Pharmacologic compliance with antibiotic therapy for acute otitis media: influence on subsequent middle ear effusion.

The frequency of otitis media with effusion (serous otitis media, secretory otitis media) detected after conventional antibiotic treatment of acute otitis media is approximately 50%. The relationship between pharmacologic compliance and the frequency of otitis media with effusion was studied. A Micrococcus lutea bioinhibition test was used to detect the presence of a substances in the urine that inhibited the growth of this antibiotic-sensitive organism. On the fourth, seventh, and tenth days of antibiotic treatment for acute otitis media urine specimens were collected. Otitis media with effusion was detected in 53% of 66 children who had positive results for M lutea bioinhibition compliance test for all three urine specimens. Electro-acoustic impedance measurements (tympanograms) confirmed the presence of effusion in all children in the effusion group tested by this method. The M lutea bioinhibition assay is a practical, in-office method to validate periodically, during treatment, pharmacologic compliance with antibiotics used for treatment of acute otitis media. Pediatrics 68:619-622, 1981.

Cholesteatoma medial to an intact tympanic membrane in 34 young children.

Thirty-five cholesteatomas medial to intact eardrums were treated in 34 children between 1976 and 1982. Six (18%) children had never had a documented episode of otitis media. Seventeen (50%) children, in whom the lesion was diagnosed at an early stage, underwent simple excision of the cholesteatoma without the need for extensive middle ear surgery. Findings from postoperative audiograms were normal for all such children. Cholesteatoma has recurred in eight (23%) children to date. Most recurrences were diagnosed 15 months or less after surgery. Routine careful otoscopic examination is essential in order to discover cholesteatoma at an early stage and to avoid hearing loss and the need for extensive otomastoid surgery. In order to perform an accurate examination of the eardrum, a halogen-illuminated otoscope and pneumo-otoscopy should be used by the pediatrician routinely. Particular attention should be paid to the posterior-superior quadrant of the tympanic membrane where a cholesteatoma is usually located.

Refined localization of the branchiootorenal syndrome gene by linkage and haplotype analysis.

Branchiootorenal (BOR) syndrome is a common autosomal dominant form of hearing impairment previously mapped to 8q. This report refines the localization of the BOR syndrome gene by haplotype analysis to the interval flanked by markers D8S553 and D8S286. By multipoint linkage analysis, the disease locus most likely is flanked by markers D8S530 and D8S279.

Adenotonsillar hypertrophy and upper airway obstruction in evolutionary perspective.

Primary care physicians have become increasingly reluctant to refer children for tonsillectomy and adenoidectomy (T and A) during the past years while antimicrobial therapy for pharyngotonsillitis and otitis media has become more common. Consequently, more children retain tonsils and adenoids throughout the childhood years. Airway compromise from adenotonsillar hypertrophy is reported in 11 cases-8 with insidious onset, 3 with acute onset. Typical symptoms occurring during sleep include snoring, snorting, enuresis and obstructive apnea. Daytime symptoms included hyponasal speech, oral respiration and morning cephalgia. Methods of assessing children with adenotonsillar hypertrophy and airway compromise included polysomnography and acoustic analysis of respiratory sounds. It appears that airway compromise from adenotonsillar hypertrophy is more common now that fewer children are undergoing T and A, is being more commonly recognized because of improved methods of assessment, or both.

An improved stent method for choanal atresia repair.

Short intranasal stents secured sublabially have a number of advantages. They provide secure fixation without an external ligature across the columella. The short length of the stents allows them to sit only in the operative area where the atretic plate had been removed. This lessens the chance of intranasal syechiae formation and eliminates the possibility of erosion in the anterior septal and nasal alar region from pressure exerted by a stent. Since the stents do not protrude from the nose, they cannot be grasped by infants or young children and are not visible externally. School-aged children who have undergone repair of a unilateral choanal atresia can attend school without having to suffer the embarrassment of being teased about a tube protruding from the nose. When compared with other stenting methods, the posterior stent is more secure and less likely to become dislodged.

