Laboratory diagnosis of immune inner ear disease

Inherited antithrombin deficiency is associated with an increased risk of thrombosis, primarily venous rather than arterial. Most affected individuals have inherited only a single copy of an abnormal antithrombin (AT) gene. Homozygously affected individuals, although rare, have a severe thrombotic history of early onset and often affecting the arteries. We report two new cases of type II HBS (heparin binding site) deficiency in which the propositi are homozygous for the previously reported mutation 99 Leu to Phe, and who have a severe thrombotic history. These cases are considered alongside existing homozygote and compound heterozygote cases.     

Multichannel cochlear implantation with inner ear malformation: case report of performance and management

A small percentage of children who have received the Nucleus multichannel cochlear implant have cochlear malformations of the inner ear and consequent partial electrode insertions. This case describes one child with a cochlear "common cavity" who received an implant at a young age. The case has been further complicated by a gradual malfunction of the electrode array, although the device has not failed completely. Despite the increase in the number of nonfunctional electrodes over time, the subject has continued to improve in auditory, speech production, and language development over a 30-month period. Methods are discussed for clinical monitoring of both performance and the stability of electrodes over time. The circumstances of this case reinforce the importance of postimplant collaboration with families and school staff and highlight the need for objective measures to evaluate both longitudinal changes in performance and device integrity.     

Foreign bodies in the ear; report of 40 cases

BACKGROUND: Foreign bodies in the external ear canal present a frequent situation in pediatric practice. It is generally benign, but infectious complications may occur. POPULATION: Between January 1996 and March 1997, 35 children with a foreign body in the ear canal were treated in the ENT department of Robert-Debré hospital. RESULTS: Age ranged from 15 months to 14 years, with a mean of 6.5 years. The sex ratio was 1.2 (19 boys/16 girls). Five children had bilateral foreign bodies. The most frequent foreign bodies were pearls (8/40) and cockroaches (7/40). Eleven children had complications: ear canal laceration in five cases, otitis externa in five cases, cervical adenitis in three cases, general septic syndrome with fever in one case, and drum perforation in one case. CONCLUSION: The methods of extraction and the management of the complications are discussed.     

Werdnig-Hoffmann disease: report of two cases?]

Two cases of familial spinal muscular atrophy with onset in infancy are reported. The author makes an attempt to categorize them and concludes by Werdnig-Hoffmann disease.     

Dissecting the frog inner ear with Gaussian noise. II. Temperature dependence of inner ear function

Wiener kernel analysis was used to characterize the auditory pathway from tympanic membrane to single primary auditory nerve fibers in the European edible frog, Rana esculenta. Nerve fiber signals were recorded in response to white Gaussian noise. By cross-correlating the noise stimulus and the nerve fiber response, we computed (1) the full second-order Wiener kernel, and (2) the diagonals of the zeroth- to fourth-order Wiener kernels. These diagonals are usually referred to as polynomial correlation functions. The measured Wiener kernels were fitted with a 'sandwich' model. A new fitting procedure was used to compute the response characteristics of (1) the first filter, (2) the static nonlinearity, and (3) the second filter, which form the functional components of the model. The first filter is a bandpass filter. In the majority of low frequency fibers, with best excitatory frequency (BEF) < 800 Hz, this filter was tuned to two frequencies. This dual tuning mechanism gives rise to 'off-diagonal' components in the second-order Wiener kernel. The static nonlinearity resembles a rectifier, and is dominated by second-order (quadratic) nonlinearity. As a function of BEF, the shape of the nonlinearity changes systematically. Finally, the last filter in the model was a low pass filter. Across fibers, its cutoff frequency f-3dB ranged from 106 to 434 Hz.     

Laser applications in inner ear surgery

Argon and carbon dioxide lasers can cause heat shrinkage of the membranous labyrinth without injury to hearing. The argon laser can penetrate the membranous labyrinth and cause destruction of targeted vestibula special sensory epithelium also without injury to hearing. Consequently, these lasers have been used successfully in two treatment techniques for benign paroxysmal positional vertigo, which are discussed in detail. The early techniques for laser assisted resection of the pars superior portion of the inner ear with hearing preservation are discussed.     

