Progressive hearing loss in infants with asymptomatic congenital cytomegalovirus infection.

Congenital cytomegalovirus (CMV) infection is a major public health problem because 30,000 to 40,000 neonates with the infection are born each year in the United States. Although 90% of the congenitally infected infants are asymptomatic at birth, evidence is accumulating that these infants are at risk for audiologic, neurologic, and developmental sequelae. The current study describes the audiologic outcome of 59 infants with asymptomatic congenital CMV infection compared with 26 control infants. Eight of 59 infected infants had congenital sensorineural hearing loss (SNHL) but none of the control subjects did. Longitudinal audiologic assessments revealed that 5 of the 8 infants had further deterioration of their SNHL; a ninth infant with initially normal hearing experienced a unilateral SNHL during the first year of life, with further deterioration subsequently. The frequency of SNHL was similar for infected infants born to mothers with recurrent CMV infections during pregnancy (2 of 9) and for those born to mothers who experienced primary CMV infections (5 of 26). There was a significant difference between the occurrence of hearing loss in infected infants with normal computed tomographic scans (2 of 40) compared with those with either periventricular radiolucencies (4 of 13) or calcifications (1 of 3). Children with SNHL often have no identified cause of the loss; thus, it is likely that many of these children had asymptomatic congenital CMV infection. Given the progressive nature of SNHL associated with asymptomatic congenital CMV infection, longitudinal audiologic assessments are mandatory.

cis-platinum ototoxicity in children.

Despite the recognized ototoxicity of cis-platinum, a clinical outline for the audiologic evaluation of patients receiving this drug has not been clearly defined. In a practical approach to this problem, the audiograms of 48 pediatric patients referred for monitoring during planned cis-platinum therapy were reviewed. Eleven patients tested with auditory brain-stem response (ABR) audiometry demonstrated several limitations of this modality. Fourteen children underwent initial ABR testing followed by at least two pure-tone audiograms. The remaining 23 patients had their hearing evaluated by pure-tone audiometry only. Various factors such as patient age, cis-platinum dosage, and cranial radiation exposure were analyzed for apparent effect. Younger patients tended to be more susceptible to audiologic changes with the administration of cis-platinum. The proportion of patients who demonstrated a hearing loss increased with successive dosing as did the severity of the hearing loss. Prior exposure to cranial radiation was strongly linked to the development of hearing loss following cis-platinum therapy. Guidelines are presented regarding the use of clinical audiometry in the screening of these pediatric oncology patients.

The effect of steroid therapy on recovery from tonsillectomy in children.

A prospective, randomized, double-blind study to determine the postoperative effects of steroids in tonsillectomy was performed on 25 children from 4 to 12 years of age. A single intravenous dose of dexamethasone or a placebo was administered at onset of surgery. Other preoperative and postoperative medications, including antibiotics, anesthesia, and surgical techniques were standardized as noted in this article. The ability to return to a full or semifull diet occurred on the third and fourth postoperative days, significantly sooner in the steroid-treated patients than in the control patients. By the fifth and sixth days, the control group were eating as well as those children who received steroids. No significant differences were observed in postoperative pain, nausea, emesis, fever, or in the need for postoperative pain medications. This preliminary article concludes that a single preoperative dose of steroid results in an earlier return to a normal (full) diet in children who had undergone tonsillectomy.

Ossifying fibroma of the mid-face and paranasal sinuses: diagnostic and therapeutic considerations.

Ossifying fibroma is benign, usually slow-growing tumor that may behave in an unpredictably aggressive fashion. Most of these unusual tumors affect the mandible. Their behavior in the mid-face and paranasal sinuses is not well documented. In our review of the subject, and of the cases presented, it appears that these lesions behave more aggressively than their mandibular counterparts. A more aggressive approach may be more beneficial than expectant observation or curettage in the initial management of this neoplasm.

Laryngotracheal stenosis: a 5-year review.