The inferiorly based superior tympanomeatal flap for removal of congenital cholesteatoma.

We believe that the IB-STMF is preferable to the P-TMF for removal of a congenital cholesteatoma from the anterior superior portion of the mesotympanum. Though developing an IB-STMF may require extra time and special care to avoid tearing the flap, the exposure that is achieved affords the best chance for complete removal of the mass, thus decreasing the chance for persistence or recurrence of the lesion.

Diagnosis and management of spontaneous cerebrospinal fluid-middle ear effusion and otorrhea.

OBJECTIVES/HYPOTHESIS: Spontaneous leak of cerebrospinal fluid (CSF) into the middle ear can occur in adults without a history of temporal bone trauma or fracture, meningitis, or any obvious cause. Therefore, clues may be lacking that would alert the otolaryngologist that fluid medial to an intact eardrum, or fluid emanating from an eardrum perforation, is likely to be CSF fluid. A review of relevant medical literature reveals that herniation of the arachnoid membrane through a tegmen defect may be congenital, or CSF leak may occur when dynamic factors (i.e., brain pulsations or increases in intracranial pressure) produce a rent in the arachnoid membrane. Because tegmen defects may be multiple rather than single, identifying only one defect may not be sufficient for achieving definitive repair. Data on nine cases of spontaneous CSF leak to the ear in adult patients from four medical centers are presented and analyzed to provide collective information about a disorder that can be difficult to diagnose and manage. STUDY DESIGN: Retrospective review of nine cases of spontaneous CSF middle ear effusion/otorrhea. RESULTS: The majority of patients presented with symptoms of aural fullness and middle ear effusion. Many developed suspicious clear otorrhea only after insertion of a tympanostomy tube. Two patients had multiple defects in the tegmen and dura, and five patients had meningoencephaloceles confirmed intraoperatively. Five patients underwent combined middle cranial fossa/transmastoid repair. Materials used in repair included temporalis fascia, free muscle graft, Oxycel cotton, calvarial bone, pericranium, bone wax, and fibrin glue. CONCLUSIONS: CSF middle ear effusion/otorrhea can develop in adults without a prior history of meningitis or head trauma or any apparent proximate cause. Although presenting symptoms can be subtle, early suspicion and confirmatory imaging aid in establishing the diagnosis. Because surgical repair by way of a mastoid approach alone can be inadequate if there are multiple tegmen defects, a middle fossa approach alone, or in combination with a transmastoid approach, should be considered in most cases.

Management of otitis media and the New Agency for Health Care Policy and Research Guideline.

In these times of increasing dominance of managed care and diminishing autonomy for practitioners of medicine, any government pronouncement can be viewed as yet another of many annoyances. For otolaryngologists who yearn for the days when there were not so many intermediaries involved in patient care, the newly issued Clinical Practice Guideline, Otitis Media with Effusion in Young Children (see reference 2 for highlights) may seem more of an ominous threat than anything remotely beneficial. However, given the inexorable changes that are occurring, the Agency for Health Care Policy and Research (AHCPR) otitis media guideline is probably going to be more helpful than harmful.

Balancing pediatric otolaryngology training for fellows and residents at a children's hospital.

