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Introduction and objective: Trisomy 21 is the most frequent chromosomal alteration and, because of the increase in life expectancy, requires more specialized care. Ear, nose, and throat (ENT) manifestations are a dominant focus of morbidity. Hearing problems are more common when compared to the rest of the population and can impact educational and social development, affecting their quality of life. The aim of this study was to analyze the results of the otological and auditory follow-up of children with Down syndrome (DS), evaluated in the ENT Department of the Hospital Clínico Universitario de Valladolid (HCUV). Method: Prospective/retrospective longitudinal study of 83 children with DS who attended consultation for childhood hearing loss between 1993 and 2021. Results: We detected 66% of external ear malformations. Middle ear pathology was present in 93%. Hearing loss was found in 61%, being bilateral in 78%. Of those diagnosed, 87% were mild/moderate and 6% were profound. Mixed hearing loss was the most frequent type, followed by sensorineural hearing loss. ENT surgery was performed in 62%, with adenoidectomy being the most frequent. Discussion: ENT follow-up recommended for children with DS is a difficult task to organize, as it is necessary to develop specific planning to make it possible in our public health system. Conclusions: Given the frequency and important repercussions of hearing impairment in children with DS, early identification and indefinite follow-up is essential to try to promote their correct development. [ABSTRACT FROM AUTHOR]

Introduction and objectives: Idiopathic Sudden Sensorineural Hearing Loss (ISSNHL) is commonly defined as greater than 30 dB of hearing loss in at least 3 audiometric frequencies occurring over 3 days or less, of unknown cause. Since the effect of steroid therapy in this syndrome is currently under discussion, we will evaluate the efficacy of intratympanic corticosteroid (ITC) as rescue treatment. As a secondary objective, we will study the influence of certain factors considered risk or prognostic. Patients and methods: The results of two population groups with similar age and hearing loss are compared. The first of 99 patients received exclusively systemic treatment with corticosteroids. In the second group of 70, with ISSNHL refractory to general treatment, we instilled intratympanic corticosteroids. We compared the differences in mean hearing threshold at diagnosis and at 3 months, as well as the general degree of recovery. Results: Intratympanic application of 3 doses of dexamethasone (8mg/ml) in refractory ISSNHL did not produce, in our population, a significant change with respect to systemic treatment. We also analyzed the influence of selected prognostic factors. Discussion: We do not know the efficacy of corticosteroid treatment in idiopathic sudden deafness. The type of lesion and patient characteristics could be decisive in the final prognosis and therapeutic outcome. Conclusions: Rescue treatment with ITC has not provided significant hearing improvement in the population studied. The factors with the worst prognosis are the presence of vestibular symptoms, profound hearing loss, high-frequency hearing loss in audiometry, the beginning of treatment after 2 weeks and fundamentally, the lack of improvement of at least 10 dB on the 4th day of treatment. [ABSTRACT FROM AUTHOR]

BACKGROUND: Cochlear implantation has proven to be an effective treatment for severe bilateral sensorineural hearing loss. Common cavity is an inner ear malformation, a rare anomaly that occurs in 25% of all inner ear malformations, it is important to evaluate the severity of inner ear deformity and other associated anomalies during the preoperative assessment in order to identify any complication that may potentially occur during the surgery and subsequent patient management. CLINICAL CASE: A 4-year-old male patient underwent cochlear implantation due to a common cavity, operative findings included an abnormal facial nerve anatomy, had complete insertion, and have demonstrated improved performance after implantation. CONCLUSIONS: The cochlear implant is a safe surgery in most patients with this type of malformation. It is a surgical procedure with a high risk of Gusher, so we have to be prepared to deal with this type of event. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To measure auditive abilities in patients with cochlear implant. MATERIALS AND METHODS: A descriptive, retrospective, observational study was done from January 2013 to January 2018 with patients of National Institute of Pediatrics of Mexico submitted to cochlear implant. Auditory ability was evaluated through Meaningful Auditory Integration Scale (MAIS). RESULTS: There were inpatients a month after birth and in patients with known risk factors, to ensure that they will have it when they are discharged, to establish early diagnosis and determining the possible cause of sensorineural hearing loss.cluded 31 patients, 15 men and 16 women. Of the 31 patients, 8 had bilateral implants and 24 unilateral implant, patients with bilateral implant obtained a score of 88.7% (35.05/40); patients with unilateral cochlear implant obtained 83.2% (33.3/40), 3 cases obtained 29-31 points, meaning a limited auditory ability. CONCLUSIONES: In the population studied at the National Institute of Pediatrics who underwent cochlear implant, it is extremely important to carry out hearing screening in [ABSTRACT FROM AUTHOR]

