The lived experience of the early postoperative period after colorectal cancer surgery.

Colorectal cancer is one of the most common cancer diagnoses and undergoing colorectal cancer surgery is reported to be associated with physical symptoms and psychological reactions. Social support is described as important during the postoperative period. The purpose of this paper was to describe how patients experience the early postoperative period after colorectal cancer surgery. Interviews according a phenomenological approach were performed with 13 adult participants, within 1 week after discharge from hospital. Data were collected from August 2006 to February 2007. Analysis of the interview transcripts was conducted according to Giorgi. The essence of the phenomenon was to regain control over ones body in the early postoperative period after colorectal cancer surgery. Lack of control, fear of wound and anastomosis rupture, insecurity according to complications was prominent findings. When caring for these patients it is a challenge to be sensitive, encourage and promote patients to express their feelings and needs. One possibility to empower the patients and give support could be a follow up phone call within a week after discharge.

Distal shunt obstruction in children with myelomeningocele after bladder perforation.

PURPOSE: We studied short-term complications and particularly the signs of shunt dysfunction after augmented bladder perforation in patients with myelomeningocele and ventriculoperitoneal shunts. MATERIALS AND METHODS: In our series of bladder augmentations in 27 patients with myelomeningocele and a ventriculoperitoneal shunt in the last 10 years (1994 to 2004) we noted 4 who were 8 to 16 years old at our institute with bladder perforation 2 to 5 years after augmentation. Three patients received a colonic augmentation and 1 received an ileal augmentation. One patient underwent surgery for small bowel obstruction 2 years after the primary operation, when a hole in the augmented bladder was identified and oversewn. The other 3 bladder perforations occurred spontaneously or after failure to catheterize. An additional patient with spontaneous perforation underwent auto-augmentation elsewhere. RESULTS: After primary open abdominal surgery and enterocystoplasty there was no sign of shunt dysfunction in any patient. Bladder perforation and leakage of free urine into the abdominal cavity occurred in 4 of the 5 patients. In those patients severe symptoms of shunt dysfunction, including headache and high intracranial pressure, were noted 2 to 7 days after perforation. In patient 1 there was only urine leakage into a small cavity close to the bladder and no acute signs of post-perforation shunt dysfunction. In all cases the shunt was externalized for 1 to 6 weeks without further complications. CONCLUSIONS: In patients with myelodysplasia who have bladder perforation and free urine in the abdominal cavity the peritoneum is chemically inflamed by urine. Resorption of cerebral liquor may be disturbed, leading to shunt dysfunction and high intracranial pressure. Therefore, it is important for the urologist to recognize and evaluate postoperative signs and symptoms of increased intracerebral pressure in patients with bladder perforation. If found, early computerized tomography of the brain is recommended.

Estrogen and hearing: a summary of recent investigations.

Is the female sex steroid estrogen the key to preserved hearing in the aging human? This question remains unanswered, but hearing loss is more profound in elderly males than females. There are also well-known sex differences in the auditory brainstem response (ABR), i.e. women have shorter latencies than men. Moreover, menopausal women who are administered hormone replacement therapy have slightly better hearing than those who are not, and women with Turner's syndrome (45,X), who are biologically estrogen-deficient, show longer ABR latencies and early presbyacusis. These findings are also supported by animal experiments. When boosted with estrogen or testosterone the non-reproductive female midshipman fish alters its inner ear auditory mechanism so that it can hear the male's hum-like call. If estrogen receptor beta is knocked out in mice, severe progressive hearing loss occurs, leading to early deafness. In apparent contradiction to these findings, there have been case reports suggesting that hormone replacement therapy and oral contraceptive use can lead to hearing loss, but of another type, namely acute sudden deafness. Such contradictory aspects of the action of estrogen are commonly found and may spring from the fact that there are two estrogen receptors, alpha and beta, both of which are present in the inner ear of mice, rats and humans. Knowing how sex steroids can alter hearing ability may give important clues as to how estrogen can preserve hearing in humans. In this review we present a summary of current knowledge about hearing and estrogen.

