Historical ties between otolaryngology-head and neck surgery and aviation and space medicine.

Otolaryngology-head and neck surgeons have been involved in the development of aviation and space medicine since the beginning of this century. More than 75 years ago, otolaryngologists revised the physical examination for pilots, organized "boards of medical examiners" to test pilot applicants, coined the term "flight surgeon," and helped organize the first medical research laboratories at Hazelhurst Field in New York. These laboratories were transformed in 1922 into the School of Aviation Medicine at Brooks Field, Texas, which in turn subsequently was relocated to Randolph Field, Texas. During World War II the Director of Research at the school was Colonel Paul A. Campbell, MD, an otolaryngologist. In 1959, the school moved back to Brooks Air Force Base and was renamed the Aerospace Medical Center. Since manned space flight began in the 1960s there have been many joint research efforts between principal investigators in otolaryngology-head and neck surgery and NASA. Several otolaryngology-head and neck surgeons have served or currently serve as consultants and advisors to many of NASA's standing committees. The space environment offers a new frontier for development and research in the specialty and for better understanding of vestibular function and related disorders.

Neuroendocrine neoplasms of the larynx.

Neuroendocrine neoplasms of the larynx are a rare group of tumors that include carcinoid tumor, atypical carcinoid tumor, and small cell carcinoma. These neoplasms pose interesting diagnostic, prognostic, and therapeutic dilemmas, and they are, as a whole, aggressive tumors with a tendency for local and distant spread. The authors of this study examined six new cases of laryngeal neuroendocrine neoplasms. One case manifested itself as a primary atypical carcinoid tumor and caused a "carcinoid syndrome." The remaining five cases were small cell carcinomas of the larynx. Histologic, immunocytochemical, DNA flow cytometric, and p53 studies were performed on all cases. The expression of neuron-specific enolase and chromogranin were the most useful markers in this group of tumors. Overexpression of p53 protein was present in the majority of cases, including the atypical carcinoid tumor. The implications of these studies for diagnosis, classification, and treatment are discussed.

Radiation therapy in the management of paragangliomas of the temporal bone.

The use of radiation therapy for the treatment of paragangliomas of the temporal bone remains controversial. Few studies exist that provide sufficient information on staging the long-term follow-up to clarify this issue. A retrospective study was undertaken of 38 patients with temporal bone paragangliomas treated with radiation therapy between 1956 and 1991. All patients received radiation therapy, either as a primary modality (14 patients), in combination treatment with surgery (13 patients), or as salvage therapy (11 patients). Mean disease stage (Fisch classification system) for each group was C2, B, and C1, respectively. The follow-up period ranged from 1 to 27 years (median 11.5 yr). Local control was achieved in 79% of the primary radiation therapy group, 100% of the combined treatment group, and 91% of the salvage therapy group. Complications resulting from radiation therapy were few and minor. This study demonstrates that the use of radiation therapy for temporal bone paragangliomas offers effective local control as a primary treatment modality, in combination with surgery, and as salvage therapy with few long-term complications.

Atypical and malignant lipomatous lesions of the head and neck.

Although liposarcoma is the second most common soft-tissue sarcoma in adults, the incidence of liposarcoma of the head and neck is low. There are only 83 reported cases of head and neck liposarcoma. We report four cases of atypical and malignant lipomatous lesions of the head and neck and discuss their histologic classification and treatment implications. The histologic nature of liposarcoma is correlated clinically with treatment outcome. Recently, many authors have adopted the term atypical lipoma to describe well-differentiated lipomatous lesions in superficial extremity locations because of their tendency toward local recurrence only, without metastases or patient mortality. Although we accept the classification atypical lipoma for superficial, well-differentiated lesions, we believe that histologically similar lesions in nonsuperficial locations in the head and neck are best designated "well-differentiated liposarcoma."

Temporal bone findings in craniodiaphyseal dysplasia.

To our knowledge, this is the first description of the histopathology of the temporal bone from a case of craniodiaphyseal dysplasia. The air spaces of the mastoid, external auditory canal, and middle ear cavity are reduced by hyperostotic bone. The ossicles are also deformed by the bony overgrowth. Anomalous ossicles with hyperostosis could affect the air conduction. Even though the internal auditory canal is somewhat elongated and narrow, no labyrinthine abnormalities can be attributed to the abnormal bone. Facial nerves run through abnormal courses but the geniculate ganglion cells are not involved. The VIIIth nerve dysfunction in this case could be due to mechanical damage of the nerve fibers and/or impaired vascular supply by the hyperostosis.