OBJECTIVE: To identify the findings observed by magnetic resonance imaging of the posterior fossa in subjects with sudden sensorineural hearing loss and alteration of the auditory evoked potentials of the brainstem. MATERIALS AND METHODS: A cross-sectional, observational, analytical and retrospective study was done from April 2018 to February 2020, in which the studies of patients with sudden sensorineural hearing loss and altered brainstem auditory evoked potentials, who underwent posterior fossa magnetic resonance imaging, were reviewed in order to evaluate the imaging findings. RESULTS: One-hundred studies were analyzed, showing alterations in all of them. Of the structures of the inner ear evaluated, it was observed that in 93%, the semicircular canals were altered, in particular the decrease in diameter and signal intensity. It was also evidenced that the laterality of the affected semicircular canals has a strong and positive correlation with the laterality of hearing loss. CONCLUSIONS: The main alteration associated with the subjects with sudden sensorineural hearing loss was the decrease in diameter and signal intensity in the semicircular canals, and a close correlation was evidenced between the laterality of the hearing loss and the affection of semicircular canals. [ABSTRACT FROM AUTHOR]

BACKGROUND: Osseous labyrinth malformations constitute 20% of the causes of congenital sensorineural hearing loss, various characteristics have been used to classify them. However, most of the information of internal auditory canal anomalies reported are about stenosis or absence of the internal auditory canal. CLINICAL CASE: A 60-year old female patient with past medical history of left chronic otitis media with dehiscence of tegmen tympani in whim during study protocol a bilateral variant was reported in both internal auditory canals. CONCLUSIONS: Within cochlear anatomical variants it has been reported its enlargement, stenosis, and aplasia. In this study, we report the case of an anatomical variant presenting without sensorineural hearing loss, withstanding the clinical conduct in face of it. The cystic dilation of our patient could be associated with internal auditory canal diverticula in the context of idiopathic intracranial hypertension due to the size of the internal auditory canal in conjunction with the tympanic tegmen dehiscence. Further anatomic studies need to be done to report the prevalence in our population. [ABSTRACT FROM AUTHOR]

Auditory neuropathy is a type of deafness in Mexico that is not early diagnosed. Auditory neuropathy is the presence of otoacoustic emissions with absent or diminished auditory evoked potentials. That is, a functional cochlea with a nerve with poor function. México's health problem begins with a neonatal hearing screening that only includes otoacoustic emissions; therefore, the loss of nerve conduction of the auditory stimulus is not assessed. In this study, a literature review of auditory neuropathy in Mexico was carried out and the impact on the country's public health is analyzed. [ABSTRACT FROM AUTHOR]

Sensorineural hearing loss is the most frequent sensory deficit. It is usually not syndromic and has a genetic etiology. It is usually secondary to a Mendelian inheritance (autosomal dominant and recessive, and linked to the X chromosome). In general, the type and onset of hearing loss lead us to the dominant or recessive pattern. The dominant one is usually postlingual and progressive; while the autosomal recessive is prelingual, not progressive and severe to deep. The different autosomal dominant hearing loss are a group called DFNA, with different numbers depending on the subtype (genes) of hearing loss. In recessive cases, DFNB also with different numbers and in the same way the hearing loss linked to X as DFNX. With recent genetic tools, such as next-generation sequencing, for the identification of genes that cause hearing loss, at least 120 genes have been found, but we are far from the end, there are still many genes to discover. In about 50% of patients we do not reach the etiological diagnosis, we cannot find the gene or genes involved. Genes' mutations and variants are not the same in all populations, it is necessary to study patients from different origins. [ABSTRACT FROM AUTHOR]