Urethral injection for stress urinary incontinence: long-term results with dextranomer/hyaluronic acid copolymer.

Urethral injection is a convenient, minimally invasive means of treating stress urinary incontinence (SUI). We present long-term follow-up data from 20 patients originally recruited in 1994-95 (mean age 67 years) to receive urethral injection with dextranomer/hyaluronic acid (Dx/HA) copolymer. The majority of patients had failed previous therapy for SUI. If the first injection was unsuccessful, up to two further injections were offered. Only 3 patients (15%) failed to show a response to treatment. Sixteen women were reassessed during 2001 (4 had died of causes unrelated to the study treatment). A sustained response throughout the follow-up period was reported in 9/16 patients (57%), with incontinence recurring in just 4 (25%). None of the 7 patients with persistent or recurrent incontinence were cured by subsequent treatments, including surgery. In conclusion, urethral injection with Dx/HA copolymer offers promising long-term efficacy in the treatment of SUI, regardless of old age or failure to respond to previous therapy.

Effect of estrogen and antiestrogens on the estrogen receptor content in the cochlea of ovariectomized rats.

Older women in the normal population tend to develop less severe hearing loss as compared to males in the same age. In Turner syndrome (45,X), estrogen deficiency is one of the predominant problems. Ear and hearing problems are common among these patients. Does estrogen have an impact on the hearing organ? Twenty-four rats were ovariectomized and treated with vehicle (controls), estradiol or selective estrogen receptor modulators such as tamoxifen and ICI182780, in order to study the effects on the estrogen receptor levels and distribution in the inner ear. The cochleas were stained immunohistochemically using antibodies against estrogen receptor alpha and beta. No major difference in estrogen receptor content in the cochleas was observed among groups. There was however a potential down regulation of estrogen receptor alpha in the marginal cells of stria vascularis in the rats that were substituted with ICI182780 (pure antiestrogen) as compared to those given estradiol or tamoxifen. When investigating the tissues with light microscopy no change in inner ear anatomy could be observed.

Endoscopic treatment with dextranomer-hyaluronic acid for vesicoureteral reflux: histological findings.

PURPOSE: Dextranomer-hyaluronic acid co-polymer is the first endoscopic bulking agent approved for vesicoureteral reflux in the United States. We evaluated the histopathological changes associated with this treatment in children with vesicoureteral reflux. MATERIALS AND METHODS: Children 1 to 11 years old in whom treatment with dextranomer-hyaluronic acid co-polymer for grades III or greater vesicoureteral reflux had failed were eligible for the study. Failure was defined as persistent vesicoureteral reflux on voiding cystourethrography done approximately 3 months after implantation. At ureteral reimplantation the implant and surrounding ureteral tissue were resected and fixed for histopathological analysis. Tissue sections (4 to 5 microm.) were stained for routine histology and examined under a light microscope. Patients with a similar grade of vesicoureteral reflux who had not undergone endoscopic treatment served as the control group. RESULTS: The study population comprised 23 patients with vesicoureteral reflux, of whom 13 with a mean age of 2 years 8 months at diagnosis underwent 1 to 3 treatments with dextranomer-hyaluronic acid co-polymer. The remaining 10 patients with a mean age of 1 year 10 months at diagnosis did not receive the bulking agent before ureteral reimplantation. The implant remained in situ 13 to 39 months (mean 22). On ureteral reimplantation the implant was located at the site of injection in 12 of the 13 patients. Histologically a granulomatous inflammatory reaction indicated by giant cell infiltration was observed at the implantation site. At ureteral reimplantation 9 implants were pseudo-encapsulated. Calcification was present in 9 ureters, while the eosinophil count was greater than 5 cells per 0.125 mm2 in 7 ureters treated with dextranomer-hyaluronic acid co-polymer. Mast cell infiltration was similar in the treatment and control groups. CONCLUSIONS: Endoscopic treatment with dextranomer-hyaluronic acid co-polymer for vesicoureteral reflux is associated with a granulomatous reaction of the giant cell type, inflammatory cell infiltration and implant pseudo-encapsulation. They are typical histological findings associated with implantation of a foreign material. Dextranomer-hyaluronic acid co-polymer remains safe and effective for vesicoureteral reflux in children.

