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Autonomic Dysreflexia↗
Biomedical subjects
D J Franklin
Publications and source records attributed to D J Franklin.
Establishing the etiology of childhood hearing loss.
The cause of hearing loss in children is often difficult to identify. We evaluated a cohort of 114 children (47 boys, 67 girls) referred with newly diagnosed hearing loss (non-otitis media) to identify factors predictive of etiology and type of hearing loss. Clinical (history and physical examination), laboratory, and radiographic data were collected. One hundred children (87.7%) had sensorineural hearing loss, and 14 (12.3%) had conductive or mixed hearing loss. The cause of hearing loss was identified in 54 children (48%). Patients with isolated aural atresia (n = 7) or with a known diagnosis of congenital cytomegalovirus infection (n = 21) were excluded from further data analysis. We conducted statistical analysis to identify factors predictive of the cause and type of hearing loss. Clinical factors that aided in identifying a cause included abnormal physical examination findings (p = 0.001) and craniofacial anomalies (p = 0.006). Computed tomography of the temporal bones was the only diagnostic test predictive of cause (p < 0.001). Factors predictive of the type of hearing loss detected (sensorineural vs. conductive or mixed) were abnormal physical examination findings (p = 0.01) and craniofacial anomalies (p = 0.004). An exhaustive laboratory or radiographic workup did not prove beneficial in identifying the etiology of hearing loss in our series.
Audiometry↗
Life-threatening presentations of fibrovascular esophageal and hypopharyngeal polyps.
Giant fibrovascular polyps of the esophagus and hypopharynx are benign tumors of the upper digestive tract. Although a rare cause of asphyxiation, laryngeal impaction by a regurgitated polyp of the esophagus may be the initial symptom that brings the patient to request medical attention. Two new cases of giant fibrovascular polyps with dramatic and potentially life-threatening presentations illustrate the unpredictable behavior of these unusual tumors. Both patients presented to the emergency center with a history of coughing and eructation followed by temporary airway obstruction that was relieved by clenching a regurgitated fleshy mass between the teeth. Diagnostic and therapeutic intervention requires aggressive airway management, radiographic and endoscopic evaluation, and definitive surgical treatment. Tracheotomy was required in one patient, and successful endotracheal intubation provided satisfactory airway control in the second. Esophagoscopy revealed the origin of both tumors to be near the cricopharyngeal muscle. Complete surgical excision was curative in both cases.
Aged↗
Head and neck manifestations of erythema multiforme in children.
Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis are related disorders of skin and mucous membranes, which are typically associated with antecedent medication use or infection. We review 108 cases of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis from Texas Children's Hospital, Houston, Texas, from 1981 to 1991, and illustrate the characteristic skin and mucosal lesions. In addition, we describe in detail two unusual cases requiring intensive airway management. Head and neck manifestations were present in 4 of 79 patients (5%) with erythema multiforme and 26 of 28 patients (93%) with Stevens-Johnson syndrome. In Stevens-Johnson syndrome, mucosal involvement of the lip (93%), conjunctiva (82%), oral cavity (79%), and nose (36%) were most common. Antecedent medication use was identified in 59% of erythema multiforme patients and 68% of Stevens-Johnson syndrome patients. We note a striking increase in the number of cases in our series caused by cephalosporins. Fifty percent of Stevens-Johnson syndrome patients required supplemental hydration or alimentation because of the severity of the oral cavity involvement. The head and neck mucosal manifestations largely respond to local care, and the routine use of prophylactic antibiotics or systemic steroids is not recommended.
Adolescent↗
Chronic otitis media after tympanostomy tube placement caused by Mycobacterium abscessus: a new clinical entity?
Infections with nontuberculous mycobacteria (NTM) are being identified with increasing frequency, but the otologic manifestations of NTM infection are not well defined. Mycobacterium abscessus is a ubiquitous rapidly growing mycobacterium (RGM) known to cause disease by inoculation after trauma. Though reported following open heart and breast augmentation surgery, it is not recognized as a cause of sporadic post-tympanostomy tube otorrhea. This report presents detailed clinical information on six sporadic cases and partial information on 15 additional cases of ear infection caused by RGM over the past 7 years. Of these, 20 of 21 cases (95%) were attributable to M. abscessus, 14 of 21 (67%) subjects lived in a southern coastal state and 16 of 16 with available histories had previously undergone placement of tympanostomy tubes. Each isolate exhibited resistance to many antibiotics, with 50 percent exhibiting high level mutational resistance to aminoglycosides related to prior topical aminoglycoside use. Therapy was difficult, requiring debridement and prolonged antibiotic therapy. M. abscessus is a problematic infection requiring specific diagnosis and treatment and should be sought as a cause of refractory post-tympanostomy tube otorrhea.
