Molecular markers of putative spermatogonial stem cells in the domestic cat.

Spermatogonial stem cells (SSCs) are an important tool for fertility preservation and species conservation. The ability to expand SSCs by in vitro culture is a crucial premise for their use in assisted reproduction. Because SSCs represent a small proportion of the germ cells in the adult testis, culture success is aided by pre-enrichment through sorting techniques based on cell surface-specific markers. Given the importance of the domestic cat as a model for conservation of endangered wild felids, herein we sought to examine culture conditions as well as molecular markers for cat SSCs. Using a cell culture medium for mouse SSCs supplemented with glial cell-derived neurotrophic factor (GDNF), germ cells from prepuberal cat testes remained viable in culture for up to 43 days. Immunohistochemistry for promyelocytic leukaemia zinc finger (PLZF) protein on foetal, prepuberal and adult testis sections revealed a pattern of expression consistent with the labelling of undifferentiated spermatogonia. Fluorescence-activated cell sorting (FACS) with an antibody against epithelial cell adhesion molecule (EPCAM) was used to sort live cells. Then, the gene expression profile of EPCAM-sorted cells was investigated through RT-qPCR. Notably, EPCAM (+) cells expressed relatively high levels of CKIT (CD117), a surface protein typically expressed in differentiating germ cells but not SSCs. Conversely, EPCAM (-) cells expressed relatively high levels of POU domain class 5 transcription factor 1 (POU1F5 or OCT4), clearly a germ line stem cell marker. These results suggest that cat SSCs would probably be found within the population of EPCAM (-) cells. Future studies should identify additional surface markers that alone or in combination can be used to further enrich SSCs from cat germ cells.

[Analysis of the cochlear implant as a treatment technique for profound hearing loss in pre and postlocutive patients]. / Evaluación del implante coclear como técnica de tratamiento de la hipoacusia profunda en pacientes pre y post locutivos.

INTRODUCTION: These are the objectives planned for this study: 1. Evaluate the results from the communication point of view. 2. Evaluate the cochlear implant (CI) impact on the quality of life. 3. Evaluate medical complications and technical failures. 4. Assess direct and indirect costs generated during the phases of a cochlear implantation programme. 5. Determine which factors have a high impact on the clinical evolution and the financial cost. MATERIALS AND METHOD: A population of 877 patients, postlingual and prelingual, adults and children, have been studied. They were treated in 5 Spanish centres with cochlear implant programmes. Audiometric tests and global questionnaires on life quality have been carried out. Medical and CI technology complications have also been computed. Direct and indirect economic costs of a cochlear implant have been calculated. RESULTS: Postlocutive-implanted patients reached the 40 dB SPL threshold in the Pure Tone Audiometry, and this result was maintained during the 12-year evolution. In Vowels test, it evolved from a 30% on pre-stimulation to 80-90%, in Disyllables words test it evolved from a 10% to a 50-60%, and in CID Sentences test it evolved from an 18% to a 60-70%. In the prelocutive population, results were influenced by the child's age at implantation. The best results were obtained by the children who had been implanted earlier. Those implanted between 0 and 3 years old evolved in the Vowels test from 0% during pre-stimulation to 95%, from a 0% to a 90% in Disyllables words test and from a 0% to a 90-95% in CID Sentences test. Also, the speech acquisition and development of the pre-locutive population was also influenced by the implantation age. An 80% of postlocutive adult patients stated a mood and sociability improvement after the cochlear implantation. They did not show health changes in general nor relevant modifications in the attention they usually received from relatives and friends. Severe medical-surgical complications were registered for a 3.42% of the cases, a 7.06% of mild medical-surgical complications and a 3.07% of technical breakdowns in the internal components of the CI. Financial cost of implantation for a post-locutive adult oscillated between 36,912 Euro and 37,048 Euro, and between 37,689 Euro and 44,273 Euro for a pre-locutive child. CONCLUSIONS: Cochlear implants clearly enhance communication skills of the implantees. Results obtained for the prelocutive implanted population justify the creation of hearing screening programmes in new-borns. Postlocutive implanted adults have expressed satisfaction for the results obtained. However, they did perceive some limitations in situations of unfavourable acoustic conditions. An analysis of direct and indirect costs related to a CI programme has been made. It may be useful to carry out reports on the cost-benefit ratio in this field. The low index of complications observed shows which cochlear implant treatment technique complies with the adequate safety margins. The factors influencing the most in the evolution are: duration of hearing deprivation, age at implantation, cochlear anatomy and functionality of the auditory pathway, patient's and relative's motivation, and the coexistence of other handicaps associated to hearing losses.

