I. Kaufman Arenberg

Melanocytes of the Vestibular Labyrinth and Their Relationship to the Microvasculature

The melanocytes of the vestibular labyrinth as seen in colored guinea pigs show a characteristic pattern of distribution in the wall of the utricle and in the ampullae, but they are not present in the wall of the saccule. They are found mainly in well-vascularized regions of apparent secretory or metabolic importance, including the “dark cell” areas. Their dendrite-like processes are often in intimate contact with the capillaries, although no pinocytotic vesicles or other indications of transfer of substances between the melanocytes and the capillary endothelium are seen under the electron microscope. In the human ear, the apparent density of the melanocytes varies with skin color. They are numerous in the cochlea, especially in the bony wall of the modiolus and on the osseous spiral lamina, and they occur also in Reissners membrane and in the stria vascularis. In the vestibular system they are found in the wall of the saccule as well as in the utricle, the crus commune, and the ampullae, but not in the semicircular canals. They tend to be diffusely scattered, rather than to form the well-defined, intensely pigmented areas that are characteristic of the guinea pig. Close contacts with capillaries are seen mainly in the tympanic portion of the spiral ligament. The significance of melanin and the melanocytes in the labyrinth is unknown, but both the anatomical relation of certain melanocytes to capillaries, and the biochemical relation of melanin to the catecholamines support the hypothesis that they may have a vasomotor function. Other evidence, including the not infrequent association of sensorineural deafness with hereditary disorders of pigmentation also suggests that the melanocytes may play a role of some biological consequence in the inner ear.

Demonstration of Autoantibodies to the Endolymphatic Sac in Meniere's Disease†

Recent evidence suggests that immune mechanisms may underlie some cases of Menieres disease. This study was conducted to determine whether an autoimmune mechanism is involved. Sera from 30 patients with Menieres disease were reacted with human endolymphatic sacs and examined by indirect immunohistochemistry and fluorescence microscopy. Three of the samples (10%) showed positive staining, indicating immunoglobulin G (IgG) binding against the sac. No positive staining occurred when sera from healthy individuals or phosphate‐buffered saline was used as a control. Clinical data showed an association between immunoreactivity and extent of disease (worse hearing over a shorter disease course and bilateral involvement). This study suggests that, in some cases of Menieres disease, autoantibodies directed against human endolymphatic sac are present in the sera, supporting the theory that a specific autoimmune reaction takes place in a minority of patients with Menieres disease.

Immunoperoxidase study of the endolymphatic sac in meniere's disease

A growing body of evidence suggests that some cases of Menieres disease may be mediated by immune mechanisms. Because endolymphatic sac dysfunction is believed to be an underlying cause of Menieres disease, this study used immunohistochemical techniques to demonstrate the presence of immune complex deposition in the sacs of patients with Menieres disease. Positive immunoglobulin G (IgG) staining was noted in 10 of 23 sac biopsies from Menieres patients, with 2 specimens showing perivascular deposition. Only 1 of 5 control specimens was only slightly positive for IgG. Clinical correlation showed a statistically significant increase in disease bilaterality (P<.05), larger summating potential/ action potential (SP/AP) ratios with electrocochleography (ECoG), and a tendency toward worse hearing and more progressive disease among the immunopositive Menieres patients. The results provided histological evidence of immune injury in the endolymphatic sacs of patients with Menieres disease.

Ventilation tube surgery and middle ear irrigation

Tympanostomy and insertion of ventilation tubes has become one of the most commonly performed operations in the United States. Most authors reporting complications of this procedure describe a postoperative rate of otorrhea in the range of 10%–20% with some reports much higher. This rate of presumed suppuration would generally be considered high by surgeons operating in other areas of the body.

Ultrastructural analysis of endolymphatic sac biopsies. Biopsy technique and identification of endolympathic sac epithelium.

This study describes the technique and instrumentation necessary to perform a double-wall biopsy of the endolymphatic sac (ELS). Since this technique protects the lumen of the ELS, which is within the center of the biopsy sample surrounded by the lateral and medial wall, the ultrastructure of the ELS epithelium, luminal contents, and perisaccular connective tissue tends to represent the in vivo status. The biopsy specimen can be analyzed by electron microscopy for clinical-pathologic correlation. Ultrastructural analysis (1) provides definite identification of the ELS and epithelium, (2) may provide insight into a possible role of the ELS in the pathogenesis of endolymphatic hydrops, and (3) can provide much improved clinical-pathologic correlation over only light microscopic studies.