OBJECTIVE: To determine the feasibility of providing surgical, endoscopic, and patient contact experience of high educational value at a children's hospital sufficient for adequately training contemporaneously both residents in otolaryngology-head and neck surgery and fellows in pediatric otolaryngology. DESIGN: Retrospective review of operating room case logs and assignment of cases based on arbitrary perception of inherent case complexity and skill and experience that are required to manage the case. SETTING: Tertiary care children's hospital located in a major metropolitan area. MAIN OUTCOME MEASURES: (1) Volume of surgical and endoscopic cases assigned retrospectively to junior resident, senior resident, or fellow. (2) Score on newly developed self-assessment skill list in pediatric otolaryngology. RESULTS: During 1 year, there were 3224 surgical and endoscopic procedures performed in the operating room. Of the total number of procedures, only 44 (1.4%) were designated as being exclusively assigned for hands-on experience to a fellow, but 380 (11.8%) were appropriate for both a senior resident and a fellow and therefore were apportioned in an alternating fashion. A self-assessment instrument has been developed to assess competency and comfort in the management of otolaryngic disorders, both surgical and nonsurgical, in children. CONCLUSIONS: The volume and assortment of surgical and endoscopy cases at a tertiary care children's hospital can provide the basis for a rich, practical hands-on experience for residents and fellows. Since few surgical or endoscopic cases require pediatric fellowship training for mastery, becoming a pediatric otolaryngologist depends on acquiring skills and competence that exceed the technical skills acquired in the operating room.

Relative incidence and alternative approaches for surgical drainage of different types of deep neck abscesses in children.

OBJECTIVES: To determine the relative frequency of retropharyngeal abscesses (RPAs) vs lateral pharyngeal abscesses (LPAs) and to analyze alternative approaches for surgical drainage. DESIGN: Retrospective chart review. SETTING: Tertiary care children's hospital. PATIENTS: Seventy pediatric patients who were evaluated, admitted, and treated for presumed deep neck abscesses (RPAs and LPAs) between January 1, 1986, and December 31, 1996. INTERVENTION: Intravenous antibiotic therapy and surgical drainage. MAIN OUTCOME MEASURE: Clinical resolution of the abscess. RESULTS: Fifty-eight patients were evaluated with computed tomographic scan. Thirteen of these patients did not have surgical intervention. Of 12 patients diagnosed as having an isolated RPA, all had intraoral surgical drainage and 9 had evidence of pus at surgery. Twenty-one patients had an isolated LPA. Sixteen of these underwent intraoral drainage and 5 underwent external drainage. Purulence was found at surgery in 14 and 2 patients, respectively. The remaining 12 patients had a combination of RPA and LPA. Eight patients underwent intraoral drainage, and 4 patients required both intraoral and external approaches. Purulence was found at surgery in 5 and 4 patients, respectively. Of the 12 patients who were not evaluated with computed tomographic scan, two thirds were treated prior to 1987. Six of these 12 patients underwent surgical drainage via an intraoral approach, and 4 of the 6 patients had pus. The remaining 6 improved without surgery. CONCLUSIONS: Most deep neck abscesses in children are located in the retropharyngeal or in the lateral pharyngeal space medial to the great vessels. Therefore, most can be managed successfully with intraoral rather than external drainage. External approaches are better reserved for those abscesses that are lateral to the great vessels or that involve multiple spaces. In this patient population, LPAs were more commonly seen than RPAs.

Otologic manifestations of Wolf-Hirschhorn syndrome.

OBJECTIVE: To determine if haploinsufficiency for chromosome 4p16.3 in Wolf-Hirschhorn syndrome (WHS) is associated with cochlear hearing loss. DESIGN: Case series. SETTING: Tertiary care center. PATIENTS: Six patients with WHS were identified through a database and charts were retrospectively reviewed. MAIN OUTCOME MEASURES: Presence of sensorineural hearing loss as assessed by brainstem auditory evoked response. RESULTS: One of the 6 patients had sensorineural hearing loss. Three of the 6 patients had chronic otitis media with effusion and underwent bilateral tympanostomy tube placement; 2 of these 3 had cleft lip and palate, and 1 had a bifid uvula. One of the 6 patients had spontaneous nystagmus. Five of the 6 patients had preauricular and/or auricular abnormalities. CONCLUSIONS: More than 25 genes for nonsyndromic hereditary hearing impairment have been mapped. One of these genes, DFNA6, was identified through linkage analysis of a family with dominant, progressive, low-frequency sensorineural hearing loss. DFNA6 maps to chromosome 4p16.3, a region that is partially deleted in patients with WHS. In our series, we identified the second patient with WHS in the literature with bilateral sensorineural hearing loss. The incidence and type of otologic findings are consistent with those reported in the literature. Analysis of patients with chromosomal rearrangements represents one strategy toward identifying candidate genes for genetic hearing impairment.

