Language evaluation in children with pre-lingual hearing loss and cochlear implant

IntroductionThe cochlear implant is an effective device for children with severe and/or profound prelingual hearing loss, since it provides considerable improvement in oral language acquisition through the auditory pathway. The use of a cochlear implant contributes to the development of auditory perception, favoring the acquisition of the linguistic processes related to communication skills, which might have a positive effect on other areas of development.ObjectiveThe aim of this study was to verify the performance of children using cochlear implants for expressive and receptive oral language.MethodsThis was a prospective cross-sectional study that used the following tests: Child language test in the phonology, vocabulary, fluency and pragmatics areas, and the Peabody picture vocabulary test. Thirty children participated in this study, of both genders, aged between 36 and 72 months, with severe and/or profound bilateral sensorineural hearing loss, without other impairments and users of unilateral cochlear implant with full electrode insertion for a minimum of 12 months.ResultsThe longer duration of the cochlear implant use, the younger age at surgery and the better performance in the auditory perception of speech influenced the performance in expressive and receptive oral language. Even though when compared to the normative language acquisition process, the results showed that these children had patterns of linguistic skills that are below their chronological age; the results indicate that these children are developing expressive and receptive oral language skills, and this is the outcome that should be taken into account in this study.ConclusionThe longer duration of the cochlear implant use, the younger age at surgery and the better performance in the auditory perception of speech influenced the performance in expressive and receptive oral language skills, but not in all the studied semantic categories.     

Quality of life and cochlear implant: results in adults with postlingual hearing loss

Introduction

Considering the variability of results found in the clinical population using a cochlear implant, researchers in the area have been interested in the inclusion of quality of life measures to subjectively assess the benefits of the implantation.

Sensorineural hearing loss in the acute phase of a single episode of acute otitis media

IntroductionAcute otitis media is a disease with high global prevalence, that can lead to several acute complications and auditory sequelae. Data regarding the auditory evaluation in the acute phase of acute otitis media are scarce.ObjectiveTo evaluate the main audiometric changes (air and bone conduction thresholds) in the initial phase of an acute otitis media episode.MethodsA case-control study was performed. Patients diagnosed with acute otitis media with less than 7 days of evolution in relation to the complaint onset were selected, and healthy volunteers were selected as controls. The acute otitis media and control groups were submitted to pure tone and vocal audiometry.ResultsThe acute otitis media group included a total of 27 patients (30 ears). Hearing loss was present in 90.0% of the ears with acute otitis media, with conductive loss in 14 (46.67%) and mixed loss in 13 (43.33%). Both the air and bone conduction thresholds obtained with the tonal audiometry in the acute otitis media group were significantly worse than the controls at all tested frequencies (p < 0.05). In patients with acute otitis media, we observed that the thresholds for frequency >1 kHz (bone conduction) and 3 kHz (air conduction) were significantly worse in patients with tinnitus compared to patients without tinnitus.ConclusionDuring the first 7 days of evolution after the onset of an isolated episode of acute otitis media, we observed significant increases in bone and air thresholds at all frequencies, especially >2 kHz, compared to healthy ears.     

Hearing rehabilitation in cerebral palsy: development of language and hearing after cochlear implantation

IntroductionAuditory rehabilitation in children with bilateral severe-to-profound sensorineural hearing loss with cochlear implant has been developed in recent decades; however, the rehabilitation of children with cerebral palsy still remains a challenge to otolaryngology and speech therapy professionals.ObjectiveTo verify the effectiveness of cochlear implants in the development of auditory and language skills in children with cerebral palsy.MethodsA prospective analytical study. The evaluation of auditory responses to speech test was applied to the children in this study at regular intervals following implantation. Standardized tests that assess and quantify the development of auditory and language skills were administered and speech therapy video records and speech therapy files were analyzed. All children went through individually tailored intensive audiological rehabilitation programs following cochlear implantation.ResultsTwo participants had gradual auditory and language development when compared to other participants who reached advanced levels in hearing and oral language classifications.ConclusionThe use of the Cochlear implant enabled participants to reach advanced stages of hearing and language skills in three of the five participants with cerebral palsy in this study. This electronic device is a viable therapeutic option for children with cerebral palsy to help them achieve complex levels of auditory and language skills.     

