Bobby R. Alford

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.

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 worlds 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.

Dichotic listening, event-related potentials, and interhemispheric transfer in the elderly

Objective To determine the basis for the large, age-related asymmetries in dichotic listening performance scores reported by Jerger et al. (1994). Design Behavioral and electrophysiologic responses to dichotic listening tasks in both verbal and nonverbal paradigms were obtained in four groups of subjects: young adults with normal hearing, elderly persons with presbyacusis, elderly persons with presbyacusis and marked dichotic deficits, and patients with lesions of the corpus callosum. Results In comparison with the young group the two elderly groups showed an increasing left-ear deficit on the verbal task, and an increasing right-ear deficit on the nonverbal paradigm. The pattern of results obtained in the elderly persons with marked dichotic deficits was similar to the pattern of results in the group with callosal lesions. Conclusions With age, there may be a significant loss of efficiency of interhemispheric transfer of auditory information through the corpus callosum. Such age-related deficit might have important implications for the effective use of binaural information by elderly persons.

Development of speech intelligibility in children with recurrent otitis media.

This study defined developmental functions for Pediatric Speech Intelligibility word and sentence materials presented in quiet and in competition for 25 normal children and 25 children with recurrent otitis media. Ages ranged from 24 to 56 mos. In normal children, developmental functions for Pediatric Speech Intelligibility speech materials showed earlier development of performance (1) in quiet than in competition and (2) for words than for sentences (competing condition). In children with otitis media, developmental functions were normal for both words and sentences in quiet and for sentences in competition. However, developmental functions were grossly abnormal for words in competition.

Traumatic Infratemporal Facial Nerve Injury: Management Rationale for Preservation of Function

A retrospective review of 29 cases of infratemporal 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 transmastold-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.

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.

INNER EAR MORPHOLOGY IN DOWN'S SYNDROME

A comparative study was made on four pairs of temporal bones from patients with Downs syndrome (trisomy 21) and 15 pairs of temporal bones from other infants of the same age range. Spiral reconstructions showed chochlear length to be slightly shorter in temporal bones from patients with Downs syndrome than that in the controls. Based upon these dimensional measurements, a developmental anomaly of the vestibular apparatus was found.

Current concepts of frontal sinus surgery: An appraisal of the osteoplastic flap-fat obliteration operation†‡

The osteoplastic flap‐fat obliteration operation has been utilized in a variety of clinical problems (osteomas, chronic infection, mucocele, extensive trauma) for the past eight years. The experience suggests that in carefully selected patients it is an effective operative procedure for the surgical control of these problems. Two failures, which were probably preventable, occurred in the 53 patients operated upon by this technique. Long term animal experiments and clinical experience support the merit of using fat as an obliterating material in the infected sinus. The only unfavorable long term result in otherwise successful patients has been the forehead embossment that has occurred over the frontal sinus osteoplastic flap area.

Frey's Syndrome - A Technical Remedyr

The symptoms of Freys syndrome are occasionally of sufficient magnitude to warrant curative treatment. In four patients, a fascia lata graft was placed under the involved skin in an attempt to offer permanent relief from the cutaneous discomfort suffered during eating. Two of these patients have excellent long-term results. The other two patients were lost to long-term follow-up but were asymptomatic several months following grafting.

Inner Ear Anomalies in Dogs

Congenital deafness in dogs has been known for a long time and inner ear anomalies have been previously documented in different kinds of dogs: in the white Bull-Terrier,l in the Dalmatian,*-5 and in the Collie.* Cochleosaccular degeneration was found exclusively in these reports. In the present study, by utilizing serial temporal bone sections, inner ear end organs in four different species of dogs were studied in order to investigate the developmental pathology in the different structures of the inner ear apparatus among these species.