Advantages of binaural hearing provided through bimodal stimulation via a cochlear implant and a conventional hearing aid: a 6-month comparative study.

CONCLUSIONS: Our study data demonstrate the additional benefit derived from continued use of a contralateral hearing aid (HA) post-cochlear implantation for speech recognition ability in quiet and in noise. Postoperative bimodal stimulation is recommended for all subjects who show some speech recognition ability in the contralateral ear as it may offer binaural listening advantages in various listening situations encountered in everyday life. OBJECTIVES: To assess the benefits derived from bimodal stimulation for experienced HA users implanted with a cochlear implant (CI) (score=20% in disyllabic test). The correlation between pre- and postoperative performance on speech perception measures was examined to determine additional criteria for recommending bimodal stimulation postoperatively. MATERIAL AND METHODS: A within-subject repeated-measures design was used, with each subject acting as their own control. Assessments were carried out preoperatively in aided monaural and best-aided conditions and at 6 months postoperatively in CI-alone, contralateral HA-alone and bimodal listening conditions. Speech recognition using Spanish words and sentences materials was assessed at conversational level and for soft speech in quiet. Speech comprehension in noise was assessed using word materials at a signal:noise ratio of +10, for coincident speech in noise and for spatially separated speech in noise. Twelve adult native Spanish subjects with a severe-to-profound hearing impairment who were experienced with optimally fitted conventional amplification and who displayed suboptimal speech understanding preoperatively were enrolled in the study. Preoperatively, conventional amplification was worn by five subjects binaurally and by seven monaurally. RESULTS: Postoperatively, superior speech recognition ability in quiet and in noise for disyllabic words was achieved using bimodal stimulation in comparison to performance for either monaural aided condition. Mean improvement in speech recognition in the bimodal condition was significant over performance in the CI-alone condition for disyllabic words in quiet at 70 (p=0.006) and 55 dB SPL (p=0.028), for disyllabic words in noise at +10 dB with speech and noise spatially separated with the noise source closest to the contralateral HA (S0NHA) (p=0.0005) and when the noise source was closest to the CI ear (S0NCI) (p=0.002). When testing word recognition in noise with speech and noise sources coincident in space, word scores were superior in the bimodal condition relative to the CI-alone condition but this improvement was not significant (p=0.07). The advantages of bimodal stimulation included significant effects of binaural summation in quiet and significant binaural squelch effects in both the S0NHA and S0NCI test conditions. All subjects showed superior performance in the binaural situation postoperatively relative to the best-aided condition preoperatively for one or more test situations.

A purine-related metabolite negatively regulates fixNOQP expression in Sinorhizobium meliloti by modulation of fixK expression.

5-aminoimidazole-4-carboxamide nucleotide (AICAR) is a negative effector of cytochrome terminal oxidase cbb3 production in Rhizobium etli. In this work, the effect of AICAriboside (AICAr), the precursor of AICAR on the expression of the Sinorhizobium meliloti fixNOQP operon encoding the symbiotic terminal oxidase cbb3, was analyzed. AICAr reduced the microaerobic induction levels of fixN-lacZ and fixT-lacZ gene fusions 18- and seven-fold respectively, and both genes were activated by the transcriptional activator FixK. A fixK-lacZ fusion presented 14-fold-reduced induction levels in microaerobic cell cultures in the presence of AICAr. AICAr also reduced three-fold the microaerobic expression levels of the nifA-lacZ fusion, whose expression as well as that of fixK is controlled by the two-component system FixL-FixJ. In contrast, AICAr had no effect on the expression levels of a hemA-lacZ fusion. These data suggest that AICAr prevents fixNOQP induction by the inhibition of fixK transcription.

Expression pattern of Rhizobium etli ccmIEFH genes involved in c-type cytochrome maturation.