Otoconia in young and elderly persons: a temporal bone study.

Using serially sectioned human temporal bones, the volumes of otoconia masses were compared between young children and elderly people. The latter group had significantly less otoconia volume than the former group, both in the macula utriculi and macula sacculi. The ratio between macula utriculi and macula sacculi was 100:70 in the young group, whereas it was 100:35 in the elderly group. Thus, the macula sacculi in the elderly group showed the severest otoconia reduction.

Characterization of the clinical features of five families with hereditary primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2.

The hereditary conditions of primary cutaneous lichen amyloidosis and multiple endocrine neoplasia type 2 (MEN 2) are rare clinical entities. The initial reports of two families in which the two conditions coincided have led to the identification of at least eight additional families with this clinical syndrome. In this report we describe the clinical features in five of these eight families. The salient feature in these five families is the presence of unilateral (46%) or bilateral (64%) pruritic and lichenoid skin lesions located over the upper portion of the back. Family members describe these skin lesions as intermittently intensely pruritic leading to scratching and excoriation of the upper back region. The presence of MEN 2 has been documented in 97% of family members with this skin lesion, the one exception being a child who is at risk for development of MEN 2A in whom the diagnosis has not yet been made. Of family members who have MEN 2A, 27% do not have an identifiable skin lesion, although the skin lesion developed in one patient two years after a curative thyroidectomy for medullary thyroid carcinoma (MTC). Four of the five families have members with pheochromocytoma; one with five affected members has only MTC. The finding of this clinical syndrome in geographically diverse portions of the world and the lack of overlap with MEN 2A without the skin lesion suggest it is a distinct clinical variant of MEN 2A.

Historical ties between otolaryngology--head and neck surgery and space medicine.

Otolaryngologist--head and neck surgeons have been involved in the development of aviation and space medicine since the beginning of this century. In the late 1910s, otolaryngologists revised the physical examination for pilots, organized "Boards of Medical Examiners" to test pilot applicants, coined the term "flight surgeon," and helped organize the Medical Research Laboratories at Hazelhurst Field in New York. These laboratories were transformed into the School of Aviation Medicine at Brooks Field, Texas, which was relocated to Randolph Field, Texas, in 1934. During World War II, the director of research at the school was Colonel Paul A. Campbell, MD, an otolaryngologist. In 1959 the school moved back to Brooks Air Force Base and was renamed the Aerospace Medical Center. In 1962, Dr. Campbell served as the director. Since manned space flight began in the 1960s, there have been many joint research efforts between principal investigators in otolaryngology--head and neck surgery and NASA. Currently, many otolaryngologist--head and neck surgeons serve as consultants and advisors to many of NASA's standing committees. The space environment offers a new frontier for specialty development and research in otolaryngology--head and neck surgery.

A comparative study of the fallopian canal at the meatal foramen and labyrinthine segment in young children and adults.

If entrapment is a critical factor in pathogenesis, the lower incidence of Bell's palsy in children compared with adults may have an anatomical basis. Histologic sections of 20 temporal bones from children younger than 2 years were examined to determine the diameter of the facial nerve and fallopian canal at the meatal foramen and in the labyrinthine segment. No statistically significant difference in the nerve/canal ratios in these areas was found. Comparisons were made with similar data from 10 adult temporal bones. The nerve/canal ratios in the labyrinthine portion were similar in both age groups; however, the ratio at the meatal foramen was significantly smaller in children. This may be due, in part, to growth of the vertical crest, which was found to increase considerably in length and width by adulthood. These results suggest that the facial nerve is not as tightly contained at the meatal foramen in children and provides a possible explanation for the relative infrequency of Bell's palsy in this age group.

Recurrent meningitis and Mondini dysplasia.

Three infants with cerebrospinal fluid otorrhea and recurrent meningitis due to Mondini dysplasia have been treated at Baylor College of Medicine, Houston, Tex; 39 other patients with this association have been described. This review highlights the characteristics and embryologic basis of the malformation. The clinical evaluation of an infant with recurrent meningitis is outlined, and the importance of brain-stem response audiometry and thin-cut computed tomography of the temporal bones is discussed. Surgical management depends on the functional status of the dysplastic ear; usually, a stapedectomy is performed and the vestibule is obliterated. Although the surgical failure rate is 30% after one procedure, early identification of the dysplasia and prompt surgical management diminish morbidity and mortality and permit early habilitation of the child with congenital hearing impairment.

