Prognostic factors for vestibular impairment in sensorineural hearing loss

The clinical course and prognosis in sensorineural hearing loss (SNHL) may be even worse if vestibular system is also involved, especially due to near location of anatomical structures in the inner ear. The aim of the study was to determine prognostic value of some clinical, audiological and demographic factors associated with SNHL in predicting a possibility of vestibular impairment. The study was conducted on 124 consecutive patients (183 ears) diagnosed for sensorineural hearing loss during 1 year in our department. In all of them, audiological (pure-tone, speech and impedance audiometry, ABR) and ENG examinations (visual ocular–motor, positional, kinetic and caloric tests) were performed. The correlations between ENG outcome and the following variables associated with sensorineural hearing loss were investigated: audiological (degree and location of hearing loss, audiogram configuration), clinical (tinnitus, vertigo, dizziness) and demographic (age, sex) factors. Normal ENG was recorded in 26.6%, vestibular impairment of peripheral type in 38.7%, and central type in 34.7% of the patients. In a multivariate stepwise linear regression analysis, the degree of hearing loss was the main variable correlating with abnormal ENG result. Tinnitus and location of hearing loss were also found to be the two other variables which, to some minor extent, can influence the ENG outcome. Peripheral vestibular impairment was observed more frequently in patients with residual hearing/deafness. The degree of hearing loss, presence of tinnitus and location of hearing loss are factors predicting the possibility of abnormal ENG outcome in sensorineural hearing loss.     

The age of diagnosis of sensorineural hearing impairment in children

Objective: To identify factors responsible for delays in diagnosis and treatment of pediatric sensorineural hearing impairment (SNHI), and to assess the thoroughness of medical evaluation in these children. Design: Retrospective analysis. Setting: State-supported school for the deaf. Patients and other participants: 291 children with SNHI, the vast majority of whom are profoundly hearing impaired. Data were collected from the school's database, individual student records, and a parental questionnaire. Main outcome measures: (1) The age of diagnosis and treatment of SNHI; (2) factors leading to a delay in diagnosis; (3) current medical evaluations used to determine the etiology of SNHI; and (4) the level of parental satisfaction with the evaluation process. Results: Many children with SNHI experience delays in diagnosis from the time of first suspicion of hearing loss. Children with a risk factor for SNHI are diagnosed no earlier than children without a risk factor. Caucasian children are diagnosed significantly earlier than either Black or Hispanic children, regardless of socioeconomic status. Inconsistent medical evaluation ensues following the diagnosis of SNHI, and parental satisfaction with this process is low. Conclusions: The average age of diagnosis of SNHI remains unacceptably high. There exists a need to enhance physician awareness of childhood deafness and to develop guidelines for the medical evaluation in cases of pediatric SNHI. Lastly, the importance of parental concern regarding a child's hearing or language development must be re-emphasized.     

Simulation of sensorineural hearing impairment

A hearing loss simulation system (HELOS) was designed and constructed to simulate various aspects of sensorineural hearing impairment. The theoretical bases for HELOS were several threshold and suprathreshold auditory phenomena typically exhibited by people with sensorineural hearing losses. In addition to providing differential attenuation of acoustic signals across the frequency range, HELOS simulated loudness recruitment, loudness discomfort thresholds, reduced dynamic range, and reduced frequency selectivity. Three basic audiometric configurations were chosen to investigate the effects of the aforementioned components of a sensorineural hearing impairment on the auditory perception of speech. They were: (1) a sloping high-frequency hearing loss; (2) a flat, severe hearing loss; (3) a severe/profound hearing loss. The battery of tests administered to a group of normal-hearing adults consisted of pure-tone audiometry and PB-word recognition tests, as well as vowel and consonant identification tests. For comparable audiometric configurations, the results from the speech-perception tests were in good agreement with the published results of similar tests administered to persons with sensorineural hearing losses.     

Risk factors for sensorineural hearing loss in children

In the last decade, tremendous progress has been made very rapidly in the development of Early Hearing Detection and Intervention (EHDI) systems as a major public health initiative. The percentage of infants screened annually in Spain has increased significantly since the EHDI systems have expanded to all autonomic regions. Historically, high risk indicators have been used for the identification of infants who should receive audiological evaluation but who live in geographic locations where universal hearing screening is not yet available, to help identify infants who pass neonatal screening but are at risk of developing delayed-onset hearing loss and to identify infants who may have passed neonatal screening but have mild forms of permanent hearing loss. In this review, the standard risk factors for hearing loss are analysed and the risk factors known to be associated with late onset or progressive hearing loss are identified. The recommendation for infants with a risk factor that may be considered as low risk is to perform at least one audiology assessment by 24-30 months. In contrast, for an infant with risk factors known to be associated with late onset or progressive hearing loss (such as cytomegalovirus infection or family history), early and more frequent assessment is appropriate. All infants should have an objective standardised screening of global development with a validated assessment tool at 9, 18 and 24-30 months of age or at any time if the health care professional or the family is concerned.     

