EI services represent the purpose and goal of the entire EHDI process. Screening and confirmation that a child is D/HH are largely meaningless without appropriate, individualized, targeted and high-quality intervention. For the infant or young child who is D/HH to reach his or her full potential, carefully designed individualized intervention must be implemented promptly, utilizing service providers with optimal knowledge and skill levels and providing services on the basis of research, best practices, and proven models. The delivery of EI services is complex and requires individualization to meet the identified needs of the child and family. Because of the diverse needs of the population of children who are D/HH and their families, well-controlled intervention studies are challenging. At this time, few comparative effectiveness studies have been conducted. Randomized controlled trials are particularly difficult for ethical reasons, making it challenging to establish causal links between interventions and outcomes. EI systems must partner with colleagues in research to document what works for children and families and to strengthen the evidence base supporting practices.

Objective: To report the adverse effects associated with prolonged high-dose prednisone for the treatment of autoimmune inner ear disease (AIED).Study Design: Prospective data collected as part of a multi-center, randomized, controlled trial for the treatment of corticosteroid-responsive AIED with methotrexate.Setting: Tertiary referral centers.Patients: One hundred sixteen patients with rapidly progressive, bilateral sensorineural hearing loss.Intervention: All patients completed a 1-month course of prednisone (60 mg/d). In Phase 2, 67 patients with improvement in hearing underwent a monitored 18-week prednisone taper, resulting in 22 weeks of prednisone therapy at an average dose of 30 mg per day. Thirty-three patients were randomized to receive methotrexate in Phase 2. Thirty-four patients received prednisone and placebo.Main Outcome Measure: Adverse events (AE) in patients treated with prednisone only.Results: Of 116 patients, 7 had to stop prednisone therapy during the 1-month challenge phase due to AE. Of 34 patients, 5 were unable to complete the full 22-week course of prednisone due to AE. The most common AE was hyperglycemia, which occurred in 17.6% of patients participating in Phase 2. Weight gain was also common, with a mean increase in body mass index of 1.6 kg/m(2) (95% confidence interval, 0.77-2.3) during the 22-week steroid course. Patients entering Phase 2 were followed for a mean of 66 weeks. No fractures or osteonecrosis were reported.Conclusion: Although high-dose corticosteroids are associated with known serious side effects, prospective data in the literature are limited. The present study suggests that with appropriate patient selection, monitoring, and patient education, high-dose corticosteroids are a safe and effective treatment of AIED.

OBJECTIVE:We analyzed pure-tone and speech audiometric results from a prospective trial of anti-inflammatory treatment of subjects with active autoimmune inner ear disease (AIED). We sought to characterize the pattern and size of the treatment effect as reflected in clinical audiometry and to identify audiometric predictors of response to steroid treatment of AIED.SUBJECTS:Adult participants demonstrated clinically established criteria for AIED (n = 116). Eligibility required audiometric evidence of active AIED as indicated by idiopathic sensorineural hearing loss with threshold elevations within 3 months of enrollment.METHODS:We evaluated audiometric changes after 4 weeks of treatment with pharmacologic doses (60 mg/day) of prednisone. We examined the relationship between audiometric pure-tone thresholds at baseline and changes in word intelligibility score (WIS) using parametric and nonparametric analyses. Magnitudes of change were assessed using independent or paired t-tests. Separate analyses were performed on subgroups that did or did not show improved WIS score with steroid treatment.RESULTS:Overall mean pure-tone averages improved from baseline to closeout of prednisone treatment in better hearing ears from 52.4 to 48.3 dB (p < .0001). Mean WIS improved in the better ear from 71.4% to 78.1% (p < .0001). Of pure-tone measures, only the six-tone average showed significant correlation with both the absolute improvements in WIS and with the percentage change in WIS after treatment. Individual frequencies at baseline showed no significant relationship with changes in WIS score after treatment. In 69 (59.5%) of 116 subjects, WIS improved (range, 2-80%) in the better ear. In these subjects, the baseline pure-tone thresholds and pure-tone averages correlated significantly and positively with improvement in WIS.CONCLUSIONS:Steroid treatment in AIED-mediated hearing loss produce variable but significant hearing gains. Neither a focal, cochleotopic region of greatest vulnerability to AIED nor frequency-specific amenability to treatment were evident. We did observe that analysis of predictors and the degree of treatment effect vary with different approaches to measuring change in the WIS. Depending on the approach adopted, the size of the treatment effect may be greatest across intermediate hearing levels at baseline. These observations offer an audiometric database that may enable greater precision in judging clinically meaningful parameters for future studies of AIED treatment and other interventions for sensorineural hearing loss.