Enlarged vestibular aqueduct and sensorineural hearing loss in childhood.

OBJECTIVE: To determine if all children with enlarged vestibular aqueducts (EVAs) have development of uniform progressive sensorineural hearing loss (SNHL). To determine whether the size of the EVA correlates with severity, frequencies involved, and stability of SNHL. To determine if the audiologic pattern of SNHL correlates with likelihood of progression of SNHL. DESIGN: Retrospective study. SETTING: Children's National Medical Center, Washington, DC, a tertiary care center with a large otologic practice. PATIENTS: Fifteen children (26 ears) with EVA on computed tomographic scan. METHODS: History, physical examination, computed tomographic scans, and serial audiograms were reviewed. Factors analyzed included age at diagnosis, audiometric configuration (high tone, midtone, low tone, flat), degree of hearing loss at presentation, length of follow-up, and presence of associated inner ear anomalies. RESULTS: Nine ears had progressive SNHL, 16 ears had stable SNHL, and 1 ear had profound SNHL. The predominant audiologic configuration was flat. The audiogram configuration does not correlate with progression of SNHL. The size of the vestibular aqueduct does not correlate with the level, type, or progression of SNHL. CONCLUSION: Our study failed to uncover factors that might be predictive of progression of hearing loss. We conclude that until a better understanding of the natural history and pathophysiologic condition of EVAs is achieved, there is no surgical or other intervention that can be demonstrated as being efficacious.

Delayed diagnosis and fate of congenital cholesteatoma (keratoma).

OBJECTIVE: To analyze clinical presentation, modes of detection, growth pattern, operative findings, and results of surgery in children 3 years old or older who had extensive congenital cholesteatoma (keratoma). DESIGN: Survey, case series. SETTING: Two academically affiliated medical centers: a children's hospital and an eye, ear, and throat hospital, both located in major metropolitan cities. PATIENTS: Twenty-five children selected according to specified criteria. INTERVENTION: Tympanomastoid surgery, ie, canal wall up and canal wall down, some with ossicular reconstructive surgery. MAIN OUTCOME MEASURE: Audiologic assessment (speech reception threshold) and recurrence (recidivism) of cholesteatoma. RESULTS: Incidence of recidivism, 52%. Hearing maintained within the range of normal to mild hearing impairment postoperatively in 91% of the patients for whom complete data are available. CONCLUSIONS: Congenital cholesteatoma may grow for years without causing signs or symptoms and, having grown without early detection, can extend to involve the epitympanum and mastoid antrum, cause ossicular erosion, and even extend to the middle cranial fossa. To adequately remove a congenital cholesteatoma that has gone undetected for many years, exposure of the anterior epitympanum is often necessary and removal of both the body of the incus and the head of the malleus often is required. Since congenital cholesteatoma usually develops in a child with a well-pneumatized mastoid that would create a large mastoid bowl if exteriorized, the otologic surgeon is likely to hesitate in using the canal wall down mastoidectomy technique. Alternatives to the canal wall down mastoidectomy technique, which can achieve reasonably good hearing results and avoid creation of a large mastoid bowl, include planned two-stage canal wall up surgery or canal preservation with primary reconstruction and close follow-up with otomicroscopy and serial computed tomographic scans.

Management of oropharyngeal trauma in children.

