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1.

Background

Chronic suppurative otitis media (CSOM) affects 65–330 million people in the developing part of the world and develops in early childhood. Knowledge of the long-term effects on hearing is scarce. Hearing loss (HL) can cause reduced ability to communicate, impair language development and academic skills.

Objective

To estimate the prevalence of HL in a population with a high-risk of CSOM and to identify the risk of permanent hearing loss from CSOM.

Methods

Ten and 15 years follow-up of two Greenlandic population-based children cohorts. Participants underwent otologic examination and audiometric evaluation. HL was defined using both the American Speech-Language-Hearing Association's (ASHA) and the World Health Organizations's (WHO) definition. Risk factors for HL were analyzed, using binomial logistic regression analysis.

Results

A total of 438 individuals aged 11–24 years participated. Using the ASHA definition of HL the prevalence was 50% (95% CI 45.3–54.7). Using the WHO definition of HL the prevalence was 2.5% (95% CI 1.1–3.98). CSOM was the main cause of HL. Co-existing CSOM (OR 16.7, 95% CI 8.29–33.65), spontaneous healing from CSOM (OR 3.75, 95% CI 1.62–8.67), and male gender (OR 2.2, 95% CI 1.28–3.78) were associated with HL. Ninety-one percent of children with CSOM developed a permanent hearing loss >15 dBHL.

Conclusion

CSOM was strongly associated with permanent hearing loss. CSOM was the main reason for HL in this population. The WHO definition of hearing loss is likely to underestimate the burden of hearing loss among children and adolescents in developing countries with a high prevalence of CSOM.  相似文献   

2.

Objective

We aimed to describe a large cohort of patients with tinnitus and sensorineural hearing loss (SNHL) in Sweden, and also to explore the possibility of finding potential possible differences between various diagnoses within SNHL. It is also of great interest to see how a multidisciplinary team was used in the different subgroups and the frequency of hearing aids use in patients with tinnitus.

Methods

Medical records of all patients who had received the diagnosis SNHL in Östergötland County, Sweden between 2004 and 2007 were reviewed. Patients between 20 and 80 years with tinnitus and a pure tone average (PTA) lower than 70 dB HL were included in the study. Patients were excluded from the analyses if they had a cochlear implantation, middle ear disorders or had a hearing loss since birth or childhood. The investigators completed a form for each included patient, covering background facts, and audiograms taken at the yearly check up.

Results

Of a total 1672 patients’ medical record review, 714 patients were included. The majority of patients (79%) were in the age group over 50 years. In male patients with bilateral tinnitus, the PTA for the left ear was significantly higher than for the right ear. The results regarding the configuration of hearing loss revealed that 555 patients (78%) had symmetric and 159 (22%) asymmetric hearing loss. Retrocochlear examinations were done in 372 patients and MRI was the most common examination.In all patients, 400 had no hearing aids and out of those 220 had unilateral tinnitus and 180 patients had bilateral tinnitus. 219 patients had a PTA > 20 dB HL and did not have any hearing aid. Results demonstrated that the Stepped Care model was not used widely in the daily practice. In our study, patients with bilateral-, unilateral hearing loss or Mb Ménière were the most common patients included in the Stepped Care model.

Conclusion

In a large cohort of patients with SNHL and tinnitus, despite their hearing loss only 39% had hearing aids. It was observed that the medical record review often showed a lack of information about many background factors, such as; patients’ general health condition, which could be a quality factor that needs improvement. Our results show that the Stepped Care model could be an effective option for providing a better access for tinnitus-focused treatment, although the number of patients in this study who were included in the Stepped Care model was low.  相似文献   

3.

Objective

Implantable hearing aids have become a valid option for the therapy of various forms of hearing loss. The Codacs? Direct Acoustic Cochlear Implant System is the first vibratory implant available for patients with severe to profound mixed hearing loss (MHL). By directly coupling sound energy into the perilymph, a very high maximum power output (MPO) is achieved over a broad frequency range. Via a conventional stapedotomy, the vibratory energy of the electromagnetic actuator is transferred directly to the perilymph through the oval window.