Pitch vulnerability in sensorineural hearing impairment

Listeners with cochlear impairment were studied in an ABX pitch-matching paradigm. These listeners showed excellent ability to adjust one sinusoid (X) to match the frequency of another (B), except when a leading tone (A) was present. Pitch shifts induced by the leading tone exaggerate the pitch contrast between tones A and B, even in normal-hearing listeners, but the effect in sensorineural listeners is several times larger and indicates a severe vulnerability of pitch. The degree of pitch distortion does not appear to bear any simple relationship to the degree of hearing loss, or to stimulus amplitudes. The effect is obtained at 'comfortable' listening levels. Vulnerability of pitch, rather than a failure in discriminative capacity, may underlie some of the qualitative perceptual difficulties found in sensorineural hearing impairment.     

Middle ear disease in young children with sensorineural hearing loss

Early identification of children with sensorineural hearing loss (SNHL), coupled with the provision of appropriate conventional amplification constitute important elements of a (re)habilitative strategy. Often overlooked, however, is the problem posed by recurrent and chronic middle ear disease in the child with coexisting SNHL. This study focuses on 437 (237 boys and 200 girls) consecutive patients with bilateral SNHL, in the moderate range or poorer, who were diagnosed prior to age 5 years at Boys Town National Research Hospital. The mean threshold shift with effusion was 25.0 dB at 250 Hz, 28.5 dB at 500 Hz, 29.5 dB at 1000 Hz, 24.5 dB at 2000 Hz, and 27.5 dB at 4000 Hz. During a median follow-up period of 3.21 years, 154 (35.2%) of these children required surgical placement of tympanostomy tubes because of the severity of middle ear disease and its impact on auditory acuity. Accurate determination of the degree and audiologic configuration of a SNHL may be seriously hindered by a coexisting middle ear effusion. If initial findings indicate the presence of a middle ear effusion, measures of auditory function must be repeated after the effusion has been resolved by medical and/or surgical intervention.     

Pathology of vascular sensorineural hearing impairment

Circulatory disorders, which are well documented in most parts of the human body, are not well documented in the inner ear, although they are expected to occur. It has been previously shown that experimental occlusion of the labyrinthine artery in animals results in severe degenerative changes, fibrosis, and new bone formation in the cochlea. Accordingly, this paper presents presumptive evidence that depriving the human cochlea of its blood supply after surgical removal of an acoustic tumor results in severe degenerative changes that progress to total ossification of the cochlear spaces. This paper also discusses similar changes seen in temporal bones of two patients with sudden sensorineural hearing impairment. Based on these observations, the author concludes that occlusive arterial disease, whether thrombotic, embolic, or spastic, plays a role in some sudden sensorineural hearing losses. Cochlea ossification, detected by polytomography, suggests a vascular etiology of the hearing impairment. Treatment with vasodilator drugs and anticoagulants is justified in these cases until a better diagnostic protocol is developed to eliminate other causes.     

Symposium on sensorineural hearing loss in children: early detection and intervention. Evaluation of the child with sensorineural hearing impairment.

When the physician is confronted with a child with sensorineural hearing impairment, he should do everything in his power to establish the cause of the hearing loss in order to diagnose those conditions that might be corrected or controlled and to counsel the parent concerning the child's future. Certain physical characteristic should alert the physician to the fact that there may be an accompanying sensorineural hearing loss. The family, gestational, and postnatal history may help establish this diagnosis. Finally, laboratory and x-ray examinations should be performed in an attempt to arrive at a definitive diagnosis.     

Hearing loss in school children. A longitudinal study of sensorineural hearing impairment

In a longitudinal study, 2325 children were hearing tested at age 7, 10 and 13 with screening audiometry. The screening level was 20 dB HL. Approximately 75% of the children passed the screening level at all ages. Hearing loss was more frequent in boys than in girls at age 13 (16%:9%). The left ear was more commonly affected than the right ear. High frequency dips increased for boys with age, but not for girls. The increasing incidence of hearing loss for boys with age is probably due to noisy leisure time activities.     