OBJECTIVE: To determine the indications for admission, requisite imaging studies, and urgent medical or surgical intervention. DESIGN: We retrospectively reviewed the charts of 26 children (age range, 5 months to 14 years) who were seen by the otolaryngology service in the emergency department at the Children's National Medical Center, Washington, DC, from 1985 to 1993 and who were diagnosed as having oropharyngeal trauma. We specifically looked for common findings in the history and physical examination on initial presentation to predict the necessary steps in evaluation and management. SETTING: Tertiary care pediatric referral center. RESULTS: Indications for admission were (1) concern about neurologic injury, (2) concern about vascular injury, (3) radiographic evidence of retropharyngeal free air or abscess, (4) pneumomediastinum, and (5) unreliable adult supervision at home. Six patients required surgery; 3 underwent retropharyngeal aspiration or incision and drainage procedures; 2 required neck explorations; and 1, who had an impaled foreign body in the parapharyngeal space, underwent surgical extraction. There were no vascular, neurologic, or other permanent injuries. CONCLUSIONS: Oropharyngeal trauma may result in palatal and posterior pharyngeal wall injury requiring closure of lacerations and management of retropharyngeal free air. Rarely does an injury lead to retropharyngeal abscess or significant pneumomediastinum. Lateral oropharyngeal injuries require increased concern about potential neurovascular impairment. However, neither the mechanism of injury nor the degree of injury correlates with the potential for neurovascular sequelae. Since neurovascular involvement may not become clinically apparent until days or weeks after the incident, admission for observation alone should be based on the distance from the patient's home to the hospital and on the level of reliable adult supervision. Indications for medical and surgical treatment of internal carotid artery thrombosis remain controversial.

Laryngomalacia. The search for the second lesion.

OBJECTIVES: To determine the necessity of rigid endoscopy in the diagnosis and management of laryngomalacia and its associated synchronous airway lesions (SALs), to analyze the incidence of SALs associated with laryngomalacia and their significance, and to determine the need for epiglottoplasty in management of laryngomalacia. DESIGN: Retrospective medical chart review. SETTING: Tertiary care children's hospital. PATIENTS: Two hundred thirty-three patients with a primary diagnosis of laryngomalacia on flexible fiberoptic laryngoscopy treated at the Children's National Medical Center, Washington, DC, from January 1, 1984, to June 30, 1994. INTERVENTION: Evaluation and treatment of laryngomalacia and associated SAL by flexible fiberoptic laryngoscopy, radiographic studies, rigid endoscopy, and other surgical procedures. MAIN OUTCOME MEASURES: Resolution of airway symptoms from laryngomalacia and associated SAL. RESULTS: Ninety patients (38.6%) underwent rigid endoscopy, and 12 patients (5.2%) required epiglottoplasty. Synchronous airway lesions were discovered in 44 patients (18.9%). Eleven patients (4.7%) had SALs that wre considered clinically significant; nine (3.9%) of these required surgical intervention. CONCLUSIONS: Rigid endoscopy in evaluation of an infant with laryngomalacia is rarely necessary. Clinically significant SALs requiring surgical intervention are uncommon. Surgical intervention for laryngomalacia also is rarely necessary.

Vocal cord paralysis in children studied by monopolar electromyography.

Twelve children with symptoms of vocal cord paralysis were studied by monopolar needle electromyography. Recordings were used to verify complete or partial paralysis as well as to evaluate the etiology of the problem, paralytic or mechanical. These results then were useful in patient management. The monopolar electromyographic technique is of value in evaluating and treating vocal cord problems.

A stepwise approach to the diagnosis and treatment of hereditary hearing loss.