Sequential bilateral cochlear implant: results in children and adolescents

IntroductionThe use of the bilateral cochlear implants can promote the symmetrical development of the central auditory pathways, thus benefiting the development of auditory abilities and improving sound localization and the ability of auditory speech perception in situations of competitive noise.ObjectiveTo evaluate the ability of speech perception in children and adolescents using sequential bilateral cochlear implants, considering the association of these variables: age at surgery, time of device use and interval between surgeries.MethodsA total of 14 individuals between 10 and 16 years of age, who demonstrated surgical indication for the use of sequential bilateral cochlear implants as intervention in the auditory habilitation process, were assessed. The speech perception ability was assessed through sentence lists constructed in the Portuguese language, presented in two situations: in silence, with fixed intensity of 60 dB SPL, and in competitive noise, with a signal-to-noise ratio of +15 dB. The evaluation was performed under the following conditions: unilateral with the first activated cochlear implant, unilateral with the second activated cochlear implant and bilateral with both devices activated.ResultsThe results of the speech perception tests showed better performance in both silence and in noise for the bilateral cochlear implant condition when compared to the 1st cochlear implant and the 2nd cochlear implant alone. A worse result of speech perception was found using the 2nd cochlear implant alone. No statistically significant correlation was found between age at the surgical procedure, interval between surgeries and the time of use of the 2nd cochlear implant, and the auditory speech perception performance for all assessed conditions. The use of a hearing aid prior to the 2nd cochlear implant resulted in benefits for auditory speech perception with the 2nd cochlear implant, both in silence and in noise.ConclusionThe bilateral cochlear implant provided better speech perception in silence and in noise situations when compared to the unilateral cochlear implant, regardless of the interval between surgeries, age at the surgical procedure and the time of use of the 2nd cochlear implant. Speech perception with the 1st cochlear implant was significantly better than with the 2nd cochlear implant, both in silence and in noise. The use of the hearing aid prior to the 2nd cochlear implant influenced speech perception performance with the 2nd cochlear implant, both in silence and in noise.     

Cochlear implant in Thalassemia patient – Case report

Introduction: Beta-thalassemias are a group of hereditary blood disorders characterized by anomalies in the synthesis of the beta chains of hemoglobin.

Iron overload occurs in thalassemia, with blood transfusion therapy being the major cause. Deferoxamine continues to be the mainstay of therapy to remove excess iron in patients requiring long-term transfusions.

One of the most important complications of deferoxamine therapy is neurosensory toxicity, including sensorineural hearing loss (SNHL).

Labyrinthine hemmorhage (LH) is thought to result from altered capillary hemodynamics or reperfusion injury. It is theorized that LH incites a reparative response that cascades from fibrosis to sclerosis and ultimately ossification of the inner ear structures.

Case presentation: We present a case of 3-year-old thalassemic child with bilateral profound sensorineural hearing loss. Patient was on regular blood transfusions with chelation therapy.

HRCT temporal bone and MRI brain and temporal bone had features of labyrinthitis ossificans (LO).

Child underwent uniateral cochlear implantation and postimplantation speech perception and production outcomes were normal.

Discussion: This case illustrates the unique feature of labyrinthitis ossificans in a thalassemia patient which has not yet reported in the English literature.

Hearing screening of all thalassemia patients and therefore early diagnosis of SNHL prompts early intervention and improved quality of life.     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

Cochlear implantation in children

Cochlear implantation has revolutionized the treatment and prognosis of children with severe to profound sensorineural hearing loss who receive limited benefits from hearing aids. Children who receive cochlear implantation at young age, in particular before 2 years of age, can be expected to reach their normal age-equivalent developmental milestones and have higher chance to integrate into the mainstream educational settings. With the positive outcomes after cochlear implantation and the improvements in technology and surgical techniques, candidacy for cochlear implantation in children has been expanding to include hearing-impaired children with significant residual hearing, severe inner ear malformations, multiple handicaps such as mental retardation or visual impairment, and auditory neuropathy. Furthermore, there is growing interest in offering bilateral cochlear implantation to give children the benefits of binaural hearing. As the candidacy criteria expand, cochlear implant programs including preoperative evaluation, surgery, and habilitation have become more complex. Therefore, candidates should be selected prudently by multidisciplinary approach and cochlear implantation in children is much better to be provided by experienced cochlear implant team consisting of experts in relevant fields for the best results.     