In different bacterial species, ccmIEFH genes have been suggested to code for subunits of a bacterial haem-lyase catalyzing the covalent attachment of haem to c-type apoproteins. In Rhizobium etli CE3 there are two copies of ccmIEFH: one in the chromosome and the other located in plasmid pf. However, the null phenotype of chromosomal ccmF mutant indicates that the gene locus of plasmid pf is not functional. Two ccmI chromosomal mutants, previously isolated, produced detectable levels of c-type cytochromes under certain culture conditions in contrast with the ccmF mutant, suggesting that ccmF could be transcribed independently. The transcriptional organization of ccmIEFH operon was established. Two promoters from the chromosomal locus were mapped by primer extension, one located upstream of ccmI and the second located upstream of ccmF. The regulation of the expression of both promoters was studied using appropriate lacZ gene fusions (ccmI-lacZ and ccmEF-lacZ). The ccmI-lacZ gene fusion was expressed in complex medium, during exponential growth, under microaerobic conditions and in a R. etli mutant that accumulates reducing power, conditions where a higher respiration rate could be limited by c-type cytochrome content. The ccmEF-lacZ fusion was also primarily expressed in complex medium and under microaerophilic conditions. The finding of two independent promoters in this gene locus could suggest that the step catalyzed by CcmFH could be a rate-limiting step for c-type cytochrome assembly under certain culture conditions.

Enhanced nitrogen fixation in a rhizobium etli ntrC mutant that overproduces the bradyrhizobium japonicum symbiotic terminal oxidase cbb3

The ntrC gene codes for a transcriptional activator protein that modulates gene expression in response to nitrogen. The cytochrome production pattern of a Rhizobium etli ntrC mutant (CFN2012) was studied. CO difference spectral analysis of membranes showed that CFN2012 produced a terminal oxidase similar to the symbiotic terminal oxidase of bacteroids in free-living cells under aerobic conditions, with a characteristic trough at 553 nm. CFN2012 produced two c-type cytochromes with molecular masses of 27 and 32 kDa, in contrast with the wild-type strain, which produced only a 32-kDa c-type cytochrome. The expression levels of the R. etli fixNOQP operon, which codes for terminal oxidase cbb3, were not affected by the ntrC mutation. However, the production levels of the two c-type cytochromes (27 and 32 kDa) were enhanced at least eightfold when the Bradyrhizobium japonicum fixNOQP operon was expressed in CFN2012 from the nptII promoter (pMSfixc), suggesting that these proteins are subunits FixO (27 kDa) and FixP (32 kDa) of cbb3 and that CFN2012/pMSfixc overproduced this terminal oxidase. CFN2012/pMSfixc showed a significant increase in its symbiotic performance as judged by the determination of nitrogenase activities of plants inoculated with this strain, suggesting that the overproduction of cbb3 terminal oxidase correlates with an enhancement in symbiotic nitrogen fixation.

Immunohistochemical expression of Bcl-2 oncoprotein in EBV-associated nasopharyngeal carcinoma correlated to histological type and survival.

Expression of Bcl-2 is associated with inhibition of apoptosis and extension of cell survival. In vitro Bcl-2 protein expression is up-regulated by the EBV-latency associated antigen latent membrane protein (LMP-1). We have investigated the relationship between the presence of EBV-DNA screened by means of sensitive nested-PCR, nasopharyngeal carcinoma (NPC) histological types according to two different schemata (WHO and Micheau classifications) and Bcl-2-124 immunohistochemical expression in 55 biopsy samples of NPC. EBV genome was detected in 100% of samples with sufficient DNA quality to support the previous view that all types of NPC are variants of EBV-infected neoplasia. Bcl-2 was observed in the basal layer of normal nasopharyngeal mucosa and also at cytoplasmic level in 42 of 55 (76.4%) NPC cases. Mitotic neoplastic cells usually showed strong cytoplasmic and chromosomal staining, a finding not well referred to previously. Bcl-2 expression was significantly associated (p<0.05) to undifferentiated NPC (UNPC) when a histological classification with only two major microscopical types was applied. No close correlations were found between the presence of EBV-DNA, NPC location, clinical stage and age or sex of the patients in relation to Bcl-2 positive expression. However, when comparing Bcl-2 expression and known survival mean of the patients, significant differences were observed (p<0.001) so that mean survivals were 31.1, 24.4, 52.2 and 54.1 months respectively for NPC patients with -, +, ++ and Bcl-2 immunoreactivity. Nevertheless this better clinical outcome in Bcl-2 NPC positive cases may depend on the histological type due to close relationship with UNPC. Only studies of larger series with long-term follow-up and multivariate analyses may document whether Bcl-2 expression is an independent prognostic marker in the evolution of NPC patients.