Progressive sensorineural hearing impairment in professional fishery divers

There have been many reports of a high rate of hearing impairment in divers. A prospective study was performed to determine whether sensorineural hearing acuity in the same divers deteriorated faster than in a normal population as they continued diving. After an observation period of approximately 5 years, audiometric examination was performed on a group of professional fishery divers who had normal hearing or sensorineural hearing loss at the time of initial study. Thirty-three ears of 18 divers were included in statistical analyses. The average hearing deterioration in the divers in 5 years, after elimination of the aging effect, was 6.6 dB (SD 4.5) and was statistically significant. We concluded that the hearing acuity of our subjects deteriorated faster than that of normal Japanese.     

Fluctuating sensorineural hearing loss in children

Childhood sensorineural hearing loss (SNHL) that fluctuates or is progressive enhances parental concern and complicates medical management, hearing aid selection, and individualized educational planning for the affected child. Despite intensive multidisciplinary evaluation and intervention, continued threshold fluctuation or a gradual decline in auditory acuity may proceed unabated in a significant percentage of these youngsters. With the adoption of universal newborn hearing screening mandates by an increasing number of states, any challenges to the accurate determination of auditory thresholds must be addressed within the first few months of life.     

儿童突发性感音神经性聋发病特点分析

目的 :探讨儿童突发性感音神经性聋 (突发性聋 )的发病特点 ,以指导临床诊断与治疗。方法 :总结2 1例 14岁以下突发性聋患儿 (儿童组 )的临床表现及预后等 ,并与 6 7例成年突发性聋 (对照组 )进行对比分析。结果 :儿童组中 11例 (5 2 .4 % )查到可能的病因 ,其中腮腺炎 4例 (19% )、上呼吸道感染 3例 (14 .3% )、耳毒性药物中毒 2例 (9.5 % )、声损伤和代谢性脑病各 1例 ;对照组 19.4 %找到可疑的病因。入院时儿童组听力受损平均88.6dB ,对照组 80 .2dB ,两组差异有统计学意义 (P <0 .0 5 ) ;治疗后儿童组听力平均提高 14 .3dB ,对照组提高37.8dB ,两组差异有统计学意义 (P <0 .0 5 )。结论 :儿童突发性聋的特点多有明确病因 ,其中以病毒感染多见 ,其耳聋程度重 ,预后较成人差     

听力损失与突发性聋预后的关系

目的：探索听力损失对突发性聋预后的影响。方法:对146例（167耳）突聋患者的临床资料进行回顾性分析,应用SAS 统计软件对纯音听阈情况与预后的关系进行统计学处理。结果:单因素分析显示,初诊时听力损失曲线为全聋型,纯音高频听阈（4?000?Hz及8?000?Hz）损失较重,高频听阈（4?000?Hz+8?000?Hz）大于低频(250?Hz+500?Hz)听阈的患者预后不佳;多因素分析显示,8?000?Hz的听阈水平与预后关系最为密切,其次为4?000?Hz听阈及听力损失类型。结论：从单、多因素分析的结果可以看出,对突聋应采用多因素逐步分析的方法进行研究,以使预后和疗效评估更具准确性和客观性;高频听力损失程度是突发性聋预后的一个可靠评估因素。     

Progression of sensorineural hearing impairment in aided and unaided ears

The possibility of "adult onset auditory deprivation" has been proposed as a condition of extensive deterioration of speech discrimination in unaided ears. Pure tone thresholds and speech discrimination were studied in a follow-up examination on 500 patients using hearing aids unilaterally. The follow-up time ranged from 5 to 24 years. In average the same amount of deterioration was obtained in both ears. Deterioration in pure tone thresholds increased after the age of 80 years and speech discrimination after 65 years respectively. The results obtained gave no support to the concept of adult onset auditory deprivation in unaided ears.     

Idiopathic sudden sensorineural hearing loss in children

OBJECTIVE: Although idiopathic sudden sensorineural hearing loss (ISSHL) is a frequent disease in adults, less is known about incidence and treatment of ISSHL in children. METHOD: A retrospective chart analysis was performed to evaluate the frequency of ISSHL in children aged under 18 years between 2000 and 2003, who were treated in our department. Children received prednisolone intravenously at an initial dose of 3mg/kg bodyweight. Prednisolone dose was reduced to half every second day. The medication was given for a maximum of 14 days or finished 2 days after the hearing normalized in pure-tone audiometry. The follow-up was continued between 3 and 14 months. RESULTS: The complete recovery rate was 57%, and the partial recovery was 36%. Initial hearing loss of 50dB and more was predictive for poor outcome in children (p=0.028). Presence of tinnitus was without relevance for the outcome. The incidence of ISSHL in the local area of about 250,000 inhabitants was 1/10,000 in children. CONCLUSION: ISSHL seems to be a less frequent disease in children than in adults. Severe initial hearing loss is coupled with poor outcome. Under treatment with prednisolone hearing improvement was found in 13 of 14 patients.