Misdiagnosis of hearing loss due to ear canal collapse: a report of two cases

BACKGROUND: In the last 15 years, family physicians and general internists have adopted flexible fiberoptic endoscopy as a procedure to screen patients at risk of premature death from colorectal cancer. There has been controversy regarding the ability of non-fellowship-trained primary care physicians to extend this experience to full colonoscopy. METHODS: The results of 1048 consecutive colonoscopy examinations performed by a family physician over a 9-year period were tabulated. Outcomes measured included the reach-the-cecum rate (RCR), use of medication, complication rate, and diagnostic yield. In a convenience sample of 110 cases, the effectiveness of the non-narcotic analgesic ketorolac was assessed by the RCR. Outcomes of cases in which ketorolac was used were compared with cases in which traditional sedation and analgesia were used. RESULT: A high diagnostic yield without significant complications was noted. The RCR for nonmedicated patients was 36%. Among all medicated cases, the RCR was 93%. In patients who were given the non-narcotic analgesic ketorolac, the RCR was 96%, compared with 95% in patients not given ketorolac. CONCLUSIONS: A family physician in rural practice was able to attain and sustain a state-of-the-art, reach-the-cecum rate over a 9-year period. This service resulted in a high diagnostic yield, high degree of safety, and satisfactory results for the community. Ketorolac is an effective alternative for patients who may be hypersensitive to narcotic analgesia/sedation.     

Functional characteristics of the inner voice and the inner ear: Single or double agency?

Double agency theories of short-term memory posit the functional independence of a phonological store (inner ear) and articulatory process (inner voice). A series of 5 experiments challenges this view. Articulatory suppression during retention of 9-item lists gives rise to a changing-state effect similar to that shown for irrelevant speech. Also, vocalized suppression is more disruptive than silently mouthed suppression, but this difference arises from vocalization itself rather than from any auditory feedback to which it gives rise. Class similarity between the to-be-remembered items and the articulatory material is not a critical determinant, but the effect occurs only with tests of serial order. For mouthed suppression, the irrelevant speech effect is only attenuated with changing-state suppression. Also, the presence of changing-state irrelevant speech abolishes the changing-state effect of articulatory suppression. Functional equivalence of codes from auditory, visual, and articulatory sources is claimed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Interleukin 8 can affect inner ear function

The chemokine interleukin 8 (IL-8) was instilled into the round window niche of rats through a small perforation in the tympanic membrane in order to study its effect on inner ear function by electrophysiological and morphological techniques. The frequency-specific auditory brainstem response (ABR) was recorded at the frequencies 4, 8, 10, 12, 16 and 20 kHz just before and 1, 2, 5 and 14 days after instilling IL-8 to ascertain the hearing level during each interval. Morphological examination by light microscopy was performed during the same interval following the instillation of IL-8. On day 1, the rise in ABR threshold was within 5 dB SPL (non-significant elevation). However, a significant threshold elevation (above 5 dB SPL) occurred in high-frequency areas (16 and 20 kHz) on day 2, and in middle frequency areas (10 and 12 kHz) on day 5 with sensorineural hearing loss type intensity-latency curves. By day 14, the elevated thresholds had returned to pre-instillation levels. In the lowest areas (4 and 8 kHz), no significant threshold elevation was detected at any time during the observation period. By light microscopy, on day 1, clusters of inflammatory cells (predominantly neutrophils) were observed just outside the round window membrane (RWM), while only a few neutrophils were detected in the cochlea. These cells were still present outside the RWM on day 2. The neutrophils had disappeared by day 5 and only macrophages were present on the middle ear side of the RWM. However, throughout the observation period, the organ of Corti and stria vascularis appeared to be intact. These results suggest that IL-8 in the middle ear cavity is able to influence inner ear function.     

Extracellular nucleotide signaling in the inner ear

Extracellular nucleotides, particularly adenosine 5'-triphosphate (ATP), act as signaling molecules in the inner ear. Roles as neurotransmitters, neuromodulators, and as autocrine or paracrine humoral factors are evident. The diversity of the signaling pathways for nucleotides, which include a variety of ATP-gated ion channels (assembled from different subtypes of P2X-receptor subunit) and also different subtypes of G protein-coupled nucleotide receptors (P2Y receptors) supports a major physiological role for ATP in the regulation of hearing and balance. Almost invariably both P2X and P2Y receptor expression is apparent in the complex tissue structures associated with the inner-ear labyrinth. However P2X-receptor expression, commonly associated with fast neurotransmission, is apparent not only with the cochlear and vestibular primary afferent neurons, but also appears to mediate humoral signaling via ATP-gated ion channel localization to the endolymphatic surface of the cochlear sensory epithelium (organ of Corti). This is the site of the sound-transduction process and recent data, including both electrophysiological, imaging, and immunocytochemistry, has shown that the ATP-gated ion channels are colocalized here with the mechano-electrical transduction channels of the cochlear hair cells. In contrast to this direct action of extracellular ATP on the sound-transduction process, an indirect effect is apparent via P2Y-receptor expression, prevalent on the marginal cells of the stria vascularis, a tissue that generates the standing ionic and electrical gradients across the cochlear partition. The site of generation of these gradients, including the dark-cell epithelium of the vestibular labyrinth, may be under autocrine or paracrine regulation mediated by P2Y receptors sensitive to both purines (ATP) and pyrimidines such as UTP. There is also emerging evidence that the nucleoside adenosine, formed as a breakdown product of ATP by the action of ectonucleotidases and acting via P1 receptors, is also physiologically significant in the inner ear. P1-receptor expression (including A1, A2, and A3 subtypes) appear to have roles associated with stress, acting alongside P2Y receptors to enhance cochlear blood flow and to protect against the action of free radicals and to modulate the activity of membrane conductances. Given the positioning of a diverse range of purinergic-signaling pathways within the inner ear, elevations of nucleotides and nucleosides are clearly positioned to affect hearing and balance. Recent data clearly supports endogenous ATP- and adenosine-mediated changes in sensory transduction via a regulation of the electrochemical gradients in the cochlea, alterations in the active and passive mechanical properties of the cells of the sensory epithelium, effects on primary afferent neurons, and control of the blood supply. The field now awaits conclusive evidence linking a physiologically-induced modulation of extracellular nucleotide and nucleoside levels to altered inner ear function.     