Modification of Christensen urease test as an inexpensive tool for detection of Helicobacter pylori.

About half the world population is infected with Helicobacter pylori. Most live in developing countries where clinical studies face the constraints of high costs of imported rapid diagnostic tests. In this work, we describe and validate a simple local urease test (LUT) to determine the presence of the bacterium in gastric biopsies, and report the incidence of infection among symptomatic patients in Caracas, Venezuela. Statistical comparison of LUT and CLOtest (Delta West, Bentley, Australia) (N = 216 patients) showed that the probability of 95% agreement between the two test was 0.936. Overall incidence of infection determined by the LUT was 65% (N = 229), and it was higher in patients from public (72%; N = 153) than from private (50%; N = 76) hospitals (p = .001). Therefore, the incidence of infection differs in two socioeconomic groups that coexist in the same city. LUT may represent an affordable tool in clinical studies needed to identify social factors that increase the risk of infection by H. pylori.

Isolation of Sinorhizobium meliloti Tn5 mutants with altered cytochrome terminal oxidase expression and improved symbiotic performance.

The relationship between whole-cell redox potential, cytochrome composition in free-living culture and symbiotic activity of Sinorhizobium meliloti was studied. Three Tn5-induced mutants with increased cellular redox potential were generated. Stationary cultures of mutants Tb9 and Tb16 in contrast to the parental strain produced the b-type terminal oxidase that may be similar to the symbiotically essential cytochrome oxidase cbb3 of Bradyrhizobium japonicum. Increase in the symbiotic effectiveness of all three mutants and in O2 consumption rate in free-living cultures was observed. Mutants Tb1 and TB16 were also characterized by an increase in fixNOQP gene expression. Consequently, the mutations probably affect at least two different steps of rhizobial respiratory metabolism operating both in free-living cells and endosymbiotic forms.

Comparative analysis of Epstein-Barr virus (EBV) detection by nested-PCR and non-isotopic in situ hybridization in nasopharyngeal carcinoma (NPC).

The aims of this study were two-fold: first, to assess the relative diagnostic performance of non-isotopic in situ hybridization (ISH) and the nested polymerase chain reaction (nested-PCR) applied to Epstein-Barr virus (EBV) detection in a series of 55 unselected nasopharyngeal carcinoma (NPC) cases and, secondly, to correlate these data with histopathological classification. Our study shows that in 76.36% of NPC cases positive nuclear signals were observed using EBV-ISH. Overall, EBV-ISH positivity varied according to histological type, in that undifferentiated carcinomas showed a higher proportion of positive cases than differentiated cell carcinomas, although ISH results do not show significant differences in relation to histological types when employing two different schemes (WHO and Micheau). However, in adequate quality DNA samples (54 NPC cases), EBV-DNA was detected in 100% of cases using a nested-PCR, supporting the previous view that all histological types of NPC are in reality variants of EBV-infected neoplasia. ISH-negative cases probably reflect a lower sensitivity than PCR, particularly when a small number of viral copies are present, as well as a variable technical effectiveness for detected EBV, independent of the NPC histological type.

Morphometric study of tumor angiogenesis as a new prognostic factor in nasopharyngeal carcinoma patients.

The aim of this study was to determine the possible prognostic significance of tumor angiogenesis (TA) in nasopharyngeal carcinoma (NPC) patients. Fifty-five NPC patients were evaluated in relation to survival. Endothelial cells were immunohistochemically stained with anti-von Willebrand factor (F-VIII), CD-31 and CD-34 antibodies, and microvessels counted in the most active areas of tumor neovascularization or hotspotsusing both a manual and an automatic method. Overall survival analysis calculated by the Kaplan Meiertest revealed that both methods were correlated with a statistical significance between intratumoral microvessel density (IMD) and overall survival, using either manual (p=0.0141) or automatic counting (p=0.0117). Other angiogenic parameters studied were perimeter, roundness and accumulative area of the microvessels using a morphometric analyzer. Moreover, our results show that cases with high IMD demonstrated a prognostic significance in relation to the accumulative area (p=0.0072).

Expression of p53 protein and tumor angiogenesis as prognostic factors in nasopharyngeal carcinoma patients.

