Conservative management of endolymphatic sac tumors in von Hippel–Lindau disease: case report

In endolymphatic sac tumors associated with von Hippel–Lindau disease, early detection and surgery have been warranted to avoid associated neurological morbidity. However, in lately discovered tumors, hearing preserving surgery is often impossible and timing of surgical resection is difficult to define. We report two cases of tumors revealed by a sudden and profound hearing loss and managed conservatively for more than 15 years without worsening of the neurological symptoms associated with the endolymphatic sac tumor. Tumor size remained stable for the first patient and a stuttering growth pattern was observed for the second patient. Initial observation may be considered a not unreasonable management paradigm in these cases.     

Endolymphatic mastoid shunt versus endolymphatic sac decompression for ménière’s disease

Objectives: This study compares the efficacy of endolymphatic mastoid shunt (EMS) versus endolymphatic sac decompression (ESD) without sac incision.Methods: The AAO-HNS Guidelines for the Diagnosis and Evaluation of Therapy in Ménière’s disease were used to identify suitable candidates for the study. All patients who failed medical management and underwent either EMS (n = 91) or ESD (n = 107) were selected for review. Data were collected and analyzed using the AAO-HNS guidelines. A questionnaire was used to assess the severity of vertigo before and after the procedure. The class of therapeutic effect for each procedure and functional level for each patient prior to and after treatment was determined. Audiograms obtained prior to surgery and 18–24 months postoperatively were compared to determine the influence of the procedure on hearing.Results: EMS and ESD were equally effective in reducing the incidence and severity of vertigo attacks with improvement in 76% and 75% of patients, respectively. Complications from the procedures were minimal.Conclusions: Both EMS and ESD are effective, nondestructive alternatives for patients who have failed medical management of Ménière’s disease. These results provide insight into the physiologic basis for the efficacy of endolymphatic sac procedures.     

Extreme sensitivity of hearing to decreases of ICP in Menière’s disease

We report the case of a Menière’s disease patient affected by normal pressure hydrocephalus (NPH) who presented a cerebrospinal fluid (CSF) pressure-dependent hearing impairment after shunting. This side-effect was not only reversible and reproducible but occurred at a high opening pressure when the valve setting was lowered by only 0.7 mmHg (10 mmH₂O). This observation suggests that hearing in Menière’s disease might be very sensitive to small reductions of intracranial pressure (ICP) and that these patients should be informed of this potential risk, which can compromise the efficacy of the shunt.     

What is the role of decorin in diabetic kidney disease?

The small proteoglycan decorin may intercept the activity of the TGF-beta system. Decorin administration has been advocated as potential therapy in renal fibrotic diseases, because of the findings of a relative deficiency of decorin and a relative excess of TGF-beta in acute glomerulonephritis. Does a similar situation pertain in diabetic kidney disease? Activation of TGF-beta seems to be crucial to tissue injury in diabetic nephropathy, but until recently it has not been established whether decorin plays any role in the manifestations of this disease. We review evidence that a surfeit rather than a deficit in decorin expression exists in diabetic renal disease, and that there exists a negative feed-back loop whereby TGF-beta1 induces down-regulation of decorin expression. Rat and mouse mesangial cells as well as mouse proximal tubular cells in culture exhibit increased decorin mRNA levels in high ambient glucose. Decorin mRNA level in the kidney of streptozotocin-induced diabetes in mice is rapidly and significantly increased following the induction of diabetes. Thus, the available evidence suggests that renal decorin is not deficient in this disorder and hence decorin supplementation does not seem to be warranted. Rather, interception of the effects of TGF-beta seems to be an approach most likely to yield beneficial results in diabetic nephropathy.     

Selective vestibular neurotomy for Meniére's disease: a neurosurgical procedure?

The objective of this paper is to report the authors' experience with a 1.5-cm suboccipital craniotomy for selective vestibular neurotomy. Data were collected on 19 consecutive patients undergoing selective vestibular neurotomy for Meniére's disease, between June 1988 and January 2004, performed by a neurosurgeon. There were 10 males and nine females. Ages ranged from 35 to 69 years (median 55). All patients had preoperative imaging, audiometric and caloric assessments. Patients were assessed postoperatively for residual vertigo and surgical complications. All patients had repeat audiometric and caloric evaluation postoperatively. Ten patients had excellent (mild residual deafness, no vertigo) and nine had good (no vertigo attacks, mild postural dizziness) results. Hearing was preserved at preoperative levels in 18 patients and improved in one patient. There were two CSF leaks--one settled with lumbar drainage and the other required wound re-exploration. Two patients developed transient facial weakness. There were no deaths. Selective vestibular neurotomy can be performed through a 1.5-cm suboccipital craniotomy without the need for an endoscope. It is a simple, safe and highly successful procedure for controlling vertigo attacks in intractable Meniére's disease. This procedure preserves hearing and should be considered for those refractory to medical treatments.     

Biochemistry of implantation in the human

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What is the biopsychosocial role of human immunodeficiency virus positivity in patients with end-stage liver disease who undergo orthotopic liver transplantation?

INTRODUCTION: Since 2003 the National Research Program for Solid Organ Transplantation in patients with human immunodeficiency virus (HIV) is active at our liver transplantation center. Patients with HIV who enter this protocol are assessed by the Consultation Liaison Psychiatry Service. The aim of the present study was to evaluate their psychiatric comorbidity. METHODS: An observational prospective study was conducted comparing end-stage liver disease (ESLD) patients with and without HIV. After the assessment, the psychiatrist compiled the Transplant Evaluation Rating Scale (TERS) and the Montgomery Asberg Depression Rating Scale (MADRS). Baseline evaluation was made before inclusion on the OLT waiting list and the follow-up evaluation was made 12 months later. RESULTS: From January 2003 to December 2006 we assessed 553 patients: 39 (6%) with HIV and 361 (94%) without HIV. The 2 groups were homogeneous for gender (75% of male patients; P=not significant [NS]) but not for age (46+/-5 vs 56+/-9; P=NS). Psychiatric history was negative in 176 (49%) patients without HIV and in 6 (15%) patients with HIV (P< .001). At baseline psychiatric comorbidity was present in 33 HIV patients (85%) and in 148 non-HIV patients (41%; P< .001). At follow-up MADRS highlighted an improvement in all of the items for HIV patients. In the non-HIV group, the variation was as follows: baseline, 7.10; follow-up, 8.15. In the HIV group, the variation was as follows: baseline, 10.20; follow-up, 4.09 (P< .001). The average score at TERS was higher among patients with HIV (43+/-9 vs 35+/-9; P=NS). CONCLUSIONS: At baseline HIV patients with ESLD showed a higher rate of psychopathology, but they improved at follow-up; the contrary happened in the non-HIV group.     

Cardioprotective role of statins in chronic kidney disease: do we have the answer?

The observational study by Szummer et al. shows that patients with advanced chronic kidney disease (CKD) are treated less with statins after myocardial infarction, even though statins benefit survival in CKD classes 1-4. The study's limitations are obvious, but such a population may be more representative. The results indicate that statins should be used more frequently after myocardial infarction in CKD classes lower than 5, a conclusion supported by the recently presented Study of Heart and Renal Protection (SHARP).