BACKGROUND: The 20% of the hearing aid's users are not complete satisfied, for this reason it is very important to consider other treatment options. OBJECTIVE: To present preliminary results of the treatment of sensorineural hearing loss with the middle ear implant Vibrant Soundbridge. MATERIAL AND METHOD: An observational, descriptive and cross-sectional study was done from January 1st 2017 to May 2018, with patients with bilateral moderate-severe sensorineural hearing loss, and despite of hearing aid presented poor discrimination to the voice, recurrent infectious pathology of external ear or occlusion sensation. The following studies were performed pre and postoperatively: audiometry logoaudiometry, tympanometry, functional gain and social utility index. RESULTS: There were included five patients. Functional gain of 8.5 dB was obtained with the use of the implant Vibrant Soundbridge, when compared with the conventional hearing aid, as well as better social utility index (11.7%) than with the use of auditory aids. CONCLUSIONS: Middle ear implants are an alternative treatment for patients with sensorineural hearing loss who do not benefit from the use of conventional hearing aids or who, due to medical reasons, can not use them. It provides better tonal gain and better word discrimination than conventional hearing aids. [ABSTRACT FROM AUTHOR]

BACKGROUND: Idiopathic sudden sensorineural hypoacusis is characterized by the sudden decrease of hearing in 30 decibels or more in at least three consecutive frequencies, an accurate cause that conditions this disease is unknown and therefore, the treatment must be instituted as early as possible. OBJECTIVE: To determine the effect of hyperbaric oxygen therapy as a complementary therapy to the steroid treatment of idiopathic sudden sensorineural hypoacusis. MATERIAL AND METHOD: A clinical, prospective, experimental, comparative controlled and randomized trial was done from January 2012 to November 2014, including patients diagnosed with idiopathic sudden sensorineural hypoacusis. Two study groups were formed: oral steroids and oral steroids plus hyperbaric oxygen therapy. We performed audiometries at the beginning, 10 and 20 days of treatment to determine the hearing gain with the average of pure tones (PTA) in decibels (dB). RESULTS: There were included 32 patients; treatment group with steroids plus hyperbaric oxygen therapy (n = 16) had a significantly higher hearing gain (mean = 44.7 dB) than patients in the oral steroid treatment group (n = 16; mean = 23 dB, t = -2.712, df = 30, p = 0.011). CONCLUSIONS: The addition of hyperbaric oxygen therapy to oral steroid therapy provides a significant additional improvement in hearing gain, the greatest hearing gain occurred in the first 10 days of treatment. [ABSTRACT FROM AUTHOR]

BACKGROUND: Sensorineural hearing loss (SNHL) is one of the leading causes of disability in children worldwide. Computed tomography (CT) of temporal bone allows an excellent visual identification of obvious and severe defects, however, these malformations only represent 1% of the anomalies found radiographically. OBJECTIVE: To describe the subtle tomographic characteristics of the inner ear in Mexican population with profound and/or severe sensorineural hearing loss. To compare between measurement values established in North American and European population. MATERIAL AND METHOD: An observational, descriptive and crosssectional study that included Mexican patients, both genders, all ages, with bilateral profound and/or severe sensorineural hearing loss with high-resolution temporal bone CT protocol. Statistical analysis was made using t-Student test. RESULTS: 200 ears were evaluated, 18 presented malformations detected by visual inspection. Using the measuring tool on a workstation of the imaging department, hypoplasia was found in 34 ears (17%), cochlear nerve hypoplastic bone channel in 39 ears (19.5%), narrow internal auditory meatus in 33 ears (16.5%), lateral semicircular canal hypoplastic bone island in 7 (3.5%) and large vestibular aqueduct in 2 ears (1%). CONCLUSIONS: The result of the ten measurements made in the inner ear is comparable to the one established in global population. The percentage of defects detected by visual inspection is below that obtained by formal measurements, so we recommend that imaging departments routinely measure and report the height of the cochlea, the diameter of the bony island of the lateral semicircular canal, and the width of the modiolar aperture. [ABSTRACT FROM AUTHOR]

