Speech outcomes after tonsillectomy in patients with known velopharyngeal insufficiency.

Introduction. Controversy exists over whether tonsillectomy will affect speech in patients with known velopharyngeal insufficiency (VPI), particularly in those with cleft palate. Methods. All patients seen at the OHSU Doernbecher Children's Hospital VPI clinic between 1997 and 2010 with VPI who underwent tonsillectomy were reviewed. Speech parameters were assessed before and after tonsillectomy. Wilcoxon rank-sum testing was used to evaluate for significance. Results. A total of 46 patients with VPI underwent tonsillectomy during this period. Twenty-three had pre- and postoperative speech evaluation sufficient for analysis. The majority (87%) had a history of cleft palate. Indications for tonsillectomy included obstructive sleep apnea in 11 (48%) and staged tonsillectomy prior to pharyngoplasty in 10 (43%). There was no significant difference between pre- and postoperative speech intelligibility or velopharyngeal competency in this population. Conclusion. In this study, tonsillectomy in patients with VPI did not significantly alter speech intelligibility or velopharyngeal competence.

Treatment patterns of pediatric nontuberculous mycobacterial (NTM) cervical lymphadenitis as reported by nationwide surveys of pediatric otolaryngology and infectious disease societies.

OBJECTIVE: To describe physician diagnostic and therapeutic strategies for pediatric nontuberculous mycobacterial (NTM) lymphadenitis, a disease for which surgical excision is recommended. METHODS: We surveyed members of the Infectious Diseases Society of America Emerging Infections Network (EIN) and the American Society of Pediatric Otolaryngology (ASPO). We asked them to report clinical and microbiologic details of recent cases of NTM lymphadenitis seen in their practices. RESULTS: 200 physicians reported a total of 277 NTM lymphadenitis cases. Cervical lymph nodes (84%) were most frequently involved, and a majority of patients were non-Hispanic white (62%) males (54%) with median age 3.0 years. Tissue culture (61%) or polymerase chain reaction (12%) was utilized most frequently to confirm NTM etiology. In most (59%) cases, an etiologic organism was not identified. In cases, where an NTM organism isolate was identified, Mycobacterium avium complex (n=82, 72%) was the most common. Surgical excision followed by adjunctive antibiotic therapy was favored in the majority (59%) of cases where a treatment method was reported. The use of surgical excision alone or antibiotic therapy alone was reported respectively in 24% and 17% of cases. Antibiotics were prescribed without diagnostic confirmation of infectious organisms in 28% of cases. CONCLUSION: Pediatric otolaryngologists and infectious disease specialists frequently treat cervical lymphadenitis empirically as NTM disease without bacteriologic confirmation. Antibiotic therapy is frequently employed with or without surgical excision.

Neonatal hearing screening with otoscopy, auditory brain stem response, and otoacoustic emissions.

A study was performed to investigate the relationship between external and middle ear factors and hearing screening results by auditory brain stem response (ABR) and transient evoked otoacoustic emissions (EOAEs). The ears of 200 well newborns aged 5 hours to 48 hours underwent screening by ABR and EOAEs, followed by otoscopic examination. The pass rates for ABR and EOAE screening were 88.5% and 79%, respectively. On otoscopic examination, 13% (53 of 400) ears had occluding vernix obscuring the view of the tympanic membrane. Cleaning of vernix was attempted in ears that failed ABR or EOAE screening. Seventeen ears that failed ABR were cleaned, and 12 (71%) of them passed repeat ABR. Thirty-three ears that failed EOAE screening were cleaned, and 22 (67%) of them passed repeat emissions testing. Cleaning vernix increased the pass rates for ABR and EOAE screening to 91.5% and 84%, respectively. Decreased tympanic membrane mobility was found in 9% of ears that could be evaluated otoscopically. Increased failure rates for both ABR and EOAE screening were found in infant ears with decreased tympanic membrane mobility, but significance testing could not be performed because of inadequate sample size. Prevalence of occluding external canal vernix and middle ear effusion as a function of increasing infant age were studied. Implications for newborn hearing screening are discussed.

Otolaryngologic manifestations of multiple pterygium syndrome.

Escobar Syndrome, or Multiple Pterygium Syndrome (MPS), is a rare syndrome with multiple congenital anomalies involving the head and neck area and limbs. Affected individuals have multiple pterygia, camptodactyly and/or syndactyly as the main features of this syndrome. Patients with MPS have a characteristic facies, including ptosis, antimongoloid slant of the palpebral fissures, hypertelorism, micrognathia, neck pterygia, and a sad flat, emotionless look to the face. We present a case of MPS recently treated at our institution for the purpose of further describing the clinical features of this syndrome, emphasizing the otolaryngologic manifestations. Increased awareness of MPS will facilitate appropriate management of this syndrome.

The use of interferon alfa-2a for life-threatening hemangiomas.

Hemangiomas are the most common benign neoplasm in the neonatal period. While most small hemangiomas involute spontaneously, up to 20% may grow to a massive size and never completely involute. Massive hemangiomas are also frequently associated with life-threatening complications, such as airway obstruction, platelet trapping (Kasabach-Merritt syndrome), and high-output heart failure. The use of interferon alfa-2a for the treatment of massive hemangiomas not responsive to traditional therapy has recently been reported. We present the successful use of interferon alfa-2a in a series of five patients with massive hemangiomas complicated by airway compromise, congestive heart failure, need for tracheotomy, Kasabach-Merritt syndrome, and failure to thrive, despite traditional therapy.

Voice quality after laryngotracheal reconstruction.