Contexte Un certain nombre de traitements ont été proposés dans la prise en charge au long cours des pertes auditives bilatérales neuro-sensorielles, rapidement progressives, répondant à la corticothérapie (atteinte auto-immune de l'oreille interne [AIED]). Le méthotrexate émerge comme le traitement de choix, mais il n'a pas été évalué rigoureusement chez les patients atteintes de AIED.

CONTEXTA number of therapies have been proposed for the long-term management of corticosteroid-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease [AIED]). Methotrexate has emerged as the benchmark agent but has not been rigorously evaluated for hearing improvement in patients with AIED.OBJECTIVETo assess the efficacy of long-term methotrexate in maintaining hearing improvements achieved with glucocorticoid (prednisone) therapy in patients with AIED.DESIGN, SETTING, AND PARTICIPANTSA randomized, double-blind, placebo-controlled trial conducted from February 3, 1998, to November 5, 2001, of 67 patients with rapidly progressive, bilateral sensorineural hearing loss at 10 tertiary care centers in the United States.INTERVENTIONRandomization to either oral methotrexate (15 to 20 mg/wk; n = 33) or placebo (n = 34), in combination with an 18-week prednisone taper. Follow-up examinations, including audiometric evaluation, were performed at 4, 8, 12, 24, 36, 48, and 52 weeks, or until hearing loss was documented.MAIN OUTCOME MEASUREMaintenance of hearing improvement achieved from prednisone treatment.RESULTSSixty-seven patients (57.8%) enrolled in the prednisone challenge experienced hearing improvement. Twenty-five patients (37%) experienced hearing improvements in both ears. Of the individuals who reached study end points, 24 (80%) of 30 end points were because of measured hearing loss in the methotrexate group and 29 (93.5%) of 31 end points were because of measured hearing loss in the placebo group (P =.15). Methotrexate was no more effective than placebo in maintaining the hearing improvement achieved with prednisone treatment (hazard ratio, 1.31; 95% confidence interval, 0.79-2.17; P =.30).CONCLUSIONMethotrexate does not appear to be effective in maintaining the hearing improvement achieved with prednisone therapy in patients with AIED.

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.

The management of infants and children with sensorineural hearing loss (SNHL), particularly ii newly identified, should involve thee coordinated efforts of pediatricians, otolaryngologists, audiologists, and aural habilitation specialists. Parent counseling and interaction with local school districts to advocate for the child with a hearing loss may also be required as the family is confronted by a series of difficult choices about the child's care, treatment, and education. This article addresses the management of common problems, such as otitis media in children with SNHL, and provides an overview of sensory aids, such as conventional hearing aids and cochlear implants.

From Boys Town National Research Hospital and the Department of Otolaryngology and Human Communication, Creighton University School of Medicine, Omaha, NE. Address for reprints: Boys Town National Research Hospital, 555 N. 30th St., Omaha, NE 68131-2136. Fax 401-498-6357.

Hearing loss in infants and children may be sensorineural, conductive, or mixed. Severity varies from mild to profound. Educational initiatives aimed at children, parents, and primary health care providers could help prevent needless permanent hearing impairment. Effective programs aimed at education and hearing conservation among children and adolescents are overdue. The causes of sensorineural hearing loss, the concept of multidisciplinary team evaluation, and measurement of hearing are discussed. Advances in genetics of hearing loss are reviewed.

This paper describes audiologic, electrophysiologic, and medical test results for a now 10-year-old girl who has had 45 episodes of reversible, sudden sensorineural hearing loss over the last 8 years. Episodes have lasted from 6 to 72 hours and often have been accompanied by a mild illness. Acoustic immittance measures have been consistent with normal middle-ear function with the exception of absent ipsilateral and contralateral acoustic reflexes. Mechanically evoked perioral reflex activity was markedly asymmetric following lower lip stimulation. The asymmetry of R1 activation between right and left side lower lip inputs raises questions about the integrity of central connections within the brain stem, including internuncial pathways coursing between trigeminal sensory relay nuclei and the facial motor nucleus. An electrocochleographic evaluation revealed cochlear microphonic but absent or markedly abnormal whole nerve action potentials. Auditory brainstem responses (ABR) have been either absent or poorly formed and significantly delayed, regardless of hearing sensitivity. Middle and late auditory evoked potentials were essentially normal. Both transient-evoked and distortion-product otoacoustic emissions were present regardless of peripheral auditory sensitivity. All medical tests have been essentially normal. Although no definitive diagnosis has been reached, beta blockers have been used with some success. Taken together, these data document a very unusual case of fluctuating hearing loss. The electrocochleographic and otoacoustic emission data suggest that the outer hair cells are functioning normally and that the loss is not cochlear in origin.(ABSTRACT TRUNCATED AT 250 WORDS)

One goal of the Americans with Disabilities Act is to enhance access to career opportunities for individuals with hearing loss. Hearing-impaired professionals are woefully underrepresented among the cadre of scientists currently involved in hearing and deafness research. Information was obtained by questionnaire from 190 consecutive attendees (13 through 17 years of age) at a summer program for gifted hearing-impaired adolescents regarding career goals, attitudes toward academic and extracurricular activities, educational placement, primary communication modality, and parental hearing status. A follow-up questionnaire completed by 80 of these youth, presently attending college, provided comparison data regarding type of college attended and academic major. Males were significantly more likely to select majors in mathematics and science-related disciplines. The percentage of college attendees majoring in the sciences was much lower than the percentage of high school students who aspired to a scientific career. Strategies for attracting qualified hearing-impaired students into science majors should include educational efforts directed at students, parents, and academic advisors.