Abscess↗
Test/retest reliability of distortion-product and transiently evoked otoacoustic emissions.
Otoacoustic emissions can be used to study cochlear function in an objective and noninvasive manner. These features of emitted responses have stimulated a great deal of investigation into the utility of evoked emissions as clinical tests of hearing. One practical and essential aspect of any clinical measure is the consistency of its result upon repeated testing of the same individual (i.e., its test/retest reliability). The goal of the present work was to conduct a systematic study of the test/retest reliability of the two evoked emission types, the transiently evoked and the distortion-product otoacoustic emissions, that have the greatest promise of becoming clinically useful. Toward this end, the short- and long-term reliabilities of these two response measures were examined in 12 normally hearing adults. The results of these experiments showed that the consistency of both measures of evoked otoacoustic emissions was generally excellent.
Adult↗
Altered susceptibility of 2f1-f2 acoustic-distortion products to the effects of repeated noise exposure in rabbits.
Hearing sensitivity and the generation of acoustic-distortion products at 2f1-f2 were examined systematically in behaviorally trained rabbits, before, during, and following regular exposure to a 95-dB SPL octave band of noise, centered at 1 kHz. During the exposure period, the octave-band noise was interrupted once every 24 h in order to monitor the progressive loss in auditory function using tests of behavioral threshold and distortion-product otoacoustic emissions (DPOAEs). When low-frequency DPOAEs from 1-4 kHz diminished to noise-floor levels, i.e., when their amplitudes were reduced by about 20-30 dB, the exposure was terminated. Subsequent recovery of behavioral thresholds and DPOAE amplitudes and detection 'thresholds' was evaluated at regular intervals over a 3-week post-exposure period. Following the recovery period, the rabbits again received the identical exposure/recovery treatment until a permanent 10 dB or greater loss in DPOAE amplitudes was achieved for any point of measurement between 2-10 kHz. The primary result was that the number of days of overstimulation required for rabbits to reach the criterion loss in DPOAE amplitudes increased for each successive exposure session. In addition, DPOAEs accurately tracked the frequency pattern described by the behavioral threshold shifts during both the development and recovery stages of exposure.
Animals↗
Distortion product emissions in humans. III. Influence of sensorineural hearing loss.
The realization that otoacoustic emissions are sensitive to cochlear disorders has resulted in the speculation that they may have considerable clinical potential as objective measures of hearing. To assess the clinical utility of one type of emission, the distortion product emission (DPE), a study was undertaken in individuals with hearing impairments representing a number of common otologic disorders. The results of this investigation provided evidence that tests of DPEs promise to satisfy a number of requirements important to clinical testing, including objectivity of measurement procedures, test-retest reliability, simple subject preparation, readily available instrumentation, and relatively brief examination periods. The fine resolution of DPEs within the stimulus frequency and level domains also permits an accurate confirmation of the pattern of hearing loss. For example, tests of DPEs detected a 20-dB hearing level impairment at a single frequency in an ear exhibiting early signs of noise-induced hearing loss, and a 10-dB improvement in sensitivity following ingestion of the hyperosmotic agent glycerol in an ear displaying a mild to moderate hearing loss due to Meniere's disease. Finally, the application of DPEs to the objective testing of otologic disorders suggests that the ability of these responses to assess the sensory component of a sensorineural disorder may contribute to the eventual understanding of the complicated pathogenesis of many cochlear diseases. When all the positive features of DPE testing are realized, the potential contribution that these measures can make in a clinical setting becomes apparent.
Adult↗
Neural infiltration of glomus temporale tumors.
Biologic behavior of glomus temporale tumors in relation to cranial nerve infiltration remains unclear. An analysis of cranial nerve involvement in relation to tumor staging was undertaken in a series of 102 glomus temporale tumors. Frequent neural infiltration was noted to occur in large glomus tumors, and a grading system of infiltration with both pathologic and surgical relevance introduced.
Adolescent↗
Menière's symptoms resulting from bilateral otosclerotic occlusion of the endolymphatic duct: an analysis of a causal relationship between otosclerosis and Menière's disease.
An association between otosclerosis and Menière's disease has been proposed on both a clinical and temporal bone histopathologic basis for well over three-quarters of a century. Controversy persists over a causal relationship between these two entities, however, and the underlying pathophysiologic mechanisms relating capsular otosclerosis with Menière's disease remain speculative. The first case of total, bilateral endolymphatic duct occlusion resulting from extensive capsular otosclerosis is presented in a woman manifesting Menière's symptoms. The severe endolymphatic hydrops resulting from otosclerotic endolymphatic duct occlusion is given as histopathologic proof of a causal relationship between these two entities. Analysis of 18 of our own temporal bone cases of extensive capsular otosclerosis without endolymphatic hydrops and review of the literature indicates the uniqueness of such a firm causal relationship in the present case.