Patients and methods

This article describes the technical principle, basic surgical aspects and audiological outcomes of two clinical studies. Additional coupling procedures and extensions to the spectrum of indications are also discussed. Surgically, the two-component system can either be implanted via a purely transmastoid approach with posterior tympanotomy, or additionally via transmeatal access to the stapes footplate. Pre- and postoperative audiological results of patients wearing conventional hearing aids and with severe to profound MHL, who were implanted with the Codacs? system at the Medical University Hannover, were compared.

Results

Significant improvements over conventional hearing aids could be achieved with initial bone conduction thresholds between 44 and 63 dB HL (Ø 54 dB HL) and an air-bone gap between 19 and 51 dB HL (Ø 34 dB HL) in patients with MHL caused by advanced otosclerosis or tympanosclerosis and an intact posterior wall of the auditory canal. The mean functional gain was 50?±?9 dB (0.5?–?4 kHz) and the monosyllabic word score was 85?% at 65 dB presentation level compared to conventional hearing aids with 25?%. Speech intelligibility in noise (S0N0) improved by 7.1 to Ø 0.3 dB SNR with Codacs?. The mean bone conduction threshold remained unchanged or showed a minimal increase in the low-frequency range.

Conclusion

The CODACS system provides an effective new treatment for patients with severe to profound MHL for the first time.  相似文献   

4.

Background

To the best of our knowledge, no empirically based consensus has been reached as to if, and to what extent, persons with hearing loss (HL) have reduced generic Quality of life (QoL). There seems to be limited knowledge regarding to what extent a hearing aid (HA) would improve QoL. The main aim of the present study was to review studies about the relationship between HL and QoL. A supporting aim was to study the association between distress and HL.

Methods

Literature databases (Cinahl, Pub Med and Web of Science) were searched to identify relevant journal articles published in the period from January 2000 to March 17, 2016. We performed a primary search pertaining to the relationship between HL, HA and QoL (search number one) followed by a supporting search pertaining to the relationship between distress/mood/anxiety and HL (search number two). After checking for duplications and screening the titles of the papers, we read the abstracts of the remaining papers. The most relevant papers were read thoroughly, leaving us with the journal articles that met the inclusion criteria.

Results

Twenty journal articles were included in the present review: 13 were found in the primary search (HL and QoL), and seven in the supporting search (HL and distress). The literature yields equivocal findings regarding the association between generic QoL and HL. A strong association between distress and HL was shown, where distressed persons tend to have a lowered generic QoL. It is suggested that QoL is lowered among HL patients. Some studies suggest an increased generic QoL following the use of HA, especially during the first few months after initiation of treatment. Other studies suggest that HA use is one of several possible factors that contribute to improve generic QoL.

Conclusions

The majority of the studies suggest that HL is associated with reduced generic QoL. Using hearing aids seem to improve general QoL at follow-up within the first year. HL is a risk factor for distress. Further research is needed to explore the relationship between HL and generic QoL, in addition to the importance of influencing variables on this relationship.
  相似文献   

5.
OBJECTIVE: To determine whether variants of the mitochondrial genome influence the risk of developing age-related hearing loss (ARHL). DESIGN: Cross-sectional study. SETTING: Eligible participants were noninstitutionalized permanent residents 49 years or older identified in a door-to-door census of 2 suburban postcode areas, west of Sydney, Australia. PARTICIPANTS: The Blue Mountains Hearing Study (BMHS) was a population-based survey of hearing loss, conducted during 1997 to 1999, among the participants of the Blue Mountains Eye Study cohort. MAIN OUTCOME MEASURES: We defined hearing impairment as the pure-tone average of audiometric hearing thresholds at 500, 1000, 2000, and 4000 Hz (> 25- but 40- but 60-dB HL [severe hearing loss]) in the better of the 2 ears. RESULTS: Of the 2765 BMHS participants, 912 (33%) were found to have ARHL. After adjusting for other hearing loss risk factors, mitochondrial DNA (mtDNA) haplogroups U and K were independently associated with a higher prevalence of ARHL compared with subjects with other haplogroups. Haplogroup U was significantly associated with moderate to severe ARHL (multivariable-adjusted odds ratio, 1.63; 95% confidence interval, 1.10-2.41). Haplogroup K was associated with severity types of ARHL in persons aged 50 to 59 years (odds ratio, 3.02; 95% confidence interval, 1.30-6.99). There was also a joint effect between mtDNA haplogroups U and K and other known hearing loss risk factors such as diabetes and past noise exposure. CONCLUSION: Findings from this older Australian population demonstrate an association between certain mtDNA haplogroups and ARHL, as well as a link to the susceptibility of other known risk factors for ARHL.  相似文献   

6.