Twenty-seven children with varying degrees of laryngotracheal stenosis were treated at Texas Children's Hospital (TCH) from January 1984 to January 1989. Nineteen children had a residual airway demonstrable preoperatively, and following laryngotracheoplasty, 18 (95%) were successfully decannulated; all but 1 had a normal voice. Eight children had complete stenosis. Of these children, 6 could be decannulated, but 3 required a second laryngotracheoplasty. Four children now have voice abnormalities.

Asymptomatic congenital cytomegalovirus infection. Audiologic, neuroradiologic, and neurodevelopmental abnormalities during the first year.

Twenty-eight infants with asymptomatic congenital cytomegalovirus infection and 13 control infants were followed up prospectively. Congenital sensorineural hearing loss was documented by auditory brain-stem responses in four infected infants (two had mild bilateral loss, one had mild unilateral loss, and one had extreme unilateral loss) but in no controls. Four infected infants had diffuse periventricular radiolucencies on computed tomographic scan; none had calcifications or ventriculomegaly. No differences between groups were noted on neurologic examination results or on the Bayley Mental Developmental Index; however, one infected infant had a severely delayed Bayley Psychomotor Developmental Index score. In addition, the mean Mental Developmental Index score of the four infected infants with diffuse periventricular radiolucencies was significantly below that of the remaining infected infants (93 +/- 8 vs 109 +/- 13). These data suggest that asymptomatic congenital cytomegalovirus infection may be associated with a broad range of audiologic, subtle neuroradiologic, and neurodevelopmental differences in early infancy.

Choanal atresia and associated anomalies: the CHARGE association.

Over the past few years there has been increasing awareness of the association of choanal atresia with other congenital defects. Thirty-one cases of choanal atresia were reviewed and other congenital anomalies were documented in 19 patients. These other anomalies were identified in 75% of the 20 patients with bilateral choanal atresia but in only 36% of the 11 patients with unilateral choanal atresia. Associated defects largely occurred in a predictable pattern described by the CHARGE mnemonic: C-coloboma, H-heart disease, A-atresia choanae, R-retarded growth, G-genital hypoplasia, E-ear defects. The implications of the CHARGE association in the management of patients with choanal atresia are discussed.

Cystic fibrosis--an otolaryngologic perspective.

Cystic fibrosis (CF) is the most common lethal genetic disorder in white patients. The protean manifestations of the disease result from exocrine gland dysfunction and include chronically debilitating pulmonary and pancreatic compromise and clinically inconsequential (although diagnostically extremely important) sweat electrolyte abnormalities. The subject of this article is the otolaryngologic manifestations of the disease, based on a retrospective analysis of 450 cases. Nasal polyposis and sinusitis occurred in 10% and 11% of patients, respectively, and polypectomy was, after laparotomy, the most common surgical procedure these children underwent. The extent of intranasal surgery for polyposis was found to be inversely proportional to the recurrence rate. A simple polypectomy was relatively ineffective treatment; when performed in conjunction with a Caldwell-Luc and either an intranasal or extranasal ethmoidectomy, the recurrence rate was less than 13%. Otologic problems, found in 8% of patients, included chronic otitis media (2.5%) and acute otitis media (5.5%). Only five patients required pressure-equalizing tubes. Recent genetic advances of immense importance are also described. Although the basic gene defect has yet to be elucidated, by use of a technique known as restriction-fragment-linked polymorphism, the gene associated with CF has been found in the middle of the long arm of chromosome 7. By following gene markers closely associated with this gene, it is possible to do carrier tests within affected families and, if certain criteria are met, perform prenatal diagnosis. Eventual isolation and characterization of the gene will follow, hopefully making prevention possible and treatment more effective.

Acquired subglottic stenosis in children.