Development of auditory and language skills in children using cochlear implants with two signal processing strategies

IntroductionThe increase in the spectral information offered by the sound processing strategy HiRes 120 has led to great expectations for the pediatric population. Due to a shorter duration of auditory deprivation and higher neural plasticity, children could benefit more substantially from the spectral information of this sound processing strategy.ObjectiveTo compare auditory and language skills in Brazilian children with cochlear implants using the HiRes and HiRes 120 sound processing strategies.MethodsThirty children, aged 1–3 years, with congenital hearing loss, were divided into two groups, according to the signal processing strategy adjusted at the time of the cochlear implant activation. The assessed children were matched according to chronological age and the time of the cochlear implant use. The auditory and language skills were evaluated longitudinally through the Infant-Toddler Meaningful Auditory Integration Scale and Production Infant Scale Evaluation, carried out before surgery, and 3, 6 and 12 months after device implantation. The Mann–Whitney test was applied for the comparison between the two groups with a 5% significance level.ResultsThe findings indicated development of hearing and language skills in the first year of cochlear implant use; however, there was no statistically significant difference in the evolution of such skills due to the adjusted processing strategy in the activation of the cochlear implant electrodes.ConclusionThe development of auditory and language skills in the assessed children was similar during the entire study period, regardless of which signal processing strategy was used.     

Cochlear implantation in autistic children with profound sensorineural hearing loss

Introduction

Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment.

Cochlear implantation in patients with Meniere's disease

Abstract

Objectives

Few studies have addressed the benefits of cochlear implantation for the small group of patients with bilateral, end-stage Meniere's disease, or unilateral disease with contralateral hearing loss from another cause. Our retrospective study evaluates the effectiveness and post-operative performance in these Meniere's disease patients and discusses these findings relative to other post-lingually deafened adults.

Methods

Among 456 adults who received cochlear implants, we identified eight (1.7%) patients with Meniere's disease who met clinical indications for implantation and a control group of seven non-Meniere's patients of comparable age and deafness. Data gathered included demographics, auditory and vestibular symptoms pre-implantation, and clinical course after implantation, including audiometric scores (consonant-nucleus-consonant (CNC), hearing-in-noise testing (HINT), and AzBio score) and audiologic evaluations.

Results

Comparing the Meniere's and non-Meniere's patients, pre- to post-implant scores averaged 15 and 16%–57 and 76% for CNC; and 4.5 and 40.5%–78 and 81% for AzBio and/or HINT tests, respectively; scores between groups did not statistically differ. Of note, five of eight patients had 0% word recognition scores pre-operatively. Fluctuations in CI performance were seen in five patients with Meniere's disease but not for those without the disease.

Conclusions

Audiologic testing found a strong likelihood that CIs did benefit our subset of Meniere's patients with severe to profound sensorineural hearing loss. Fluctuations in hearing sensitivity (perhaps owing to physical changes relative to the implant array with the spiral ganglion neurons during an acute attack or changes in spiral ganglion neurons because of the hydropic state) can often be resolved by subsequent reprogramming.     

Cochlear implant outcomes in children with motor developmental delay

Introduction

Multiple handicapped children and children with syndromes and conditions resulting additional disabilities such as cerebral palsy, global developmental delay and autistic spectrum disorder, are now not routinely precluded from receiving a cochlear implant. The primary focus of this study was to determine the effect of cochlear implants on the speech perception and intelligibility of deaf children with and without motor development delay.

Method

In a cohort study, we compared cochlear implant outcomes in two groups of deaf children with or without motor developmental delay (MDD). Among 262 children with pre-lingual profound hearing loss, 28 (10%) had a motor delay based on Gross Motor Function Classification (GMFC). Children with severe motor delays (classification scale levels 4 and 5) and cognitive delays were excluded. All children completed the Categories of Auditory Perception Scales (CAP) and Speech Intelligibility Rating (SIR) prior to surgery and 24 months after the device was activated.