Background

A hearing screening among 14- to 15-year-old pupils was performed to estimate the number of hearing-impaired individuals in the adolescent population.

Materials and methods

A total of 1,298 pupils from 30 schools in Tyrol (Austria) participated in the screening. Hearing tests were performed in a silent room at the school. Sinus tones at frequencies 0.5/1/2/4/6 kHz and at levels of 25/20/20/20/20 dB HL, respectively, were delivered via headphones to either ear. Failure of screening was defined as not hearing one or more frequencies in one or both ears.

Results

The screening was failed by 16.3% of the pupils. There was a small but not significant difference between males and females (17.0 vs. 15.2%). Most of the pupils failed at only one frequency (9.6%). Failing at two or more frequencies in the same ear occurred in 3.9% of the pupils, thereof in 1.1% bilaterally.

Conclusion

As the specificity of our screening is limited, false-positive results may result. Thus, the rate of hearing deficits in our sample is probably a bit lower than indicated by the figures above. Most of the adolescents who failed the screen failed at only one frequency. These subjects have a small elevation of their hearing threshold, not a hearing loss in the sense of a raised averaged threshold. A hearing loss in the latter sense is supposed to be present in only very few percent of adolescents, a bilateral hearing loss in perhaps less than 1%.  相似文献   

7.

Objective

Cornelia de Lange syndrome (CdLS) is a rare multisystem disorder in which hearing loss (HL) has been reported. However, no data are available concerning the association between audiological findings, clinical severity score and genotype.

Methods

The study involved 44 pediatric patients aged 1–18 years with a confirmed diagnosis of CdLS, all of whom underwent a full otolaryngological and audiological examination. The presence of NIPBL and SMC1 mutations was also evaluated.

Results

According to the severity of clinical phenotypes, 12 (27.3%) children were mild, 15 (34.1%) were moderate and 17 (38.6%) were severe. Thirty-eight children (86%) had OME. Eight children had normal hearing, including one (12.5%) with a severe phenotype. Bilateral sensorineural hearing loss (SNHL) was diagnosed in 10 children (22.7%): the degree of HL was severe in 8 (80%), all with a severe phenotype. Conductive hearing loss (CHL) was present in 26 patients (59.1%), of whom 8 (30.8%) had a severe phenotype. A severe phenotype was more prevalent among the patients with moderate to severe HL (10/16, 62.5%) than among those with slight/mild HL or normal hearing (7/28, 25.0% p = 0.013). NIPBL mutations were detected in 22 patients (50%): 13 (59.1%) with truncating mutations, four (18.2%) with missense mutations, and five (22.7%) with splicing mutations. The frequency of NIPBL truncating mutations was similar in the children with SNHL and those with CHL, whereas this kind of mutation was not found in children with normal hearing.

Conclusion

Together with SNHL, CHL is an important cause of HL in children with CdLS, and can be associated with a severe phenotype. Moreover, CHL can be associated with NIPBL mutations, particularly truncating mutations. These results highlight the importance of the early identification of audiological problems in children with CdLS and their precise genetic characterization.  相似文献   

8.

Introduction

Because management of hearing loss (HL) often requires multiple specialists, a multidisciplinary clinic, Pediatric Hearing Management Clinic, (PHMC) was established to coordinate care for children with newly diagnosed HL.

Methods

Retrospective review of patients seen in PHMC from February 2009 to April 2010.

Results

Clinic information was available for 40/41 of the patients and was included in the study. 37/41 had confirmed HL. HL was categorized into bilateral/symmetric [15], bilateral/asymmetric [12] and unilateral [10]. Sixteen patients subsequently received hearing amplification after PHMC visit. Follow up was successfully established with otolaryngology in 23/32 (72%) patients, audiology in 29/40 (73%) patients, speech pathology in 11/12 (92%) patients, and ophthalmology in 15/30 (50%) patients. Twenty-nine patients completed evaluations of PHMC. The mean score in six total categories was 3.8/4.0 (4.0 as the highest satisfaction).

Conclusion

A multidisciplinary clinic approach provides a convenient and effective way to coordinate care for children with HL.  相似文献   

9.