Subglottic stenosis is a disorder characterized by narrowing of the airway below the glottis or apposing edges of the true vocal cords. In a broad definition, the term may be used to describe airway compromise in the regions of the larynx or the trachea or both. In children, the stenosis is usually due to scar formation secondary to prolonged airway intubation, rather than to external trauma. The location and extent of the stenosis are highly variable; consequently, corrective measures need to be selected to suit the individual problem. In the present series of children, conservative treatment was adequate for lesser degrees of stenosis; those with more severe scarring required external laryngeal surgery. The preoperative evaluation and treatment plans are reviewed.

Temporomandibular joint dysfunction in infancy.

Temporomandibular joint (TMJ) dysfunction describes a pain-dysfunction phenomenon that usually afflicts persons in their 4th or 5th decade. The syndrome can be produced by a variety of etiologic factors including occlusal disharmony, articular disorders, and muscle imbalance. It may cause severe otalgia and refer pain to the temple, occiput, nape of neck, and shoulders. Often, associated joint clicking or popping, aural fullness, vertigo, tinnitus, subjective hypoacusis, and nausea occur. As it has not been previously reported in infants, we would like to describe our experience with this disorder in an 11-month-old boy who was referred to our clinic with a presumed diagnosis of otitis media. The embryology of the temporomandibular joint is reviewed and appropriate treatment with anti-inflammatory analgesics, warm compresses, orthodontics, and external brace appliances is discussed. Because of referral patterns in the infant age group, the pediatric otolaryngologist should be similar with this entity and its presentation in children.

Noise-induced hearing loss.

Hearing loss affects 30 million people in the United States; of these, 21 million are over the age of 65 years. This disorder may have several causes: heredity, noise, aging, and disease. Hearing loss from noise has been recognized for centuries but was generally ignored until some time after the Industrial Revolution. Hearing loss from occupational exposure to hazardous noise was identified as a compensable disability by the United States courts in 1948 to 1959. Development of noisy jet engines and supersonic aircraft created additional claims for personal and property damage in the 1950s and 1960s. These conditions led to legislation for noise control in the form of the Occupational Safety and Health Act of 1970 and the Noise Control Act of 1972. Protection of the noise-exposed employee was also an objective of the Hearing Conservation Act of 1971. Subsequent studies have confirmed the benefits of periodic hearing tests for workers exposed to hazardous noise and of otologic evaluation as part of the hearing conservation process. Research studies in laboratory animals, using scanning electron microscopical techniques, have demonstrated that damage to the inner ear and organ of hearing can occur even though subjective (conditioned) response to sound stimuli remains unaffected. Some investigators have employed an epidemiologic approach to identify risk factors and to develop profiles to susceptibility to noise-induced hearing loss. The need for joint involvement of workers and employers in the reduction and control of occupational noise hazards is evident.

Management of subglottic stenosis in children.

We treated 21 children for subglottic stenosis at the Texas Children's Hospital from 1975 to 1983. Ages ranged from newborn to 14 years. Fifteen (71%) were younger than 13 months of age. Prolonged intubation was thought to be a primary contributing factor in 16 of 21 (76%). Nineteen (90%) required tracheotomy. Of these, 11 (58%) were decannulated, four (21%) have not been decannulated, and four (21%) were lost to follow-up. Two of the 11 children who were successfully decannulated required laryngotracheoplasty or thyrotomy. There were three instances of complications and no deaths.

Brain abscesses in the young.

Brain abscesses in the young are rare. Only 14 such abscesses have been seen at Texas Children's Hospital since 1968. Most abscesses developed in association with congenital heart disease (5), although sinusitis and mastoiditis were precipitating causes in two patients and one patient, respectively. The latter three patients' cases are reviewed in detail. Clinical and bacteriologic findings in all patients are discussed. There were signs of increased intracranial pressure in nine patients (64%). All abscesses were drained; in several, repeated drainage was necessary. Anaerobic organisms were recovered in six patients (43%), aerobic organisms were recovered in five (36%), and both were recovered in two (14%). In one patient no growth was reported. Antimicrobial therapy was administered to all patients but one, whose abscess was completely excised. Morbidity and mortality remained significant: three patients (21%) died and one has a residual hemiparesis.

