Faculty Bibliography

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.

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.

Since December 1980, over 2,000 cases of acquired immunodeficiency syndrome (AIDS) have been reported. The charts of 72 patients admitted to the New York University Medical Center with a diagnosis of AIDS were reviewed with particular emphasis on presenting signs, symptoms and laboratory values. Symptoms tended to be non-specific and most often resembled an upper respiratory infection. Over 95% of the patients presented with either diffuse adenopathy, oral or facial lesions consistent with Kaposi's sarcoma, white oral lesions, or anergy. Laboratory findings included leukopenia, increased erythrocyte sedimentation rate, thrombocytopenia and anemia. The in-hospital mortality rate was 26%. The current status of our knowledge concerning AIDS is reviewed and discussed. The frequency and types of presenting signs and symptoms in the head and neck are reported in order to alert the otolaryngologic community to this entity

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.

In patients with acoustic neurinomas protruding less than 2 cm from the porus acusticus and free of the brain stem, hearing can often be preserved. Our neurosurgical/otologic team uses the combined suboccipital/transmeatal approach with the patient in the seated position. This operation has been attempted on 21 patients from 1974 to 1982. Nine had tumors larger than 2 cm that touched the brain stem; one had useful hearing preserved. Of the 12 patients with tumors smaller than 2 cm, hearing at or near the preoperative level was preserved in seven. In one patient, hearing was improved. There have been up to 7 years of follow-up with no recurrences of the neurinomas or diminution of hearing evident on computed tomographic scan. All had preservation of the seventh cranial nerve, while complications were mild and comparable to the remainder of our 101 patients operated on during the same period. We will review the surgical technique and audiologic results in detail

Cockayne's syndrome is a triad of dwarfism, retinal atrophy, and deafness. Over thirty cases have been presented in the literature. We have examined and audiometrically tested three patients (ages 13 to 17) with confirmed Cockayne's syndrome and have analyzed the temporal bones of another who died at age 24. To our knowledge this is the first reported temporal bone analysis of a patient with Cockayne's syndrome. Audiograms revealed bilateral symmetric sensorineural hearing loss that was greatest in the high frequencies. Temporal bone examinations revealed inner and outer hair cell losses in the basal turn of the cochlea with corresponding neuron losses in the spiral ganglion. We have found that the clinical and histopathologic features resemble those of presbycusis and conclude that this corresponds well with the generalized, rapid, premature aging process characteristic of this disease