Objectives/Hypothesis:

To describe the audiological profiles in a Japanese family with autosomal dominant hereditary sensorineural hearing loss (SNHL) and to identify the causative gene.

Study Design:

A family study at an academic tertiary referral center.

Methods:

A family with autosomal dominant hereditary SNHL was enrolled. Hearing loss (HL) of affected members showed mid‐frequency SNHL in childhood and progressed at lower frequencies with age, resulting in low‐frequency SNHL. To understand the pathology of HL of this family, we performed a genetic analysis of WFS1, TECTA, and GJB2 by direct sequencing, and further audiovestibular examinations, including speech audiometry, distortion product otoacoustic emissions, electrocochleography, auditory brainstem responses, and electronystagmography for some affected members.

Results:

A heterozygous A‐to‐C nucleotide transversion (c.2507A>C), resulting in a lysine‐to‐threonine substitution at codon 836 (K836T) was identified in exon 8 of WFS1. K836T was segregated with HL in the 15 participants in the genetic study but was not detected in the 212 normal chromosomes. Only polymorphic changes were detected in TECTA and GJB2. Audiovestibular examinations indicated purely cochlear HL and normal vestibular function. The summating potential/action potential ratios in electrocochleography increased in the bilateral ears of the proband.

Conclusions:

The family described with autosomal dominant inheritance of K836T of the WFS1 gene demonstrates a progressive hearing loss in the lower frequencies. Laryngoscope, 2010  相似文献   

10.

Objective

Newborn hearing screening was implemented in Flanders about fifteen years ago. The aim of this study was to determine the aetiology of hearing loss detected by the Flemish screening programme.

Methods

From 1997 to 2011, 569 neonates were referred to our tertiary referral centre after failed neonatal screening with Auditory Brainstem Responses. In case hearing loss (HL) was confirmed, further diagnostic testing was launched. A retrospective chart review was performed analysing the degree of HL, risk factor and aetiology.

Results

Metabolic disorders (0.5%), infectious diseases (35.8%), congenital malformations (6.1%) and genetic abnormalities (19.8%), whether or not syndromic, were retained. In 35% of the subjects no obvious aetiology could be determined in the current study.

Conclusion

In contrast to the literature findings, this series shows a genetic syndromic cause in 80% of the genetic bilateral HL cases. On the other hand connexin positive diagnoses were mostly underrepresented in this study, showing the need for better screening.  相似文献   

11.

Objectives

The aim of the study was to determine the characteristics of patients who did not match the audiometric criteria of idiopathic sudden sensorineural hearing loss (SSNHL) but complained of acute hearing loss.

Methods

By thorough medical chart reviews, historical cohort study was performed with consecutive data of 589 patients complaining of acute unilateral sensorineural hearing loss without identifiable causes between 2005 and 2013. Those patients demonstrating a hearing loss of at least 30 dB at three consecutive frequencies based on pure tone audiometry were classified as group I; the others were classified as group II. Patients'' characteristics, final hearing, and hearing improvement rate (HIR) between the two groups were compared.

Results

Group II exhibited distinctive characteristics, including an early age of onset of the hearing loss (P<0.01), an absence of accompanying diabetes (P<0.01) and hypertension (P<0.01), and better unaffected hearing and final hearing compared with group I (P<0.001). However, the HIR of the patients in the two groups was not significantly different (P>0.05).

Conclusion

Patients who did not meet the audiological criteria of SSNHL exhibited distinctive characteristics compared to SSNHL patients.  相似文献   

12.

Objective

After 20 years of experience with different types of middle ear implants, we analyzed our database about the Vibrant Soundbridge (VSB) to know the rate of complications, the effect on the residual hearing and the audiometric gain in our center.

Method

The study was retrospective and included all VSB implants bound to the long process of the incus in our tertiary medical center between january 1999 and february 2015. We observed the effect of surgery on residual hearing by comparing bone and air conduction thresholds before and after implantation. The functional results of the implant were quantified by measuring, at several post-operative intervals, the thresholds with the VSB in pure tone audiometry and speech audiometry, in quiet and in noise.