Adult↗
Sudden sensorineural hearing loss as a presentation of multiple sclerosis.
While auditory involvement in multiple sclerosis is not infrequent, sensorineural hearing loss as the initial manifestation of this disease is rare. Auditory brain-stem evoked responses, speech audiometry, acoustic reflexes, and electronystagmography yield significant objective information concerning brain-stem involvement. Magnetic resonance imaging objectively documents white matter demyelinization. The acute onset of the retrocochlear type of auditory dysfunction without other evidence of central nervous system involvement can be an early manifestation of this disease. Two patients with multiple sclerosis had sudden sensorineural hearing loss, with one patient experiencing this as the sole initial complaint.
Acute Disease↗
Management of petrous apex lesions.
Advancements in imaging pose new diagnostic and therapeutic dilemmas as smaller lesions in the relatively inaccessible regions of the petrous apex and clivus are identified. Differentiation of true pathology from artifact and anatomic variants is critical in management of these lesions. We describe our experience with five patients diagnosed with petrous apex lesions: three, cholesteatomas; one, cholesterol granuloma; and one, false-positive. Soft-tissue obliteration of the temporal bone defect with postoperative follow-up using high-resolution computed tomographic scanning and magnetic resonance imaging is proposed as an alternative to exteriorization.
Adolescent↗
Preauricular pilomatrixoma: a diagnostic pitfall.
Although pilomatrixomas are well known to dermatologists and dermatopathologists, surgeons encountering these lesions over the parotid gland frequently do not consider this benign neoplasm in the differential diagnosis. Presentation of a subcutaneous preauricular lesion, coupled with an erroneous cytologic diagnosis of malignancy, can potentially result in histologic "overdiagnosis" and more aggressive therapy than is required for these benign hair follicle neoplasms. A case is reported to illustrate this potential diagnostic trap, and a series of 206 cases collected at the University Hospital of Zurich from 1975 to 1987 is reviewed.
Adolescent↗
Jugular foramen peripheral nerve sheath tumors.
Though jugular foramen nerve sheath tumors are uncommon, they involve a critical area of the skull base. Therefore, a precise classification system is needed to accurately define the extent of these tumors and reflect their surgical management. A series of seven cases is reviewed incorporating such a classification system to illustrate the management of these lesions using the infratemporal fossa type A approach.
Adolescent↗
An investigation into EEG asymmetry in young children.
Language performance is thought to be related to the functional specialisation of the brain, and particularly to the degree of asymmetry of activity in the two cerebral hemispheres. A microprocessor-based system has been constructed to investigate the relationship between the analogous asymmetry of the EEG and language ability in young children. The system is based on the Intel 8086 microprocessor, with software written both in the high level language PL/M-86 and 8086 assembly language. A test script, instructing the child to perform various lateralised tasks, is played from a cassette recorder to provide a constant stimulus across subjects. The computed power spectra of the resulting EEG waveforms, and the hemispheric specialisation, represented by a derived dominance factor, have been analysed both visually and numerically. Results from a test group of 16 children have not shown a strong correlation between the dominance factors and the related reading quotient.
Alpha Rhythm↗
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.
Diagnosis, Differential↗
Sjögren's syndrome in children.
Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of the lacrimal and salivary glands; it eventually leads to keratoconjunctivitis sicca and xerostomia. The disease usually affects middle-aged women and is second to rheumatoid arthritis as the most commonly diagnosed connective tissue disorder. Surprisingly, only 31 cases have been reported in the pediatric population. The diagnosis should be considered, however, in children with a foreign body sensation in the eyes, chronic dryness of the eyes or mouth, or recurrent salivary gland enlargement. This has prompted us to describe our experience in treating five children with SS at Texas Children's Hospital, Houston. One 5-year-old child with no other evidence of connective tissue disease was diagnosed as having primary SS. The remaining four children either had juvenile rheumatoid arthritis or systemic lupus erythematosus in addition to SS, and the diagnosis of secondary SS was made. Other autoimmune disorders associated with SS include polyarteritis nodosa, scleroderma, and polymyositis. Interestingly, one child with secondary SS and chronic lymphocytic thyroiditis developed papillary carcinoma of the thyroid. Both forms of SS are probably more prevalent than the literature would suggest. Recognition of this fact is important if the immediate complications of corneal damage and severe dental decay are to be prevented. Symptomatic treatment with steroids, eyedrops, artificial saliva, and prophylactic fluoride treatments is available. With an adequate follow-up program the late morbidity associated with SS, such as malignant disease, can be minimized.
Adolescent↗