Hearing loss and tinnitus are well-known secondary effects of acetyl salicylic acid. Tinnitus intensity and hearing loss grade are directly dose and time administration drug dependent. Hypoacusiaprognosis and reversible tinnitus effects correlate with suppression, patient intrinsic factors and opportune treatment. The different mechanisms of aspirin through the whole auditory pathway affect outer hair cells, spiral ganglion, inferior colliculus and auditory cortex. This paper reports the case of a 15-year-old female patient with acute aspirin intoxication and superficial right neurosensorial hearing loss and bilateral tinnitus. Hypocusia was documented in auditory tests, electrical evoked otoacustic emissions were performed and patient was treated with prednisone. She showed a complete recovery of both symptoms after two weeks of treatment even subjacent diagnosis of osteogenesis imperfecta. [ABSTRACT FROM AUTHOR]

For the last 40 years, bone anchored hearing aids have proved to be useful for the treatment of conductive or mixed unilateral or bilateral hearing loss, as well as for sensorineural unilateral hearing loss. Through these years, important improvements have been done in this type of implants, and one of them that presents some of the best features is the Alpha I model by Sophono, which has the advantage of not requiring an abutment, instead of it gets coupled by magnetism. In this article we report the case of a 61-yearold female with unilateral microtia-atresia syndrome, who gained an important hearing improvement with the use of one of this bone anchored devices. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Childhood hearing loss is an important health problem given its serious and irreversible consequences in child's social and cogni-tive development, which explains the establishment in 2001 of a Neonatal Hearing Screening Program in the Valencian Community, being the General Hospital of Castellon a reference centre in the Province. From 2007, coinciding with Hospital Mission Statement, we established the universal screening. OBJECTIVE: To analyze the results obtained in the province of Castellon in the nine years of the Early Detection Program Hearing loss and test the effectiveness of the Universal Screening Program, for which their results were compared with those obtained in previous screening program in which only infants with risk factors were studied (Not Universal Program). PATIENTS AND METHOD: A retrospective study on the early detection of hearing loss in newborn children has been carried out, in order to analyze the results obtained during the ten workingyears of theprogram. RESULTS: Both the risk factor incidence and the diagnosed neurosensory hearing loss have increased from 11/1,000 and 0.29/1,000 to 33/1,000 and 2/1,000 respectively. Ototoxic medication and a weight under 1,500 g are the most common risk factors in both screening tests, while hyperbilirubinemia is currently a rare factor (1.8%). Finally 16.6% ofpatients without established risk factor did not exceed otoacoustic emissions (OEA) and were diagnosed with bilateral sensorineural hearing loss in auditory potentials made. CONCLUSIONS: After comparing the results of both phases ofscreening the efficacy of Universal Detection Program was proved. The establishment of the Universal program has allowed a greater sensitivity when diagnosing newborns suffering from hearing loss and needing treatment such as hearing prosthesis or cochlear implant, considering 16.6% offailed OEAs present neurosensory hearing loss. [ABSTRACT FROM AUTHOR]

Objective. Evaluate the cost-benefit, cost-utility, and cost-effectiveness of cochlear implantation, comparing it to the use of hearing aids in children with profound bilateral sensorineural hearing loss. Methods. The nonparametric propensity score matching method was used to carry out an economic and impact assessment of the cochlear implant and then perform cost-benefit, cost-utility, and cost-effectiveness analyses. Primary information was used, taken randomly from 100 patients: 62 who received cochlear implants (treatment group) and 38 belonging to the control group who used hearing aids to treat profound sensorineural hearing loss. Results. An economic cost differential was found-to the advantage of the cochlear implant-of close to US$ 204 000 between the implant and the use of hearing aids over the expected life span of the patients analyzed. This amount refers to the greater expenses that hearing-aid patients will have. With this adjusted figure, the cost-benefit indicator shows that for each dollar invested to treat the cochlear-implant patient, there is a return on the investment of US$ 2.07. Conclusions. The cochlear implant produces economic benefits for the patient. It also produces health utilities since positive cost-utility (gain in decibels) and cost-effectiveness (gain in language discrimination) ratios were found. [ABSTRACT FROM AUTHOR]