Result

The mean age for the study population was 4.09 ± 1.86 years. In all 262 patients the mean CAP score after surgery (5.38 ± 0.043) had a marked difference in comparison with the mean score before surgery (0.482 ± 0.018) (P = 0.001). The mean CAP score after surgery for MDD children was 5.03, and was 5.77 for normal motor development children (NMD). The mean SIR score after surgery for MDD children was 2.53, and was 2.66 for NMD children. The final results of CAP and SIR did not have significant difference between NMD children versus MDD children (P > 0.05).

Conclusion

Regarding to the result, we concluded that children with hearing loss and concomitant MDD as an additional disabilities can benefit from cochlear implantation similar to those of NMD.     

Cochlear implant in patients with residual hearing

Objective: The postoperative speech perception abilities of severely hearing-impaired patients with multi-channel cochlear implant were compared with preoperative speech perception performance with conventional hearing aids. Methods: Cochlear implantation was performed in six severely to profoundly hearing-impaired patients. They had unaided pure-tone thresholds of 70–100-dB HL and aided thresholds of 35–90-dB HL in the better ear, but were not able to perceive speech sounds well with hearing aids. Results: Postoperatively, all the patients had significantly improved speech perception performance, exceeded the average skills of profoundly deaf cochlear implant users, and were able to communicate without writing. Conclusion: These results imply that cochlear implant may be indicated for severely to profoundly deaf subjects, if they receive little or no benefit from conventional hearing aids.     

Cochlear implant in Cogan’s syndrome

Post-lingual deafness is a stressful condition which is rendered even more painful by the sudden emotional isolation that the patient suffers. Cogan’s syndrome is a rare autoimmune cause for post-lingual deafness characterized by non-syphilitic interstitial keratitis, bilateral audio vestibular deficiencies and systemic vasculitis. World over very few cases of Cogan’s syndrome have been reported. Cochlear implant surgery in such a patient is a challenging but highly satisfactory experience due to the multitude of clinical problems the patient faces. This demands a proper work up, meticulous surgery and stringent post-operative follow-up. Here we present a patient with atypical Cogan’s syndrome, diabetes mellitus and hypothyroidism. She went into a major depression with suicidal tendency following the complete loss of hearing. We performed cochlear implant surgery in this patient, but not before facing several clinical obstacles, helped by a dedicated team consisting of a rheumatologist, endocrinologist, neurophysician, psychiatrist, anaesthetists and audiologist. The results are extremely satisfying for the patient and all the people involved. This case underlines the prime importance of hearing in maintaining the psychological well being of a human being.     

Profile of patients assessed for cochlear implants

IntroductionKnowledge of the characteristics related to profound hearing loss is a matter of great importance, as it allows for the etiological and prognostic identification and strategic planning for public health interventions.ObjectiveTo assess the different etiologies of hearing loss, age at diagnosis of the hearing loss, its relation to language acquisition, and the age at the first consultation in this service for cochlear implant assessment.MethodsThis was a historical cohort, cross-sectional study, using retrospective analysis of the records of 115 patients with confirmed sensorineural hearing loss, who were followed in a university hospital, based on gender, age of hearing loss, age at the first consultation, language, and hearing loss etiology.Results and conclusionThe majority of patients assessed for cochlear implants attend the first consultation when they are older than one year (an alarming mean of 3.8 years in the prelingual group) in spite of the early diagnosis of hearing loss. This reflects an already deficient health care system, in terms of referral. The idiopathic cause remains the most frequently identified. Among the known causes, the most prevalent are perinatal causes and meningitis.     