Perilymphatic fistula: An unusual cause of meniere's syndrome in a prepubertal child†

Non‐traumatic, spontaneous perilymph fistula of the oval window is an unusual cause of Ménières syndrome in a child. In this case, all of the audio‐vestibular criteria for a diagnosis of Ménières syndrome (or clinical endolymphatic hydrops), including a temporary threshold shift after the glycerol test, were demonstrated. At exploratory tympanotomy, a perilymph fistula, demonstrated at the footplate margins was plugged with fat. There was significant immediate postoperative and six‐month follow‐up improvement in perception of pure tone, speech reception threshold (SRT) and discrimination score.

Presence of acidic protein-bound carbohydrates in the endolymphatic sac and duct of fetal, neonatal and adult rats, and adult humans.

Seymour’* felt that the endolymphatic sac was an auxiliary site of endolymph production, “to insure complete filling of the phylogenetically older utriculus and semicircular canals.” C i r n i n ~ , ~ working with rabbit embryos described glandular structures in the pars rugosa, and felt that the endolymphatic sac produced endolymph during prenataI life. This secretory activity of the sac in rabbits was felt necessary to assure early endolymphatic filling of the primitive labyrinthine cavities.

Vincent's Violent Vertigo: An Analysis of the Original Diagnosis of Epilepsy vs. the Current Diagnosis of Meniere's Disease

The authors propose to correct the historical misimpression that Vincent van Goghs medical problems resulted from epilepsy. Rather, the authors propose his main medical problem was Meniéres disease. The authors have reviewed the 796 personal letters written by van Gogh. The symptoms of his Vertigo attacks, their presentation and duration as described in these letters, taken as a whole, are consistent with the clinical picture of Meniéres disease, not epilepsy. They point out that Prosper Meniéres description of his syndrome was not well known at the time of van Goghs death, and was often misdiagnosed as epilepsy. During the last years of his life, van Gogh was labeled epileptic, although no rigid criteria for this diagnosis are evident. This diagnosis is still prevalent in the art history literature today. His symptoms included episodic vertigo and dizziness, physical imbalance, hearing symptoms, ear noises (tinnitus) as well as a presumed secondary psychological reaction to his physical symptomatology. van Goghs diagnosis of epilepsy is based on written diagnosis in his medical records in 1889 when he was interred (voluntarily) in St. Remy at an asylum for epileptics and lunatics.

Radiographic classification of the vestibular and cochlear aqueducts: The paired correlation between normal and abnormal vestibular aqueduct and cochlear aqueduct anatomy†‡

Multidirectional tomography (MDT) can be useful in determining the caliber, shape, and course of the vestibular aqueduct (VA) and cochlear aqueduct (CA). Clinical decisions have been based on the findings from MDT. Unfortunately, the clinical utility of these observations has been confusing and controversial because similar MDT techniques were not used. This study will address some of the difficult questions and clinical controversies derived from MDT observations. This new perspective has evolved with the use of high resolution computed tomography (HRCT). An analysis of 750 petrous bones for the occurrence of the various types of VAs and CAs using Gados classification, further vestibular aqueduct and a variation of Gados classification for the cochlear classification is reported. The distribution of the possible paired types of VA and CA arc evaluated. MDT results indicate that the paired analysis in patients with inner ear dysfunction is not useful, cost effective, diagnostic, or of prognostic value. MDT can provide clinically valid observations of periaqueductal and perilabyrinthine pneumatization which is helpful in anticipating the size and position of the endolymphntic sac at the time of surgery for those few patients who may benefit from endolymphatic system surgery. However, when a comparison is made between MDT and CT of 60 ears in those same patients, the clinical limitations of MDT for inner ear diagnosis and prognosis became apparent. The future for HRCT scanning with reformatting holds potential for clinically meaningful visualization of inner and middle ear structures previously expected from MDT imaging.

Middle Ear Irrigation During Insertion of Ventilation Tubes

Tympanostomy and insertion of ventilation tubes has become one of the most commonly performed operations in the United States. Most authors reporting complications of this procedure describe a postoperative rate of otorrhea in the range of 10-20% with some reports much higher. This rate of presumed suppuration would generally be considered high by surgeons operating in other areas of the body. It is a commonly accepted surgical practice to follow incision and drainage of a relatively closed space infection with irrigation of that space. This is true in the surgery for the paranasal sinuses, deep space infections of the neck, joint spaces and abscesses in general. However, this practice is not routinely performed when incising and draining the middle ear. We have completed a prospective controlled double blind study on post tympanostomy tube otorrhea utilizing irrigation of the middle ear. In 220 consecutive cases, the use of middle ear irrigation reduced postoperative infections in the first 6 months from 16 to 4%. Irrigation was also found to be useful in removing very thick effusions from the middle ear by displacement, including those effusions localized in the hypo or epitympanum which were not initially identified at the time of incision and suction. A soft plastic, angled irrigation catheter with radial ports was developed for this purpose.