Results

53 VSB were implanted in 46 patients aged between 22 and 81 years old (average 53.9). 48 patients (90%) suffered from a sensorineural hearing loss, and 5 patients from a mixed hearing loss due to an otosclerosis (but only 3 of them have undergone stapedotomy). There were no major complications (e.g. facial palsy, dead ear or postoperative infection). The placement of the implant created an insignificant deterioration of the air conduction thresholds (5,6 dB HL) and bone conduction thresholds (2.2 dB HL) at 6 weeks post-implantation. The bone conduction thresholds increased by 4.7 dB HL 2.5 years after surgery in comparison with the preoperative results, which is also considered clinically insignificant. With the implant turned on, the pure tone audiometry thresholds in open field, in quiet, were significantly improved (gain of 13.9 dB on average on frequencies from 250 to 8000 Hz), particularly at frequencies of 1000, 2000 and 4000 Hz as the average gain on these frequencies amounted to 19.4 dB. The speech intelligibility in a cocktail party noise was also improved by 18.3% on average at 6, 52 and 104 weeks post-implantation.

Conclusion

The Vibrant Soundbridge with the electromagnetic vibrator fixed to the long process of the incus is a safe active middle ear implant with no major complications; it has no significant impact on the residual hearing. The VSB is particularly suitable for patients who are unable to wear conventional hearing aids due to anatomical or infectious problems in the external ear canal, or in case of poor audiometric results with conventional hearing aids. The VSB brings significant hearing gain, as it is particularly efficient in frequencies for the speech range and higher, resulting especially in better speech intelligibility in noisy environments.  相似文献   

13.

Objective

While hearing loss following temporal bone fracture is a well-described phenomenon, few data exist on auditory dysfunction in patients with traumatic brain injury (TBI) without temporal bone fracture. Herein, we aim to systematically review hearing loss after TBI without bony fracture and describe its etiologies.

Data sources

Pubmed, Embase, Cochrane databases.

Review methods

A systematic review of the literature from 1966 to January 2017 was performed using Preferred Reporting Items for Systematic Reviews and Meta-analyses recommendations. Data were obtained from studies that investigated hearing loss in TBI without skull fracture according to an a priori protocol with inclusion and exclusion criteria. Variables included type and severity of hearing loss, as well as pathophysiology of hearing loss.

Results

There were 13 studies with 773 patients that met study criteria. Overall, there was one prospective cohort study, four retrospective cohort studies, two case-control studies, and six case reports. The studies with the highest level of evidence report a change in hearing of at least 10–15?dB across a range of frequencies in as many as 58% percent of TBI patients without bony fracture, which was transient or chronic. The mechanism/severity of injury may impact the rate of hearing loss.

Conclusions

Hearing loss after TBI in the absence of bony injury appears to be a clinically significant but poorly characterized phenomenon.  相似文献   

14.

Objectives

The aim of this study is to evaluate the sensitivity of our hearing screening program. The evaluation was done using a questionnaire for parents of children who participated in the NHS for a targeted time frame of two years. A survey was accomplished to identify children who passed our screening protocol in the newborn period, but who were later identified to have hearing loss.

Methods

For the survey a one-year cohort was established in 2008 which included 500 children who received a hearing screening at our Center with the ABR newborn screener, MB11 BERAphone®, two years before. Two hearing questionnaires were chosen for the survey. The LittlEARS questionnaire (MED-EL Medical Electronics GmbH) for investigating the hearing behavior of the children during the first two years of life and a second, custom-developed questionnaire (Würzburger questionnaire) investigating some aspects which are not included in the LittlEARS tool, such as speech/language development, general development as well as pathological factors that might eventually lead to a temporary hearing loss.

Results

Analysis of the Würzburger questionnaires revealed normal speech development for 92.9% of the children. For 4.7% male and 2.4% female children delayed speech development was reported. Although twice as many males were found, the statistical comparison showed no significant difference according to gender. The results of the LittlEARS questionnaire are identical to those of the Würzburger questionnaire in 98.3% of the investigated cases and in 1.7% of the cases slightly different results but on borderline: The LittlEAR scores showed normal auditory development for the childrens’ age but the Würzburger questionnaire results showed delayed speech development.

Conclusions

Based on the follow-up analysis and the results from the two questionnaires, no permanent hearing loss was found in any child two years after they passed the newborn hearing screening. Thus, we conclude that the sensitivity of the screening test was 100%, based on survey results 2 years post screening.  相似文献   

15.