Objective To evaluate the usefulness of antioxidant therapies on prevention of cisplatinum-induced sensorineural hearing loss (SHL) in oncologic patients. Patients and method Repetitive pure tone audiometries were done in patients suffering nasopharyngeal carcinoma submitted to chemotherapy with cisplatinum. The records were made before and after chemotherapy, after finishing radiotherapy and one year later. The mean air conduction threshold for tones between 250 and 8,000 Hz was measured, as well as the mean one just for 8,000 Hz and the deafness average. A N-acetylcysteine (NAC) solution of 200 mg/cc was administered via intratympanic (IT), 1.5 cc weekly, from week -3 to +6 of chemotherapy in 12 ears. A superoxide dismutase (SOD) solution of 8 mg/cc was injected in similar conditions in 13 ears. Other 26 ears were treated with oral NAC at 600 mg/day all over the chemotherapy period. There was a control group of 47 ears without antioxidant treatment. Results One year after beginning chemotherapy, the four groups showed sensorineural hearing loss, but there was a lower impairment in those submitted to IT NAC and SOD. The thresholds at 8,000 Hz of ears treated with IT NAC were less altered than the rest in this year, but the deafness average was much lesser among ears with IT SOD, in a significative statistically way. Oral administration of NAC did not provoke hearing protection. Conclusions Intratympanic treatment with NAC and especially with SOD before and during chemotherapy offers a useful tool in prevention of cisplatinum-induced sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Bartter syndrome (BS) is a heterogeneous group of autosomal recessive hypokalemic salt-losing tubulopathies. Five types of BS caused by different genetic defects have been identified, and one of them is associated with sensorineural deafness (BSND). Mutations in the recently described BSND gene, mapped in chromosome 1p31, have been reported to be associated with BSNS. This gene encodes barttin, an essential B-subunit ClC-ka and ClC-kb channels. Both subunits are co-expressed in basolateral membranes of renal tubules in the thin and thick ascending limb of Henle's loop and in the stria vascularis of the inner ear. We studied two venezuelan sisters, daughters of consanguineous parents from a small town called Jadacaquiva, in the peninsula of Paraguan., Venezuela. Sequence analysis of the BSND gene showed that the affected members were homozygous for C to T transition in axon 1. This alteration results in a missense mutation, G47R that has been previously shown to abolish the stimulatory effect on the subunit barttin of the ClC-Kb channel. The patients with the G47R mutation presented polyhidramnios, premature birth and salt loss. Nevertheless, glomerular filtration rate is normal. Clinical manifestations are moderate in patients with G47R mutation with regard to other patients reported with BSND. This is the first report of BSND in Venezuela. [ABSTRACT FROM AUTHOR]

BACKGROUND The sudden hearing loss must be considered like a medical urgency within the field of the otolaryngology. Factors exist that influence the effectiveness of the treatment, making difficult their study, like the severity of hearing loss, type of audiometric curve, presence of vertigo, time of beginning of treatment, age and personal antecedents. Nevertheless a high rank of spontaneous resolution has been documented. OBJECTIVE To analyze the effectiveness of the treatment administered to all the cases of sudden hearing loss of January to September of 2003 at National Medical Center, IMSS, of the city of Leon, Guanajuato. MATERIAL AND METHODS We made a retrospective and descriptive study of 13 patients with diagnosis of sudden hearing loss. We divided the patients in three groups in agreement to the type of treatment: combined (prednisona, pentoxifilina, acyclovir), six patients (46.2%); treatment with vasodilator (cinarizina), five patients (38.5%); without treatment two patients (15.3%). RESULTS In the first group an improvement of 66% was observed, 40% in the second and without improvement in third. CONCLUSION We concluded that the combined treatment group presented better results than the treatment only with vasodilator. The patients without treatment were not observed any type of improvement. [ABSTRACT FROM AUTHOR]