Sounds of sufficient loudness and duration will damage the human ear resulting in temporary or permanent hearing loss, often accompanied by tinnitus. Irreversible inner ear damage from repeated sound overstimulation can occur at any age, including early infancy, resulting in permanent noise-induced hearing loss. A typical noise-induced hearing loss impairs the understanding of speech sounds, which could degrade performance in the classroom and on the job. Utilizing current knowledge about ear protection, noise-induced-hearing loss is entirely preventable except in cases of accidental exposure. Unfortunately, widespread and consistent use of appropriate ear protection devices by individuals at risk has been an elusive public health goal.

Sensorineural hearing loss (SNHL) which is sudden in onset, fluctuating, and/or progressive complicates medical management, hearing aid selection, and individualized educational planning for a hearing-impaired child. In spite of multidisciplinary evaluation and intervention, a gradual decrease in auditory acuity may continue unabated in a significant number of cases. Intercurrent middle ear disease and inconsistent audiologic technique can account for threshold variation in some cases. The study population consisted of 229 children (132 boys; 97 girls) aged 1 to 19.9 years at first audiogram which revealed at least a mild degree of sensorineural hearing loss in one or both ears (35 unilateral), and who demonstrated threshold variation of 10 dB or more in at least one ear at one or more of the standard audiometric test frequencies (250, 500, 1000, 2000, 4000, and 8000 Hz) and were without concurrent middle ear disease (mean length of follow-up, 4.9 years; mean number of audiograms, 10.3). Of 365 ears demonstrating threshold variation of 10 dB or more, 22 (6%) had purely progressive losses without intercurrent upward fluctuation, 208 (57%) had fluctuating thresholds with gradually progressive losses, and 135 (37%) had intermittent threshold fluctuation without permanent deterioration. The probability of contralateral threshold fluctuation if one ear fluctuated was 0.91, while the probability of contralateral progressive SNHL if one ear progressed was 0.67. Demographic data, presumptive etiology, degree of initial SNHL, audiometric configuration, and symmetry of threshold variation were considered as potential predictors of the likelihood of threshold fluctuation and/or progression.

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.

Occupational noise exposure remains the most commonly identified cause of noise-induced hearing loss (NIHL), but potentially hazardous noise can be encountered during leisure-time activities. NIHL in the pediatric population has received scant attention. This study focuses on 114 children and adolescents (ages 19 and under: 90.3% males) who were diagnosed as having probable NIHL on the basis of history and audiometric configuration. In 42 children the loss was unilateral, while the remaining 72 had sensorineural losses of varying configurations in the contralateral ear. The mean age of referral for evaluation was 12.7 years (range 1.2 to 19.8, SD 4.21), although 26% of these losses were diagnosed in children aged 10 years and younger. Such irreversible, but potentially preventable losses, should be given high priority on the public health agenda. Comprehensive, age-appropriate educational programs must be developed for elementary and secondary students and their parents to acquaint them with potentially hazardous noise sources in their environment.

This study assessed the effectiveness of a broad based psychotherapeutic intervention with a sample of 72 children sexually abused at a residential school for the deaf. An untreated comparison group emerged when about half of their parents refused the offer for psychotherapy provided by the school. Treated and untreated children were randomly assigned to two assessment groups: those who participated in a pretreatment assessment and those who did not. Houseparents at the residential school used the Child Behavior Checklist (CBC) to rate the pretreatment assessment children before treatment and all 72 children one year after the implementation of psychotherapy. Children receiving therapy had significantly fewer behavior problems than children not receiving therapy. There was a differential response to therapy on the basis of sex. Boys receiving therapy had significantly lower scores on the following CBC scales than the no treatment group: Total, Internal, External, Somatic, Uncommunicative, Immature, Hostile, Delinquent, Aggressive, and Hyperactive. There were no differences on the Schizoid and Obsessive scales. Girls receiving therapy had significantly lower scores than the no treatment group on the following CBC scales: Total, External, Depressed, Aggressive, and Cruel. There were no differences on the Internal, Anxious, Schizoid, Immature, Somatic, and Delinquent scales.