Background and aim

Speech perception is the most important social task of the auditory system. Consequently, speech audiometry is essential to evaluate hearing aid benefit. The aim of the study was to describe the correlation between pure-tone hearing loss and speech perception. In particular, pure-tone audiogram, speech audiogram, and speech perception with hearing aids were compared.

Materials and methods

In a retrospective study, 102 hearing aid users with bilateral sensorineural hearing loss were included. Pure-tone loss (PTA) was correlated to monosyllabic perception at 65 dB with hearing aid and with maximum monosyllabic perception with headphones.

Results

Speech perception as a function of hearing loss can be represented by a sigmoid function. However, for higher degrees of hearing loss, substantial deviations are observed. Maximum monosyllabic perception with headphones is usually not achieved with hearing aids at standard speech levels of 65 dB.

Conclusion

For larger groups, average pure-tone hearing loss and speech perception correlate significantly. However, prognosis for individuals is not possible. In particular for higher degrees of hearing loss substantial deviations could be observed. Speech performance with hearing aids cannot be predicted sufficiently from speech audiograms. Above the age of 80, speech perception is significantly worse.  相似文献   

16.
Abstract

Objective: To assess the American Medical Association (AMA) guide to the evaluation of binaural hearing impairment (BHI) as a procedure for estimating severity of hearing loss from audiograms. Design: The BHIs of Australian war veterans were calculated from their hearing threshold levels (HTLs) and compared with their scores on a hearing questionnaire, the hearing measurement scale (HMS). Study sample: The HTLs of 282 Australian war veterans were measured at frequencies from 0.25 to 8 kHz and scores on the HMS were obtained from 154 of those veterans. Results: No grounds could be found for altering the frequencies included in the average HTL or the high fence of 92 dB HL used in calculating the monaural hearing impairments (MHIs) of the veterans, and no grounds could be found for altering the ratio of 5:1 used in determining the BHI from the MHIs of the better and worse ears. However, agreement between HMS score and BHI was improved by reducing the low fence used in calculating MHI from 25 to 15 dB HL. Conclusion: A modified version of BHI provided an improved procedure for estimating severity of hearing loss from audiograms but would not be suitable for compensation purposes.  相似文献   

17.

Objective

Newborn hearing screening (NHS) is used worldwide due to its feasibility and cost-efficiency. However, neonates with late-onset and progressive hearing impairment will be missed by NHS. Genetic factors account for an estimated 60% of congenital profound hearing loss. Our previous cohort studies were carried out in an innovative mode, i.e. hearing concurrent genetic screening, in newborns to improve the abilities or early diagnosis and intervention for the hearing defects. In this study, we performed the first clinical practice of this mode in Tianjin city.

Methods

A large cohort of 58,397 neonates, born between December 2011 and December 2012, in 44 hospitals in Tianjin, were screened for 20 hot spot hearing loss associated mutations from GJB2, GJB3, SLC26A4 and MTRNR1(12S rRNA). The data of genetic screening results was comprehensively analyzed with newborn hearing screening (NHS) results.

Results

We developed an accurate, high throughput genetic screening method and applied it to a total of 58,397 newborns in Tianjin. 3225 (5.52%) infants were detected to carry at least one mutation allele in GJB2, GJB3, SLC26A4 or MTRNR1. 34 (0.58‰) infants were positive for hearing loss caused by GJB2 or SLC26A4 mutations (homozygote or compound heterozygote). 54(0.93‰) infants are heterozygous of various genes. 109(1.87‰) infants had the pathological mitochondrial DNA mutation.

Conclusion

Accurate, comprehensive hearing loss associated genetic screening can facilitate genetic counseling and provides valuable prognostic information to affected infants. This united screening mode of this study was a promising clinical practice.  相似文献   

18.

Introduction

Labyrinthine fistula is one of the most common complications associated with cholesteatoma. It represents an erosive loss of the endochondral bone overlying the labyrinth. Reasons for cholesteatoma-induced labyrinthine fistula are still poorly understood.

Objective

Evaluate patients with cholesteatoma, in order to identify possible risk factors or clinical findings associated with labyrinthine fistula. Secondary objectives were to determine the prevalence of labyrinthine fistula in the study cohort, to analyze the role of computed tomography and to describe the hearing results after surgery.

Methods

This retrospective cohort study included patients with an acquired middle ear cholesteatoma in at least one ear with no prior surgery, who underwent audiometry and tomographic examination of the ears or surgery at our institution. Hearing results after surgery were analyzed according to the labyrinthine fistula classification and the employed technique.