What To Do Do suspect a genetic cause in all cases of hearing loss. Do develop a working knowledge of common types of HHI that you may draw on to aid in diagnosis. Do think of HHI when the audiogram reveals a hearing loss with a "cookie bite" configuration. Do refer the infant to a geneticist in cases where you suspect a syndromic HHI, a nonsyndromic HHI, and in cases of "cryptogenic" hearing loss where an underlying HHI may be present. Often, the associated symptoms are subtle and best detected by a professional who deals with these issues on a daily basis. Do get the infant or family plugged into an audiologist or otolaryngologist and speech pathologist who will preferably work as a team to maximize aural rehabilitation and ensure serial follow-up. It is never too early to fit a child with hearing aids. Do refer to the HHIRR center at Boys Town. Do refer to the correct "deaf" organization or "blind-deaf" organization. Do think about working up other siblings or family members. Do keep in mind that some members of the "deaf society" may regard deafness as an alternative lifestyle and may not be amenable to their child's referral for additional workup and aural rehabilitation. What Not To Do Do not assume the child is deaf and nothing can be done. Do not wait until the child is older to refer to an otolaryngologist, speech therapist, and audiologist. Do not order a sonogram. Do not order a temporal bone CT scan on newborns. Do not forget about other siblings who may have a similar pathology. Do not forget that some forms of HHI can present beyond infancy. The pediatrician is the front line and can play a major role in the diagnosis, workup, and treatment of HHI. Armed with the proper degree of suspicion, careful elicitation of family history, meticulous physical examination, evaluation of the audiogram, and adequate fund of knowledge of common types of genetic deafness, the pediatrician can make a timely diagnosis and appropriate referrals. This avoids delay in detection of significant hearing impairment and the associated lack of essential skills in speech, language, and social interaction. No child is too young to have some type of hearing assessment. Early detection and intervention are best done with a multidisciplinary team approach with a neonatologist or pediatrician, audiologist, speech therapist, and otolaryngologist. In the future, blood tests using genetic probes may be available to screen for many types of HHI.

Management of otolaryngic patients susceptible to malignant hyperthermia without dantrolene.

Malignant hyperthermia (MH) is an adverse reaction most frequently associated with the administration of halogenated inhalational anesthetic agents and the depolarizing muscle relaxant succinylcholine. Characteristic findings are a hypermetabolic state accompanied by extreme hyperpyrexia, acidosis, rhabdomyolysis, and generalized muscle rigidity, often involving the masseter muscles. Dantrolene sodium, which was approved in 1979 by the FDA for use in the prevention of MH in high-risk patients, has neurologic and gastrointestinal side effects. At the Children's National Medical Center (CNMC), 24 children identified as being at risk for the development of a MH reaction were anesthetized for otolaryngic procedures by using "non-triggering" anesthetics and without use of dantrolene sodium. These patients represent 56% of all patients at risk for MH or masseter muscle rigidity (MMR) reactions during an 8-year period at the CNMC. There were no complications. Concomitant muscle biopsies were performed, and caffeine/halothane contracture studies were completed in 18 of these patients, demonstrating 11 susceptible or equivocal responses. The data suggest that children undergoing common otolaryngic procedures who are at risk for development of MH may be safely anesthetized without the use of prophylactic dantrolene sodium.

External auditory canal stenosis and partial atresia without associated anomalies.

Unilateral or bilateral ear canal stenosis or atresia occurring without microtia or associated anomalies may go undetected during early childhood. Summaries of ten cases illustrate difficulties in making early diagnoses. An unusual case of inherited isolated bilateral external canal atresia occurring in a mother and daughter is included. Potential pitfalls in diagnosis and management are discussed.

Differentiating upper from lower airway compromise in neonates.

Technologic advancements and improvements in supportive care have resulted in increased survival of very low birth weight and premature infants. With salvage of these high risk newborns, many difficult management problems arise. Respiratory distress of the newborn is the most common airway problem that affects these patients and subglottic edema or stenosis may frequently complicate management of the airway. Decision-making in airway maintenance and respiratory care can be problematic due to multiple factors that must be considered. The otolaryngologist is often consulted when upper airway obstruction is suspected. Relevant respiratory physiology is reviewed in order to help direct therapeutic decision-making. Parameters of assessment are enumerated and methods for choosing among therapeutic alternatives are presented. A paradigm to aid in differential diagnosis is described.