Relapsing polychondritis presented as inner ear involvement

A patient with Buerger's disease is described in whom reconstruction of a gangrenous forefoot with a free flap has been successfully performed. In order to limit the amputation level, a more aggressive approach toward reconstruction in young patients seems appropriate. To our knowledge, this is the first described case of free flap reconstruction of a gangrenous defect in a Buerger patient.     

Histologic demonstration of glycosaminoglycans in inner ear fluids

We have shown previously that rat pancreatic periacinar fibroblastoid cells (PFCs) can be cultured from isolated pancreatic acini. In the present study, immunocytochemical examination of the PFC extracellular matrix was performed using antibodies against prolyl hydroxylase alpha and beta subunits, types I, III, and IV collagen, fibronectin, and laminin. The PFC content of alpha-smooth muscle actin and platelet-derived growth factor (PDGF) receptor were studied by immunoblotting. We demonstrated that PFCs synthesized extracellular matrix and expressed alpha-smooth muscle actin and PDGF receptors. These results suggested that PFCs resemble myofibroblasts and may play a critical role in pancreatic fibrosis. Conversely, pancreatic-type phospholipase A2 (P-PLA2), one of the pancreatic digestive enzymes, has been shown to induce DNA synthesis of Swiss 3T3 fibroblasts. To determine whether this enzyme is involved in pancreatic fibrosis, we studied P-PLA2's proliferative and chemotactic effects on PFCs as well as its digestive activity. The proliferative and chemotactic effects were investigated using 3H-thymidine incorporation and a chemotactic assay, respectively. P-PLA2 had both proliferative and chemotactic effects. P-PLA2 is considered a growth factor for PFCs and is implicated in pancreatic fibrosis.     

Addison's disease in pregnancy: a report of six cases

OBJECTIVE: To review six cases of pregnancy with Addison's disease at Obstetrics and Gynecology Hospital, Shanghai Medical University from 1949 to 1994. METHOD: This is a clinical retrospective analysis. Five of the 6 patients were treated with hormone replacement therapy, and the remaining one received no hormone treatment due to lack of symptoms during pregnancy and neglect of previous adrenal surgery by obstetricians. RESULTS: The patient received no treatment occurred Addisonian crisis and died soon after delivery. The other five patients had smooth course of delivery and postpartum period. CONCLUSIONS: Hormone replacement therapy is important for patients with Addison's disease in pregnancy especially for acute decompensation of adrenal function. Pregnant women with history of adrenal surgery should be monitored carefully even without any symptoms and hormone treatment is necessary. Vaginal delivery is encouraged unless there is obstetric indication.     

Removal and processing inner ear specimens for morphological research on guinea pig ear

Seventy (70) dissections of temporal bones of grown-up guinea pigs were done by the AA. in order to get a suitable approach to obtain inner ear studies, aiming at a morphological investigation.     