蜗神经磁共振成像在感音神经性聋中的应用

目的探讨蜗神经磁共振成像在感音神经性聋(SNHL)中的应用价值。方法采用GE TwinExct1.5T磁共振扫描仪对130例(260耳)SNHL患者进行蜗神经磁共振成像及内耳水成像。蜗神经成像序列为斜矢状位FRFSET2加权像,内耳水成像为3DFIESTA序列。结果 90例大于等于18岁的患者中,85例170耳显示蜗神经、迷路正常;1例2耳前庭导水管扩大;1例1耳蜗神经信号缺失;3例6耳蜗神经细小;40例小于18岁的患者中,19例38耳显示蜗神经、迷路正常;2例3耳Michel畸形;6例12耳Mondini畸形;1例2耳共腔畸形;12例24耳前庭导水管扩大;21例畸形中7例14耳蜗神经信号缺失。2例4耳显示内听道狭窄伴蜗神经细小。结论磁共振内耳成像对诊断SNHL有着重要的价值,对判断患者内耳蜗神经发育情况及内耳畸形有着不可替代的作用,是这类患者进行人工耳蜗植入术前必要检查。     

Cochlear implantation following cerebellar surgery

Abstract

Cochlear implantation in patients with known central nervous system conditions can result in wide-ranging outcomes. The aim of this study is to report two cases of cochlear implantation outcomes in patients with acquired cerebellar ataxia following cerebellar surgery. The first is a female implanted with the Nucleus 24 implant in September 2000 and the second is a male implanted with a MED-EL Sonata Flexsoft electro-acoustic stimulation in July 2009. Programming these patients resulted in significant non-auditory stimulation which resulted in less than optimum map fittings. The patients did not gain any open set speech perception benefit although both of them gained an awareness of sound with the device. However, patient 2 elected to become a non-user because of the limited benefit.     

Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review

IntroductionCurrently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants.ObjectiveReview the available evidence in the literature to answer the following: “What is the performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants?”MethodsSystematic review of the literature through electronic database consultation, considering publications in the period 2002–2013.ResultsTwenty-two studies met the criteria and were included in the systematic review.ConclusionThe analyzed studies demonstrated that after cochlear implant surgery, individuals with auditory neuropathy spectrum disorder improved their performance of hearing skills and had similar performance to that of children with sensorineural hearing loss using cochlear implant.     

Association between interleukin-6 polymorphism in the −174 G/C region and hearing loss in the elderly with a history of occupational noise exposure

IntroductionThe biological processes involved in noise-induced hearing loss (NIHL) are still unclear. The involvement of inflammation in this condition has been suggested.ObjectiveTo investigate the association between interleukin – 6 (IL-6) polymorphism and susceptibility to NIHL.MethodsThis was a cross-sectional study with a sample of 191 independent elderly individuals aged >60 years of age. Information on exposure to occupational noise was obtained by interviews. Audiological evaluation was performed using pure tone audiometry and genotyped through PCR by restriction fragment length polymorphism – PCR-RFLP. Data were analyzed using the chi-square test and the odds ratio (OR), with the significance level set at 5%.ResultsAmong elderly with hearing loss (78.0%), 18.8% had a history of exposure to occupational noise. There was a statistically significant association between the genotype frequencies of the IL-6 −174 and NIHL. The elderly with the CC genotype were less likely to have hearing loss due to occupational noise exposure when compared to those carrying the GG genotype (OR = 0.0124; 95% CI 0.0023–0.0671; p < 0.001).ConclusionThis study suggests there is an association of polymorphisms in the IL-6 gene at position – G174C with susceptibility to noise-induced hearing loss.     

Prevalence of potential candidates for electric-acoustic stimulation implant in a hearing-impaired population

ObjectiveTo estimate the prevalence of potential electric-acoustic stimulation (EAS) implant candidates in a hearing-impaired population through a review of auditory examinations.MethodsIn total, 7356 patients underwent audiometric examination in our department between 2011 and 2014. The prevalence of patients meeting the audiometric criteria for EAS and standard cochlear implant (CI) was assessed.ResultsThe percentage of EAS implant candidates meeting the pure-tone audiometric criteria was 0.71% (n = 34) among the hearing-impaired individuals (n = 4758) examined in our department, whereas 2.52% (n = 120) met the criteria for standard CI. Among the 34 EAS implant candidates, 2 individuals (5.83%) received EAS implant surgery after approval of the EAS device in Japan.ConclusionsThere was a lower prevalence of EAS implant candidates than standard CI candidates. Nevertheless, healthcare professionals should carefully examine the audiograms of patients with high frequency hearing loss with regard to meeting the indication criteria for EAS implant. This will enable patients to gain access to adequate information relating to further examinations and treatment options.