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B R Alford

Publications and source records attributed to B R Alford.

At least 19 recordsLinked to original sources
PubMed1992

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.

Adolescent↗
PubMed1991

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.

Aerospace Medicine↗
PubMed1990

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.

Audiometry, Evoked Response↗
PubMed1990

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.

Adolescent↗
PubMed1989

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.

Adrenal Gland Neoplasms↗
PubMed1989

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.

Blindness↗
PubMed1989

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.

Amyloidosis↗
PubMed1987

Intracranial and intratemporal facial neuroma.

Primary tumors of the facial nerve are relatively rare and have a variety of presenting symptoms. This article reviews 248 cases of facial neuroma in the world's literature and adds seven cases that were managed at the Baylor College of Medicine. Facial weakness was most common symptom, with facial spasm or tics, hearing loss, and masses in the external auditory canal also being frequently seen. At surgery, the tympanic, vertical, and labyrinthine segments were the most commonly involved areas. On the basis of this review, general principles have been drawn up to help the clinician in diagnosis and management of this (at times) difficult problem.

Adult↗
PubMed1987

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.

Facial Nerve↗
PubMed1985

Tumors of the parapharyngeal space.

We retrospectively studied tumors of the parapharyngeal space treated at the Baylor College of Medicine Affiliated Hospital System, Houston, from 1972 to 1985. Of the 42 lesions, 30 (71.4%) were benign and 12 (28.6%) were malignant. Tumors of neurogenic origin were present in 17 (40.5%). Tumors of salivary gland origin were present in 16 (38.1%): ten were benign, six were malignant. Nine (21.4%) of the patients presented with miscellaneous lesions, six of which proved to be malignant. We have found that a preoperative arteriogram is no longer routinely indicated. High-resolution computed tomography is now the best initial diagnostic study because it helps determine the size and extent of the tumor, differentiate tumors of parotid and extraparotid origin, demonstrate degree of tumor vascularity, and separate benign from malignant lesions.

Adenoma, Pleomorphic↗
PubMed1980

Effect of off-vertical tilt and macular ablation on postrotatory nystagmus in the squirrel monkey.

The slow phase eye velocity (SPEV) and duration of post-rotatory nystagmus (PRN) were studied in squirrel monkeys (Saimiri sciureus) after a ramp speed rotation (0-200 degrees/sec, with 1 degree/sec2 angular acceleration). When the results were compared between straight upright vertical rotation, 9 degrees tilt rotation, and 18 degrees tilt rotation, faster decay both in SPEV and in duration was found in the tilt rotation situations. Difference in nystagmic decay curves by tilting rotation axis could be from the convergence of macula-semicircular canal inputs. Subsequently bilateral macular ablation (two-stage) was performed. The difference in nystagmus decay curves between three different rotations was reduced; therefore, the change of gravity direction perceived through gravity receptors other than macular endorgans was minimal and did not produce a difference in three different rotations.

Acoustic Maculae↗
PubMed1979

Otorhinolaryngology.

Explore the source record for details and available documents.

Head and Neck Neoplasms↗
PubMed1979

Bilateral sudden hearing loss and metastatic pancreatic adenocarcinoma.

We report a rare case of bilateral sudden and profound hearing loss caused by bilateral temporal bone metastasis from a primary adenocarcinoma of the tail of the pancreas. The hearing loss was the patient's only reason to seek medical care. Seventh nerve involvement was very minimal, when compared with that of the eighth nerve.

Adenocarcinoma↗
PubMed1979

Detection of foreign bodies with computerized tomography.

Computerized tomography scanning has been proven useful in defining complex anatomical areas of the head and neck and in delineating soft-density foreign bodies. This was helpful in a child in whom all other modalities, including surgical exploration, had failed to localize a foreign body.

Abscess↗