Prevention of hearing impairment from infection and ototoxic drugs.

Infectious diseases are a primary cause of hearing impairment and produce about 25% of profound losses. Of these, one fifth are congenital. The major infections include rubella, cytomegalovirus, measles, pertussis, meningitis, and acute otitis media. Hearing loss from ototoxicity is also observed with a number of drugs, notably the aminoglycosides, loop diuretics, and cisplatin. Preventive measures are defined according to primary, secondary, and tertiary principles. Three principles of prevention are considered: direct action, defined objectives, and the variability of effective prevention according to cause.

Onset of hearing loss in children with bacterial meningitis.

Auditory brainstem responses were evaluated in 37 children with bacterial meningitis within 48 hours of admission. Four children (two with Haemophilus influenzae type b, and two with Streptococcus pneumoniae) had definite abnormalities of hearing detected at admission. Two of these children had severe-to-profound hearing losses which have persisted. Hearing losses were greatly reduced in the other two children (one child also was ataxic) when repeat testing was performed. Auditory brainstem responses in two additional children suggested brainstem dysfunction at admission (one child died and the other has had severe sequelae). Hearing losses occur early in the course of bacterial meningitis in some children. Further studies are required to confirm the use of auditory brainstem responses in detecting hearing losses early in high-risk children with bacterial meningitis.

Prospective comparative trial of moxalactam versus ampicillin or chloramphenicol for treatment of Haemophilus influenzae type b meningitis in children.

In a prospective, randomized study, moxalactam in 44 children was compared with ampicillin or chloramphenicol in 47 children for the treatment of Haemophilus influenzae type b meningitis. Both groups were comparable in terms of clinical and laboratory findings at admission. The hospital course, neurologic sequelae including deafness, and number of deaths were the same for both groups. The incidence of adverse reactions also was the same except that diarrhea and thrombocytosis occurred significantly (P less than or equal to 0.04) more frequently in children given moxalactam. Moxalactam was equivalent to ampicillin or chloramphenicol in the treatment of H. influenzae type b meningitis in children.

Studies of normal hearing.

Auditory function changes continually from birth to old age. A variety of methods to assess hearing have evolved since the invention of the audiometer. Types of measurement include: electrical response in the central nervous system, cochlear acuity and speech responses. While some of these tests correlate fairly well with each other, their ability to represent overall hearing function is questionable. Other attempts to improve the assessment of hearing have been made in the area of self-appraisal, but these, too, have significant limitations. Most self-report and peer appraisal questionnaires have been established by studies of hearing-impaired populations. Norms for these techniques in normal-hearing populations need to be established. There is still room for valid tests of everyday communication. What we have in measurement procedures does not achieve this goal. Research studies of today will hopefully produce better definition of normal auditory function.

Congenital anomalies of the larynx.

Congenital anomalies of the larynx are infrequent, though potentially life-threatening, defects with which both pediatricians and otolaryngologists should be familiar. We reviewed the basic stages in normal laryngeal embryology and outlined a scheme for the examination of a neonate with a possible laryngeal abnormality. In particular, symptoms, laryngoscopic appearance, and treatment of the more commonly encountered anomalies should be considered.

Symptomatic congenital cytomegalovirus. Disorders of language, learning, and hearing.

Seventeen patients with symptomatic congenital cytomegalovirus (CMV) were studied longitudinally, with emphasis given to disorders of language, learning, and hearing. At a mean age of 5.5 years (range, 1 go 10 years), nine children (53%) performed in the retarded range. Eleven (65%) experienced sensorineural hearing loss, in three of whom it was progressive. Developmental verbal dyspraxia was documented in two children and suspected in a third. Disabilities in several areas of the learning process exhibited by four children with normal intelligence and hearing loss. Although the effects of congenital CMV were diverse, all of the children had developmental disorders that necessitated special education. Such patients require longitudinal follow-up that includes more than tests of intelligence and hearing. All areas of development must be evaluated to appreciate the full effect of CMV encephalitis in utero.