The objective of this study was to evaluate the possible prognostic significance of p53 protein overexpression and tumor angiogenesis (TA) in nasopharyngeal carcinoma (NPC) patients, together with other clinicopathological variables. Forty-two NPC patients were evaluated in relation to survival. Nuclear p53 overexpression in neoplastic and endothelial cells was detected by immunohistochemistry (IHC) with the monoclonal antibody DO-7 and the polyclonal antibody against factor VIII-related antigen, respectively. Thereafter, we evaluated p53 cases in order to determine their nuclear immunoreactivity from negative (-) to positive (+, ++, +++). In addition, microvessels were counted in the most active areas of tumor neovascularization or hotspots using an image computer analyzer (MicroImage). A Cox multiple regression survival analysis was used to determine the best prognostic indicators in NPC patients. As a result, tumor microvessel count, considered as a continuous variable, was the most important independent prognostic indicator in relation to survival (p = 0.0273), with a relative risk of death of 2,4399 [95% confidence interval = 1.1051 ; 5.3871] associated with the highest microvessel counts. Moreover, the only clinicopathological variable that demonstrated prognostic value in a Cox multiple regression survival analysis was histological type (p = 0.05). In addition, we did not observe any statistical association between intratumoral microvessel density (IMD), clinicopathological variables and p53 protein expression.

Spanish study group on cochlear implants for persons with marginal benefit from acoustic amplification.

Recently the U.S. Food and Drug Administration approved the use of cochlear implants (CI) in patients with severe-to-profound sensorineural hearing loss with marginal benefit (< 30% speech discrimination) from hearing aids (HA). A multicentre trial was developed to determine whether this approval could be applied to the Spanish population. Nine postlingual adults from 3 centres were selected for the study. The mean preoperative unaided PTA threshold (0.25 to 4 KHz) was 108.6 dB HL for the poorer ear and 99.8 dB HL for the better. In all subjects the poorer ear was implanted with a Mini Nucleus 22 device. A single subject design study was used to compare performance with HA preoperatively and with CI postoperatively, or CI with contralateral HA. PTA measures and Spanish open set speech recognition tests were used. Comparing pre-implant levels with results after 6 months of CI use, all subjects improved significantly on all test measures: i) Mean aided soundfield threshold: 73.6 dB vs 39.8 dB ii) Mean bisyllabic recognition: 20% vs 57% iii) Mean consonant discrimination: 20% vs 49% iv) Mean CID sentences: 22% vs 73%. Five subjects discontinued use of the contralateral HA after implantation, three continued occasionally to use the HA, and one continued to use both on a full-time basis. Patients performed significantly better post-implant on speech recognition compared with their best pre-implant HA scores. Therefore the CI appears an appropriate treatment for Spanish speakers with a severe-to-profound hearing loss and marginal benefits with HA.

[Medium and large acoustic neurinomas with normal tonal audiometry at the moment of diagnosis]. / Neurinomas acústicos medianos y grandes con audiometría tonal normal en el momento del diagnóstico.

INTRODUCTION: Early diagnosis of an acoustic neurinoma for any possible treatment is important, especially in view of the relationship between surgical morbidity and the size of the tumour. The commonest presenting symptom of these tumors is unilateral perceptive deafness. However, between 4 and 12% are diagnosed before deafness occurs, although they may be large in size. Therefore one should remember other symptoms which lead to suspicion of these tumors, and have diagnostic guidelines available to allow the earliest possible detection. PATIENTS AND METHODS: We report four cases of acoustic neurinoma or vestibular schwannoma of medium or large size in which audiological studies did not at first give rise to suspicion of the presence of this tumour, since audition was considered to be normal at conversation frequencies (500 to 3,000 Hertz) for the age of the patient or who had an audiometry curve with symmetrical deafness in both ears. CONCLUSION: We evaluated the diagnostic importance of the tests done and compared the results with data published in the literature.

Circulating auto-antibodies in Ménière's disease.

