Safety of high-dose corticosteroids for the treatment of autoimmune inner ear disease.

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 multicenter, randomized, controlled trial for the treatment of corticosteroid-responsive AIED with methotrexate.

Setting: Tertiary referral centers.

Serial audiometry in a clinical trial of AIED treatment.

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).

Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial.

Context: A 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.

Objective: To assess the efficacy of long-term methotrexate in maintaining hearing improvements achieved with glucocorticoid (prednisone) therapy in patients with AIED.

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.

Optimizing effectiveness of laser tympanic membrane fenestration in chronic otitis media with effusion. Clinical and technical considerations.

Objective: To describe the patient, disease and clinical characteristics that optimize the effectiveness of laser tympanic membrane fenestration (LTMF) to treat chronic otitis media with effusion (OME).

Setting: Four pediatric otolaryngology tertiary referral centers. IRB approved; participation by informed consent.

Effectiveness of adenoidectomy and laser tympanic membrane fenestration.

Objective: Adenoidectomy alone or with tonsillectomy (A+/-T) is an effective surgical intervention in the management of otitis media in children, especially when it is performed in conjunction with insertion of pressure equalization tubes (PETs). Otorrhea and persistent tympanic membrane (TM) perforation are frequent complications. This study evaluates the effectiveness of intermediate duration middle ear ventilation using laser tympanic membrane fenestration (LTMF) without tube insertion and as an adjunct to adenoidectomy in resolving middle ear disease within the first 90 days after surgery.

Impact of tympanostomy tubes on child quality of life.

Background: The objective benefits of tympanostomy tubes for otitis media are well established, but the subjective impact of surgery on child quality of life (QOL) has not been systematically studied.

Objectives: To determine the subjective impact of tympanostomy tubes on child QOL, and to compare the variability in QOL before surgery with that observed after surgery.

Design: Prospective, observational, before-and-after trial.

Management of the child with sensorineural hearing loss. Medical, surgical, hearing aids, cochlear implants.

Optimal comprehensive management of a child with SNHL involves constructive dialogue and coordination with the child's family and teachers. Not all children with SNHL benefit from conventional amplification, even after parents have invested significant amounts of money in hearing aids. Parents may encounter frustration and disappointment if their children fail to achieve communicative and academic goals they have established in their own mind.

Sensorineural hearing loss in children.

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.

Case of recurrent, reversible, sudden sensorineural hearing loss in a child.

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.

Hearing research career development for deaf students.

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.

Prevention of noise-induced hearing loss.

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.

Fluctuating and/or progressive sensorineural hearing loss in children.

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.

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.

Noise-induced hearing loss in children.

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.

The effects of psychotherapy on behavior problems of sexually abused deaf children.

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.

Unilateral hearing loss in children.

Recent reports suggest that early onset, severe unilateral sensorineural hearing loss (USNHL) in children may be associated with significant deficits in auditory and psycholinguistic skills and school performance. This report reviews a consecutive series of 324 children and adolescents (202 males, 122 females) with documented USNHL evaluated at the Boys Town National Research Hospital. The left ear was affected in 168 (52%) and the right ear in 156 (48%).

Correlates of vestibular evaluation results during the first year of life.

Prenatal and perinatal correlates of abnormal auditory brainstem responses in neonates have been studied extensively. In contrast, vestibular function during the first year of life has received sparse attention. Using a specially modified vestibular test battery, 65 infants (17 low-risk, 48 high-risk) were initially evaluated during their first 6 months of life.

Patterns of physical and sexual abuse of communicatively handicapped children.

Evidence suggests that handicapped children are at increased risk for abuse and neglect. Communicatively impaired youngsters are particularly vulnerable because of their limited ability to report the maltreatment. Of 482 abused handicapped children evaluated at Boys Town National Research Hospital, 212 had hearing impairment, 87 speech language disorders, 39 learning disorders, 43 behavioral-emotional disturbances, 74 mental retardation, 5 visual impairment, 3 cleft lip or palate, and 19 other disorders.

Auditory brainstem response results as predictors of behavioral auditory thresholds in severe and profound hearing impairment.

Pediatric cochlear implantation is restricted to patients with stable, bilateral profound sensorineural hearing losses who derive no benefit from conventional amplification. Obtaining reliable audiologic thresholds in a young child with sudden or early-onset hearing loss can be challenging. This study examines the accuracy with which auditory brainstem response evaluation can predict unaided and aided behavioral thresholds in a child with severe-to-profound hearing loss.

Severe versus profound sensorineural hearing loss in children: implications for cochlear implantation.

The advent of cochlear implants for children has stimulated interest in the specific sensory deficits and communicative capabilities of children with severe or profound bilateral sensorineural hearing loss. Appropriate management of these children and their families requires an appreciation of the multifaceted developmental and educational challenges confronting deaf children, even after cochlear implantation. Evaluation results from 200 children with bilateral sensorineural deafness (63 severe/137 profound, anacusic, and fragmentary) reveal significant differences between these two subpopulations.

Aided auditory thresholds in children with postmeningitic deafness.

Postmeningitic hearing loss is often bilateral, profound, and rapid in onset. Children with postmeningitic hearing loss may be identified as potential candidates for cochlear implantation. The length of time required for a definitive trial with conventional amplification, which must precede a final decision regarding cochlear implant surgery, has been the subject of debate.