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. METHODS: An observational clinical effectiveness trial was conducted in 164 ears (94 children), who had chronic OME. All patients were candidates for insertion of pressure equalization tubes (PETs) but agreed to undergo LTMF instead. Clinical and audiologic follow-up are reported at 90 days after LTMF. OUTCOME MEASURES: Clinical effectiveness was defined as an effusion free middle ear at otoscopy with A or C1 tympanogram and normal hearing. RESULTS: At 90 days, 66% of the 95 evaluable ears were effusion free, all with normal hearing. Children younger than 4 years (P<0.04), who had shorter durations of effusion (P<0.009), and who experienced longer duration of fenestration patency (FP) (P<0.009) correlated to improved outcomes. CONCLUSIONS: The use of LTMF to create 2--3 weeks of middle ear ventilation in patients with chronic OME (middle ear effusion (MEE) for 3 months or greater) is effective in 66% of 95 (58%) of ears evaluable at 90 days follow-up. This study provides basic information needed to optimize the use of LTMF today and enhance research efforts in the future.

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. STUDY DESIGN: This pilot study was a multicenter, prospective clinical cohort trial. Institutional review board approval and informed consent were obtained. The study involved four tertiary care pediatric otolaryngology departments. Fifty children (96 ears) were treated with LTMF in conjunction with A+/-T from June 1, 1998, through March 30, 1999. Ages ranged from 9 months to 12 years. Patients undergoing A+/-T who would have been recommended for PET insertion instead underwent middle ear ventilation with LTMF using the Oto-LAM device (ESC/Sharplan, Yokneam, Israel). Patients were seen at 30, 60, and 90 days postoperatively. Resolution of otitis media with effusion was determined by clinical examination, which included pneumatic otoscopy, audiometry, and tympanometry. RESULTS: Of the treated ears, 88%, 86%, and 83% had clinical resolution of middle ear disease at 30, 60, and 90 days, respectively. Preoperatively, 45% (n = 85) of ears had normal hearing; 92% (n = 49) had normal hearing at 90 days. Eighty-nine percent (n = 92) had type C2 or B tympanograms preoperatively, and 12% (n = 60) had type C2 or B at 90 days. CONCLUSION: Laser tympanic membrane fenestration in conjunction with adenoidectomy was effective in restoring normal middle ear function at 90 days post-treatment in greater than 80% of children who otherwise may have had placement of PETs.

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. SETTING: Fourteen referral-based pediatric otolaryngology practices in the United States. PATIENTS: Consecutive (64%) and convenience (36%) sample of 248 children (median age, 1.4 years) with otitis media scheduled for bilateral tympanostomy tube placement as an isolated surgical procedure. INTERVENTION: Tympanostomy tubes were inserted as part of routine clinical care. Validated measures of QOL (OM-6 survey), satisfaction with health care decision (Satisfaction With Decision Scale), and satisfaction with office visit; surveys were completed at baseline (visit 1), at surgery (visit 2), and after surgery (visit 3). MAIN OUTCOME MEASURES: Short-term changes in QOL before surgery (visit 1 to visit 2) and after surgery (visit 2 to visit 3). RESULTS: Changes in QOL before surgery were mostly trivial, and were smaller than changes observed after surgery (P<.001). Large, moderate, and small improvements in QOL occurred after surgery in 56%, 15%, and 8% of children, respectively. Physical symptoms, caregiver concerns, emotional distress, and hearing loss were most improved, but significant changes were also seen for activity limitations and speech impairment. Trivial changes occurred in 17% of children, and 4% had poorer QOL. Predictors of poorer QOL were otorrhea 3 or more days (10% of variance) and decreased satisfaction with surgical decision (3% of variance). Hearing status, child age, type of otitis media (recurrent vs chronic), and office visit satisfaction were unrelated to outcome. CONCLUSIONS: Tympanostomy tubes produce large short-term improvements in QOL for most children. The best outcomes occur when postoperative otorrhea is absent or minimal, and when parents are satisfied with their initial decision to have surgery. Further research is needed to document the long-term impact of tubes on child QOL.

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. If the child is a potential candidate for cochlear implantation, this decision not only involves the risks (albeit modest) of surgery but also often a substantial financial commitment to help defray the cost of this sophisticated electronic device. The parents may encounter conflicting advice from friends and members of the adult deaf community about the benefit of cochlear implantation. Throughout the course of all of these difficult considerations, the health professionals caring for a child with hearing loss should be a source for information, guidance, and support to the family.

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. 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.

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. 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)

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. 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.

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. 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.

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. 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.