In the last years, it has been claimed that patients suffering from Menière's disease exhibit Circulating Immune Complexes, as well as free antibodies specifically directed against type II collagen, a component of the mammal inner ear. Furthermore, by means of repeated injections of type II collagen to the guinea-pig. Menière's disease has been reproduced. Taken together, all these features strongly suggest that Menière's disease fulfills most of the postulates that Witebsky established for the classification of auto-immune diseases. However, in spite of being a very well known marker of auto-immune conditions, non organ nor species specific Circulating Auto-Antibodies (CAA) have not been reported, to our knowledge, in Meniere's disease. Based upon this fact, Anti-Nuclear, Anti-Mitochondrial, Anti-Smooth Muscle, Anti-LKM and Anti-Parietal Gastric Cells Auto-Antibodies have been investigated in 49 patients diagnosed as having Meniere's disease according to the criteria of the AAOO. All of them were asymptomatic at the time of the study. Seventy eight serum samples from Normal Healthy Subjects (NHS) constituted the control group. CAA were tested by indirect immunofluorescence on rat organs according to the method described by Coons. Additionally, the seric levels of C3, and C4 as well as the haemolytic capacity (CH-50) of the serum samples were tested. Furthermore, Circulating Immune Complexes (CIC) as well as the Complement-Mediated Mechanisms involved in the clearance of CIC (Complement-Mediated Solubilization and Inhibition of the Immune Precipitation) were investigated in parallel.(ABSTRACT TRUNCATED AT 250 WORDS)

[Vascular anomalies presenting as middle ear mass]. / Anomalías vasculares que se presentan como masas del oído medio.

We study several cases of patients who presented clinical symptoms indicating the presence of a middle ear mass (tinnitus, hearing loss, otorrhea, otorrhagia...). We had to rule out the presence of an expanding tumour (either in the form of a cholesteatoma or a glomus tumor) and the existence of vascular anomalies of the middle ear (enlarged jugular bulb or jugular megabulb deformity). Due to the possibility of a different treatment in each particular case (radiotherapy, radical surgery, jugular vein ligation or even no treatment at all), the relative value of various imaging techniques and/or a possible surgical approach before arriving to a conclusive diagnosis, is discussed.

[Surgery of the cochlear implant: complications]. / Cirugía del implante coclear: complicaciones.

From 1991 to 1994 13 patients underwent cochlear implant in our Department. The surgical results and complications in this group of patients are reviewed. In all cases the cochlear implant was successful and no major complications were recorded. The most common problems was transient dizziness or imbalance. Other complications were minor facial nerve stimulation during the programming of the device, dehiscence of the suture in the tragal region, problems in the insertion of the electrodes in the scala tympani and a case that needed reimplantation due to technical problems with the device. We consider that a proper selection of cases and a precise surgical technique are essential in order to achieve the adaptation of the cochlear implant and avoid pitfalls.

[ENT emergencies in a tertiary hospital. Study of frequency and etiology]. / Urgencias O.R.L. en un hospital terciario: estudio de la frecuencia y etiología.

We have reviewed 19,061 emergencies attended in the ENT Department of our Hospital, over a period of 3 years. And analyzed the aetiologic distribution and the frequency of the cases, finding out the most frequent pathology were the external otitis followed by epistaxis. In the paper are also considered their relation with other parameters such as sex (no differences), age (25-35 years) and seriousness, this latter not being of gravity. Finally, we've studied the amount and degree of appropriteness of the visits attended and found out that near half of the cases could be attended in a less specializated centers.

[Osteoma of the middle ear]. / Osteoma del oído medio.

Osteomas of the middle ear are extremely rare. Those of the mastoid process being most frequent encountered als the tympanic sitting. Extracanalicular osteomas of the temporal bone are scarce. Bibliography about this particular subject account for 60 communications, only 9 with reference to the middle ear. The AA. report the case of an osteoma of the left incus in an 17-years-old boy, suffering a progressive deafness dating from 3 years term. The otoscopy showed a reddish mass protruding in the tympanum. TC and MNR provided evidence of a mass involving de auditory ossicula. Through an exploratory tympanotomy could be removed the anomaly. Histopathology make sure the diagnosis of osteoma. The AA. remark some clinical and surgical features of the piece compared with the other 10 cases reported in the literature reviewed.

[Deafness in patients with osteogenesis imperfecta]. / Estudio de la hipoacusia en los pacientes con osteogénesis imperfecta.

This study deals with 8 cases of progressive deafness in patients with abnormalities in growth and skeletal disease, suffering from Osteogenesis imperfecta (5 of them are a familial case). The work is both and audiological and radiological research of the temporal bone, done in order to uncover the reasons of the patient's deafness. This curses just like otosclerosis with a conductive component (fixation of the stapes) and a sensitive one (cochlear demineralization), starting up in adolescence as bilateral and symmetric process. Because of its progressive nature the AA, recommend as treatment the ear prostheses and refuse the surgery of the stapes.