Results

We analyzed a total of 333 patients, of which 9 (2.7%) had labyrinthine fistula in the lateral semicircular canal. In 8 patients, the fistula was first identified on image studies and confirmed at surgery. In patients with posterior epitympanic and two-route cholesteatomas, the prevalence was 5.0%; and in cases with remaining cholesteatoma growth patterns, the prevalence was 0.6% (p = 0.16). In addition, the prevalence ratio for labyrinthine fistula between patients with and without vertigo was 2.1. Of patients without sensorineural hearing loss before surgery, 80.0% remained with the same bone conduction thresholds, whereas 20.0% progressed to profound hearing loss. Of patients with sensorineural hearing loss before surgery, 33.33% remained with the same hearing impairment, whereas 33.33% showed improvement of the bone conduction thresholds’ Pure Tone Average.

Conclusion

Labyrinthine fistula must be ruled out prior to ear surgery, particularly in cases of posterior epitympanic or two-route cholesteatoma. Computed tomography is a good diagnostic modality for lateral semicircular canal fistula. Sensorineural hearing loss can occur post-surgically, even in previously unaffected patients despite the technique employed.  相似文献   

19.

Objective

We conducted an attitude survey for patients with hearing loss (HL). The aim of this study was to investigate the opinions of patients or parents of deaf children regarding the deafness gene, genetic testing and a gene related HL.

Method

A questionnaire was sent to 201 individuals who visited the Department of Otolaryngology, Tokyo Medical and Dental University and who received genetic testing for HL from September 2000 to January 2006. There were 14 questions in the questionnaire that were classified into four topics related to a deafness gene and hereditary HL, genetic testing, outpatient department of medical genetics/genetic counseling, and the results of genetic testing. The study consisted of 140 respondents (70%) of 201 administered surveys.

Result

Before visiting our department, only 36% of the respondents were aware that a genetic factor was a cause of HL. Despite our explanation of a deafness gene and hereditary HL, 23% of 134 respondents answered that they had not received any such explanation. Furthermore, 14% of the 103 respondents who had answered that they receive the explanation, however, they did not fully understand it. Thirty-nine percent of the respondents made their own decision regarding the genetic testing, whereas 53.5% received the tests upon the advice of a physician or family member. In contrast, 91% of the respondents had a positive attitude towards other future genetic tests. The existence of the genetic outpatient department or genetic counseling has been seldom acknowledged, but upon learning of its availability, nearly one third of the respondents indicated that they would like to receive genetic counseling. Although no respondent had social and/or family problems after being informed that they had a deafness gene mutation, some respondents worried about the result.

Conclusion

The results of the survey suggested that the vast majority of the respondents were satisfied with genetic testing for HL and that the barriers to take the genetic test were less than expected. However, some respondents have a negative attitude towards genetic testing and counseling. Furthermore, the issue of disclosure may be burdensome to patients.  相似文献   

20.

Objectives

Although many studies have assessed sudden deafness in adults, sudden deafness has not been evaluated in children. We therefore evaluated the differences in sudden deafness between children and adults.

Methods

We compared clinical manifestations, including gender, audiogram pattern of initial hearing loss, and recovery rate after treatment in 87 children and 707 adults diagnosed with sudden deafness from September 2003 and August 2012.

Results

There were no differences in sex, side, or audiogram between children and adults (P>0.05 each). Hearing recovery rates in children and adults were 72.4% and 70.6%, respectively (P>0.05). Both children and adults with mild hearing loss showed significantly greater hearing recovery rates than individuals with profound hearing loss (P<0.05 each). The percentage with initially mild and moderate hearing loss was higher in children than in adults, as were the recovery rates of children compared to adults with initially mild, moderate-severe, and profound hearing loss (P<0.05 each). In regard to final hearing outcome after treatment, a low percentage of children showed no improvement whereas a high percentage showed complete recovery; a higher percentage of children than of adults showed complete recovery (P<0.05). Recovery rate from profound hearing loss was significantly higher in children than in adults (60.0% vs. 45.4%, P<0.05).

Conclusion

Degree of hearing loss, gender, side, and recovery rate were similar in children and adults, but the rate of complete recovery was higher in children.  相似文献   

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