubert, 1981). The results for the implanted ear indicated uneven change in performance across measures compared to results with conventional amplification prior to receiving the implant. Performance of the implanted ear did not approach performance of the better contralateral ear. Observations by teachers and guardians indicated that there was no apparent change in auditory performance even after the cochlear implant had been worn for 6 months. Overall there was no evidence that the cochlear implant worn for 6 months provided any practical benefit to this child.