Cochlear implantation in children with inner ear malformations

We performed a case study and intervention study, with follow-up of 1 to 5 years, in 4 children with inner ear malformations who underwent implantation of a multichannel cochlear implant (Nucleus, Cochlear Corporation) at ages 3 to 12 years. Malformations included a common cavity deformity, 2 incomplete partitions, and 1 case of isolated bilateral vestibular aqueduct enlargement. One child had a single-channel implant placed at 3 years of age, and this was exchanged for a 22-channel implant at age 9. One child had her implant placed at age 4.5 years, but due to complications from a cerebrospinal fluid (CSF) leak had the initial implant removed and replaced at age 5 years during repair of the CSF leak. Intraoperative findings included a CSF leak at the time of surgery in 3 patients. One patient contracted bacterial meningitis 7 months postimplantation that was thought to be secondary to acute otitis media in the unoperated ear. Bilateral CSF leaks were noted in the middle ear by a lumbar puncture radionuclide and fluorescein dye study. Successful repair of the CSF leaks and reimplantation of the cochlear implant was carried out in this patient. Mapping and programming of the implant was found to be challenging in each of these patients. All patients demonstrated improved performance after implantation. Two patients demonstrated some open-set speech perception. One patient demonstrates improved use of temporal cues in a structured closed set. One patient has achieved no significant speech recognition at this time, but does have improved sound detection and awareness. Cochlear implantation in children with congenital inner ear abnormalities can be a successful method of rehabilitation. It should be recognized that the postoperative speech perception results may be highly variable among patients, and that intraoperative complications may occur.     

Surgery of inner ear diseases (author's transl)

In the treatment of Menière's disease, surgical procedures are available when conservative measures have failed. The most distressing symptom of vertigo can frequently be arrested, tinnitus reduced and hearing improved. Sudden deafness due to round window rupture is treatable. The most important disorder with labyrinthe symptoms is the acoustic neuroma, the smaller ones of which can be operated on by otologists in collaboration with neurosurgeons using the transtemporal or translabyrinthine approach with preservation of the facial nerve.     

Conditional immortalization of hair cells from the inner ear

The aim of this work was to culture conditionally immortalized cells that possess the potential to differentiate into mechanosensory hair cells. Utricular epithelia at embryonic stage E16 were cultured from the vestibular system of the H2kbtsA58 transgenic mouse (Immortomouse) that carries a conditionally expressed immortalizing gene derived from the simian virus 40. Immunolabelling showed that the immortalizing transgene product, the T antigen (Tag), was expressed in utricular cells under permissive conditions and that it was inactivated under non-permissive conditions. Several morphologically distinct cell types proliferated when Tag was expressed, including those that resembled fibroblasts, nerve cells and epithelial cells. Mixed cultures of cells from the utricle, passaged up to 50 times every 3-4 days over a period of 5 months, were subsequently allowed to differentiate for 10 days by transferring them to non-permissive conditions. Monoclonal antibody markers were used to locate expression of hair cell specific antigens. One antibody that normally labels stereociliary bundles from postnatal stage P4-6 labelled cellular projections from a population of spheroid cells that were distributed across the culture surface. A second antibody that normally labels stereociliary bundles did not label the same structures. We conclude that utricular hair cell progenitors can be derived from the H2kbtsA58 transgenic mouse but that under the experimental conditions used they do not follow the normal pattern of differentiation.     

Otogelin: a glycoprotein specific to the acellular membranes of the inner ear

Efforts to identify the specific components of the mammalian inner ear have been hampered by the small number of neuroepithelial cells and the variety of supporting cells. To circumvent these difficulties, we used a PCR-based subtractive method on cDNA from 2-day-old mouse cochlea. A cDNA encoding a predicted 2910-amino acid protein related to mucin has been isolated. Several lines of evidence indicate, however, that this protein does not undergo the O-glycosylation characteristic to mucins. As confirmed by immunocytochemistry and biochemical experiments, this protein is specific to the inner ear. Immunohistofluorescence labeling showed that this protein is a component of all the acellular membranes of the inner ear: i.e., the tectorial membrane of the cochlea, the otoconial and accessory membranes of the utricule and saccule, the cupula of the semicircular canals, and a previously undescribed acellular material covering the otoconia of the saccule. The protein has been named otogelin with reference to its localization. A variety of nonsensory cells located underneath these membranes could be identified as synthesizing otogelin. Finally, this study revealed a maturation process of the tectorial membrane, as evidenced by the progressive organization of otogelin labeling into thick and spaced radial fiber-like structures.     

Bacterial flora in chronic inner ear infections in adults

The human genome carries multiple copies of sequences related to endogenous retroviral DNA. We report here the distribution of a new multicopy long terminal repeat (LTR) sequence that has been a part of an endogenous retrovirus-like sequence RTVL-H2. Twenty-four human chromosomes were either separated by flow sorting or by using rodent cells carrying a single human chromosome, and the DNA was subjected to Southern analyses using the RTVL-H2 DNA as a probe. The RTVL-H2 LTRs were distributed among all the human chromosomes, but the density and the profile differed from chromosome to chromosome. The same chromosome obtained from different individuals showed essentially the same chromosome-specific patterns. The distribution of the RTVL-H2 LTRs among different chromosomes did not correlate with the distribution of LTRs from another endogenous retroviral DNA, HERV-A, strongly suggesting that there is no preferred chromosome or a region thereof, for the integration. The possibility of rearrangement or amplification after integration is discussed.