Multiple endocrine neoplasia type 2a associated with cutaneous lichen amyloidosis.

PURPOSE: To describe and characterize the association of hereditary cutaneous lichen amyloidosis with multiple endocrine neoplasia type 2a (MEN 2a). DESIGN: Survey of a family for two diseases. SETTING: Evaluation of patients at a clinical research center. PATIENTS: Nineteen family members with MEN 2a. MEASUREMENTS AND MAIN RESULTS: In this family cutaneous lichen amyloidosis presented as multiple infiltrated papules overlying a well-demarcated plaque in the scapular area of the back (right or left). Immunohistochemical studies showed amyloid that stained for keratin but not calcitonin. Three family members had the characteristic skin lesion and also carried the gene for MEN 2a; two additional members carried the gene for MEN 2a, but did not manifest the observable skin changes associated with lichen amyloidosis. CONCLUSIONS: From the findings in this kindred and in another recently reported but unrelated family with an identical type of pruritic skin rash and MEN 2a, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2a is a clearly defined autosomal dominant hereditary syndrome. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than by calcitonin-like peptides. Third, known families with hereditary lichen amyloidosis should be screened to determine the true frequency of this syndrome.

Familial cutaneous lichen amyloidosis in association with multiple endocrine neoplasia type 2A: a new variant.

Multiple endocrine neoplasia type 2A (MEN 2A) is a rare hereditary disease transmitted in families as an autosomal dominant trait. We have identified a family in which the expression of a rare autosomal dominant form of cutaneous lichen amyloidosis appears to cosegregate with MEN 2A. In this family the skin lesion presented as multiple infiltrated papules overlying well demarcated plaques over the scapular area (right or left). Immunohistochemical studies demonstrated amyloid which stained for keratin but not calcitonin. A total of 19 members were screened. Three members of the family have the characteristic skin lesion and MEN 2A; two additional members have MEN 2A but have not manifested observable skin changes of lichen amyloidosis. Another unrelated Italian family with a similar type of pruritic skin rash and MEN 2A has been reported recently. Although the initial skin biopsies were negative for amyloidosis, subsequent biopsy established the association of MEN 2A with amyloidosis in this family also. When these kindreds are combined, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2A appears to be a clearly defined autosomal dominant hereditary syndrome. Whether this syndrome can be linked to chromosome 10 is not yet known. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than calcitonin-like peptides. Third, we believe that patients with the hereditary form of cutaneous amyloid should be screened for medullary thyroid carcinoma to determine the true frequency of this syndrome.

Exclusion of Usher syndrome gene from much of chromosome 4.

Usher syndrome is an autosomal recessive disease characterized by dual sensory impairments; affected individuals are born with a sensorineural hearing loss and ultimately lose their sight as retinitis pigmentosa develops. Conventional protein markers previously tested in a Louisiana Acadian kindred suggested tentative linkage to vitamin D-binding protein on chromosome 4. DNA linkage studies do not confirm this linkage relationship and exclude much of chromosome 4 as the site of the Usher syndrome gene in these families.

Stapedectomy trends for the resident.

The diminishing number of cases of otosclerosis limits the training experience in stapes surgery during residency. Results are suboptimal. Closure of the preoperative air conduction to within 10 dB of bone conduction was obtained in only 64% of resident cases. Efforts to maximize experience and improve results include closer faculty supervision, use of one stapedectomy technique, and a prospective study of individual residents' performance.

Traumatic intratemporal facial nerve injury: management rationale for preservation of function.

A retrospective review of 29 cases of intratemporal facial nerve injuries included 18 temporal bone fractures, 7 gunshot wounds, and 4 iatrogenic complications. Surgical exploration confirmed involvement of the fallopian canal in the perigeniculate region in 14 longitudinal and 3 transverse or mixed fractures of the petrous pyramid. Gunshot and iatrogenic injuries usually occurred within the tympanic and vertical segments of the facial canal and at the stylomastoid foramen. When hearing is salvageable, the middle fossa approach provides the best access to the perigeniculate region of the facial nerve. In the presence of severe sensorineural hearing loss, the transmastoid-translabyrinthine approach is the most appropriate for total facial nerve exploration. Grade I to III results can be anticipated in timely decompression of lesions caused by edema or intraneural hemorrhage. Undetectable at the time of surgery, stretch and compression injuries with disruption of the endoneural tubules often lead to suboptimal results. Moderate-to-severe dysfunction (Grade IV), with slight weakness and synkinesis, is the outcome to be expected from the use of interpositional grafts.