Francis I. Catlin

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.

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 brainstem 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.

Ossifying Fibroma of the Mid-Face and Paranasal Sinuses: Diagnostic and Therapeutic Considerations

Ossifying fibroma is a benign, usually slow-growing tumor that may behave in an un-predictably 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.

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.

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.

Management of subglottic stenosis in children.

We treated 21 children for subglottic stenosis at the Texas Childrens 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.

The immotile cilia syndrome — One cause of persistent upper respiratory tract infection

Four patients with persistent recurrent upper respiratory tract infections are presented. Electron microscopic studies of respiratory mucosal biopsies from these patients reveal ultrastructural abnormalities of cilia consisting of partial to complete loss of dynein arms, radial spoke disruption, and compound cilia. The concept of immotile cilia as an important cause of recurrent infections is receiving more attention. This study stresses the need for simple, rapid screening tests for cilial activity followed by electron microscopic evaluation in selected cases.

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.

Original Papers · Travaux originaux: 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.

The Otolaryngologist and the Hearing Aid Delivery System: Current Federal Regulations

New proposed rules and regulations concerning trade practices for the hearing aid industry have been developed in response to political pressures from Congress and consumer groups. Revisions to the trade regulations proposed by the Federal Trade Commission (FTC) include (1) more stringent limitations in advertising, (2) written permission to sell a hearing aid at home or in the buyers place of business, and (3) the right of a buyer to cancel a hearing aid sale, rental, or lease within 30 days. Testimony on hearings regarding this rule are being reviewed by the FTC presiding officer. Food and Drug Administration national standards for the labeling and sale of hearing aids became effective Aug 15, 1977. A medical evaluation by a licensed physician is required before a hearing aid may be sold. Fully informed adults may request a waiver of this requirement for religious or personal beliefs, but a medical evaluation is mandatory for persons under 18 years of age. A User Instructional Brochure, which lists several conditions for which medical advice is advised, must be reviewed with the prospective buyer. In the recognition that some physicians may be dispensers of hearing aids, the Judicial Council of the American Medical Association has recommended that a physician may dispense hearing aids if it is in the best interest of the patient, but that a physician should not be encouraged to retail hearing aids in his office if other adequate community facilities exist.