Hearing loss and congenital symptomatic cytomegalovirus infection: a case report of multidisciplinary longitudinal assessment and intervention.

More than 6000 children born annually in this country have hearing loss resulting from congenital cytomegalovirus (CMV) infection, the leading nonhereditary congenital cause of hearing loss in children. This exemplary congenital symptomatic CMV case focuses on the results of longitudinal audiologic, educational, medical, psychological, and visual evaluations and intervention. Decreased ocular motor control and visual acuity were observed as was bilateral deterioration of hearing from 3 days though 9 years of age. Treatment with dexamethasone and histamine resulted in almost complete reversal of the most recent progression of hearing loss in the left ear.

High-resolution computed tomography: determination of the cause of pediatric sensorineural hearing loss.

High-resolution computed tomograms (HRCT) reveal strikingly little variation in the dimensions of inner ear structures among people with normal hearing. However, a subgroup of people exist who have a sensorineural hearing loss (SNHL) and morphologically normal HRCT scans, yet have subtle radiographic aberrations based on measurements of inner ear dimensions. These changes might have resulted from teratogenic influences during the late first to mid second trimester, a time when the inner ear has achieved adult form, yet continues to enlarge to adult size. A retrospective study of HRCTs on three groups of ears was performed to determine inner ear structure dimensions. Group I, the control group, consisted of 50 ears with no SNHL and no discernible abnormalities on HRCT. In group II, there were 70 ears with SNHL and obvious abnormalities on HRCT. Group III, the test group, was composed of 140 ears with SNHL and "normal" HRCTs. Groups II and III were analyzed, applying the normal dimensions derived from group I. When compared to the control group (I), all of the ears in group II were abnormal. Moreover, 75% of the ears in group III had subtle differences not apparent unless measurements were made. Two or more abnormal dimensions were found in 32%. Findings and implications are thoroughly discussed.

Cavernous hemangiomas of the internal auditory canal.

Cavernous hemangiomas are vascular malformations that are seldom found in the central nervous system. Four cavernous hemangiomas of the internal auditory canal and one of the cerebellopontine angle have been reported previously. We present a series of seven (six of which have never been reported) cavernous hemangiomas limited primarily to the internal auditory canal. Whereas all cases but one had marked unilateral sensorineural hearing loss, only two had preoperative facial nerve dysfunction. There are no specific symptoms or physical or audiometric findings to differentiate cavernous hemangiomas from acoustic neuromas. However, a hemangioma should be suspected when facial paralysis is present in conjunction with a small intracanalicular tumor as demonstrated by computed tomography or magnetic resonance imaging. The former reveals calcium stippling in the lesions, whereas magnetic resonance imaging reveals a high signal intensity on both T1- and T2-weighted images.

Poststernotomy infections presenting as deep neck abscess.

A review of the world literature has failed to reveal any published reports of poststernotomy mediastinitis presenting as a deep neck infection. This article presents two such cases. Since the fascial layers of the mediastinum are a direct continuation of the cervical fascia, a number of potential pathways between the neck and mediastinum exist. Descending infection from the neck into the mediastinum is well documented. The reverse situation, an ascending infection from the mediastinum into the neck, is not described despite the potential natural pathways available for spread of infection. Our proposed mechanism for these two cases is that a surgically created pathway from the mediastinum to the lower neck allows for mediastinitis to point in the intercommunicating fascial spaces of the neck. Recognition of this clinical presentation will allow the surgeon to use prompt intervention for such a serious complication.

Perilymphatic fistula in children with preexisting sensorineural hearing loss.

Progression of preexisting sensorineural hearing loss (SNHL) in infants and children has been considered an indication for fistula exploration: is this approach warranted? On exploring 36 middle ears in 26 such children, we found a perilymphatic fistula (PLF) in four ears (11%). Although there was radiographic evidence of inner-ear deformity in one half of these children, a definite fistula was found in only four of 18 radiographically abnormal ears explored (22%). Even in the four patients with a history of an "event" that could implicate a fistula such as exertion or barotrauma, a fistula was found in only one. There are a number of possible causes for progression of a preexisting SNHL, and surgical exploration of the middle ear should not be recommended on the basis of progression alone. Instead, exploration for a suspected PLF should be strongly considered when there is also a history of an "event", and/or radiographic evidence of inner-ear abnormalities. Even under these conditions, one should be aware that fistulae are not likely to be found.

The effect of drug schedule on mouse neuroblastoma treated with cyclophosphamide.

The potential difference in survival due to scheduling of cyclophosphamide treatment was examined in A/J mice inoculated with neuroblastoma C1-1300 cells. Treatment was started on the third day post tumor implantation. There were four groups of animals each consisting of 30 mice: Group A, a control group, was injected with 0.3 cc of normal saline for a week. Group B was injected with cyclophosphamide 50 mg/kg/day every other day for five doses. This cycle was repeated in the same fashion starting on day 17. Group C was injected with cyclophosphamide 250 mg/kg on day 3 and this was repeated on day 17. Group D was injected with cyclophosphamide 50 mg/kg/day for five consecutive days. This cycle was repeated on day 17-21. The median survival for group B was 26.2 days (the same as the control group), for group C 47.7 days (87% increased survival) and for group D 63.2 days (143% increased survival with 30% possible cures). Twenty-three percent of the mice in group B died of pneumonia and another 23% had minor evidence of lung infection. Only three mice in this group had tumor at the time of death. The study suggests that the response of murine neuroblastoma as well as the development of pneumonia in A/J mice treated with cyclophosphamide might be schedule dependent.