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.

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: 90.3% 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.

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. 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.

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%). Based on speech frequency threshold averages (i.e., 500, 1000, and 2000 Hz), the losses were classified by severity as follows: borderline, 43 (13%); mild, 51 (16%); moderate, 40 (12%); severe, 19 (6%); profound, 31 (10%), and anacusic, 50 (15%). The remaining 90 children (28%) had high frequency losses (i.e., above 2000 Hz). The mean and median age of diagnosis were 8.78 years and 7.75 years. Temporal bone imaging studies, auditory brainstem responses (ABRs), and vestibular evaluations were performed on selected cases. Etiology was uncertain in 34.8% of cases, while hereditary factors (12.6%), head trauma (10.8%), and perinatal risk factors (10.7%) were the most commonly identified etiologies. Thirty-one percent of the children had scholastic or behavioral problems in school. A concerted effort aimed at early identification and intervention in cases of USNHL is warranted.

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. Results revealed normal vestibular function in 46 infants (13 low-risk, 33 high-risk) and abnormal findings at either 3 or 6 months in 19 infants (4 low-risk, 15 high-risk). Correlations between vestibular results and variables such as auditory brainstem response results, birth history, and postnatal course in the neonatal intensive care unit were analyzed statistically. While some differences were mildly significant, none were highly significant. The lack of significant correlation between abnormal auditory brainstem response and vestibular results is of particular interest.

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. The perpetrator was either a relative or a "trusted other" in 97.2% of sexual abuse cases. Handicapped males were much more likely to be victims of sexual abuse than nonhandicapped males in the general population. Children being educated in residential schools were more likely to be sexually abused than mainstreamed youngsters. These children may be at risk for abuse from a wide variety of potential perpetrators, including teachers, dormitory counselors, van drivers, clergy, classroom aides, older students, peer siblings, scout leaders, abused peers, baby-sitters, and custodians.

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. Reliable behavioral thresholds were obtained on 119 children who had no measurable click-evoked auditory brainstem responses at instrumentation limits of 100 dB HLn. These data show that an absent auditory brainstem response at 100 dB HLn does not necessarily indicate the absence of measurable unaided hearing for test frequencies ranging from 250 Hz to 4000 Hz. Average aided thresholds of better than 60 dB were present in 43% of the children for 500, 1000, and 2000 Hz and in 53% for 500 and 1000 Hz. Therefore, the absence of a click-evoked auditory brainstem response at 100 dB HLn in a young child is not prima facie evidence of the child's cochlear implant candidacy.

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. Within the profoundly impaired group, important differences were also noted in the children's ability to benefit from conventional amplification, depending on residual hearing at or above 1000 Hz. Strategies for assessing auditory function and aided benefit in severely and profoundly hearing-impaired children must involve a pediatric test battery, serial evaluations, and parental cooperation/support. When selecting candidates for cochlear implantation, it is inappropriate to categorize severely hearing-impaired children with those children having profound sensorineural losses. Even profoundly impaired populations are not homogeneous, and rehabilitation potential with conventional amplification must be determined on an individual basis, over time.

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. Twenty-five children with bilateral severe-to-profound postmeningitic sensorineural hearing loss were fitted with appropriate amplification and results of serial-aided tests were analyzed to determine the degree of benefit and changes in aided thresholds over time. Twelve children gave aided responses that clearly indicated the presence of residual hearing and good aided benefit. Ten youngsters have responded consistently in the vibrotactile range when aided, thus receiving limited benefit from conventional amplification. Of particular interest are three children who initially exhibited limited or no auditory responses but who, after a period of 16 to 25 months, showed excellent benefit with binaural hearing aids. These children are all in total communication programs and have received good auditory training. Caution should be exercised when concluding that such a child does not benefit from amplification.

Usher syndrome: clinical findings and gene localization studies.

The issue of genetic heterogeneity is a critical problem in the localization of the gene(s) for Usher syndrome. Based on the data obtained on families studied to date, the differences between type I and type II Usher syndrome appear quite distinct with regard to auditory and vestibular function. Although the majority of families can be confidently diagnosed as typical type I or type II, clinical investigations revealed four families with findings that did not fit into either of the two more common subtypes. These findings emphasize the critical importance of an in-depth clinical analysis concomitant with the linkage investigation to assure accurate subtyping of Usher syndrome. Based on an analysis of only those families with definite type I or type II Usher syndrome, approximately 17% of the genome can be excluded as a potential site of the gene for type I, and 14% can be excluded as the site for the type II gene. This study will continue until the Usher gene(s) is successfully localized.