OBJECTIVE: To ascertain changes in laryngeal anatomy and function associated with poor voice outcome after laryngotracheal reconstruction (LTR) and to specifically describe dysphonia in pediatric patients treated for subglottic stenosis. DESIGN: A survey was undertaken of patients having undergone LTR for subglottic stenosis. Twelve patients were recruited during the year 1990-1991. Nine patients were able to complete the endoscopic portion of the protocol that included fiberoptic and direct laryngoscopy. Speech samples were qualitatively evaluable in only six of nine patients (vocal quality, loudness, and intelligibility) and objectively in five of nine patients (fundamental frequency and pitch perturbation). PATIENTS: All patients in this consecutive sample had undergone LTR for congenital or acquired subglottic stenosis. The average age was 6 years. Each patient had previously undergone an average of two prior open laryngeal procedures and five endoscopic procedures. OUTCOME MEASURES: Correlation between anatomic and functional changes in the post-LTR larynx and voice outcome. RESULTS: Endoscopic evaluation of the post-LTR larynx showed a 78% incidence of altered anatomy and 44% incidence of altered function. Speech sample analysis showed the following: 100% decreased vocal quality, 50% decreased intelligibility, 100% decreased volume, and 80% low fundamental frequency and increased jitter. CONCLUSIONS: Children with high-grade subglottic stenosis and multiple prior surgeries are at high risk for poor voice outcome after LTR. Future prospective studies will allow substantiation of this observation and clarification of which changes in laryngeal structure and function arise from surgery per se.

Open reduction-fixation of mandibular subcondylar fractures. A review.

From 1973 to 1990, 392 mandibular subcondylar fractures were treated at the University of California, Davis, by the Otolaryngology Department. Of these, 17% were handled by open reduction and internal fixation. Twenty-one patients from this group were located for follow-up at an average interval of 64 months. Retrospective review shows the operation to be safe, with few complications and no permanent sequelae. Patient examination often revealed abnormalities of occlusion and mandibular function; however, these objective findings did not correlate well with patients' relative lack of subjective complaints. An 86% incidence of roentgenographic evidence of condylar disease after open reduction and internal fixation was found. We question the long-term efficacy of open reduction and internal fixation in restoring fracture alignment and maintaining mandibular height given the high rate (86%) of condylar disease in our patient population.

Sinus complications in mucopolysaccharidosis IH/S (Hurler-Scheie syndrome).

Hurler-Scheie syndrome is a genetic compound of two mucopolysaccharidoses, the Hurler and Scheie syndromes. The genetic error of metabolism caused by this syndrome produces intermediary systemic effects in the affected individuals. Lacking the enzyme alpha-L-iduronidase, glycosaminoglycans are deposited in the tissues, causing multiple systemic effects and creating many problems for the otolaryngologist. Although early bone marrow transplantation is currently being performed to prevent or reverse many of the systemic manifestations of this disorder, there remains a large population of children past the age at which bone marrow transplantation can be effective. Sinus complications have not previously been reported in the mucopolysaccharidoses and may be more frequently recognized as these patients' longevity increases. We present a patient with Hurler-Scheie syndrome who developed severe nasal polyposis requiring sinus endoscopic removal.

Vincristine-induced recurrent laryngeal nerve paralysis.

Vincristine-sulfate-related vocal cord paralysis has been reported infrequently in the literature. The neurotoxicity of the vinca alkaloids is well-known; however, the potential for cranial nerve involvement is not widely recognized. Given the complexity of the typical patient receiving such a chemotherapeutic agent, the potential for misdiagnosis is high. Many patients have primary tumors or metastatic lesions in sites that could cause the clinician to overlook this reversible cause of neurologic dysfunction. This study describes the first three reported pediatric cases of vincristine-induced vocal cord paralysis. Two patients developed increasing stridor secondary to bilateral vocal cord paralysis; the third developed a unilateral vocal cord paralysis. All resolved spontaneously upon withdrawal of the vincristine. Vinca-alkaloid-induced vocal cord paralysis is a potentially dangerous but reversible lesion. Otolaryngologists should be aware of the association between these agents and cranial nerve neuropathies.

Variations of the anterior cricoid split: the effect on airway cross-sectional area in the rabbit.

The anterior cricoid split (ACS) operation has been documented as a successful treatment for the difficult-to-extubate infant or child. The exact mechanism by which this operation is successful is still being investigated. In an effort to further elucidate the effect of the ACS procedure on the subglottic airway, four groups of rabbits were studied. Both size and shape of the subglottic area after surgical manipulation were evaluated, comparing control rabbits with ACS, anteroposterior cricoid split (A-PCS), and partial cricoid split with sparing of soft-tissue lining (partial ACS). The animals were sacrificed 4 weeks after surgery, and the larynxes were harvested. Analysis showed a significant enlargement of the subglottic lumen in all experimental groups as measured at the inner lamina of the cricoid cartilage. However, the true subglottic lumen was significantly enlarged only in the ACS group. Findings showed that true enlargement of the subglottic lumen was limited in all experimental groups to increased thickness in the soft-tissue lining of the subglottic lumen, which was most marked in the A-PCS group. A significant change in subglottic lumen shape occurred in the A-PCS group as well. These results suggest the A-PCS should be approached clinically with caution when a stent is not used.

Pediatric head and neck rhabdomyosarcoma.

Rhabdomyosarcoma (RMS), the most common malignant soft-tissue tumor in the pediatric population, occurs most often in the head and neck region. Major advancements in the management of these aggressive tumors have been achieved in the last 20 years on three fronts: chemotherapy, radiation therapy, and surgery. Surgery, with the advent of skull-base, craniofacial, and free-tissue-transfer reconstructive techniques, has once again become part of the primary management of these tumors. Prior to 1960, RMS was uniformly fatal; now, with the introduction of multimodality treatment regimens, two thirds of patients can expect long-term survival.