Vasopressin and hypercalciuria in enuresis: a reappraisal.

OBJECTIVE: To test the hypotheses that vasopressin deficiency or hypercalciuria are important in polyuric and non-polyuric bedwetting, as nocturnal polyuria is a pathogenetic factor in enuresis responsive to antidiuretic therapy with desmopressin. SUBJECTS AND METHODS: Vasopressin deficiency has been implicated as a cause of nocturnal polyuria, but measurements of vasopressin in plasma have given contradictory results, because the hormone is released in pulses. Urinary levels reflect the secretion over longer periods. Hypercalciuria has also been proposed as a pathogenetic factor. Twenty-eight enuretic children who responded to desmopressin therapy with or without added anticholinergic agents (diuresis-dependent enuresis, DE), 15 children with therapy-resistant enuresis (not diuresis-dependent, NDE) and 51 continent controls were assessed. Urinary vasopressin, calcium and osmolality were measured in the morning after a 12-h thirst provocation. Urine production was recorded for 2 days. RESULTS: Because most data were not normally distributed, the values are expressed as the median (range). There were no differences in urine osmolality; i.e. con-trols 919 (636-1232), DE 849 (462-1149), NDE 968 (664-1191) mOsml/kg); vasopressin, controls 34 (8-983), DE 26 (9-295), NDE 50 (9-116) pmol/L; or calcium excretion (expressed as the calcium/creatinine ratio), controls 0.16 (0.01-0.71), DE 0.14 (0.04-0.67), and NDE 0.23 (0.03-0.69). The DE group produced more urine, at 18.4 (9.2-52.5) mL/kg/day, than the other groups, i.e. control 12.7 (8.3-42.8) and NDE 12.1 (6.3-36.8) mL/kg/day (P = 0.008). CONCLUSION: All enuretic children with nocturnal polyuria do not have vasopressin deficiency. The urinary calcium excretion does not differ between enuretic and dry children.

The effect of endoscopic injections of dextranomer based implants on continence and bladder capacity: a prospective study of 31 patients.

PURPOSE: A prospective study was conducted to assess the efficacy of dextranomer based implants as a new bulking agent for endoscopic treatment of pediatric structural incontinence. MATERIALS AND METHODS: A total of 33 children and adolescents 5 to 18 years old with severe incontinence due to sphincteric incompetence (exstrophy-epispadias in 13, neuropathic bladder in 16, bilateral ectopic ureters in 4) were enrolled in the study. All but 1 patient wore diapers. Preoperative evaluation consisted of medical history, pad test, urine culture, urinary tract ultrasound and videourodynamics. This evaluation was repeated 6 months and 1 year after treatment and then on a yearly basis. Of the patients 14 had 2 and 1 had 3 treatment sessions to achieve a definitive result. At each evaluation the patient was considered cured-dryness interval of 4 hours confirmed by pad test, significantly improved-minimal incontinence requiring no more than 1 pad a day with less than 10 gm. leakage during pad test; and no further treatment required, and treatment failure-no significant improvement. Videourodynamics were mainly useful to study the evolution of the bladder capacity, activity and compliance. Followup after the last injection ranged from 6 to 36 months (mean 18). RESULTS: The mean injected volume was 3.9 ml. (range 1.6 to 12) and the procedure lasted a mean of 30 minutes (10 to 60). In the postoperative period 2 patients had temporary dysuria and 10 had a nonfebrile urinary tract infection. At 1 month 24 of the 33 patients (73%) were dry or improved. Two patients were subsequently excluded from study for noncompliance with followup. At 6 months 17 of 31 patients (55%), at 1 year 13 of 28 (46%), at 2 years 10 of 23 (43%) and at 3 years 10 of 20 (50%) were dry or improved. Similar success occurred in cases of neuropathic bladder (57%) and exstrophy-epispadias complex (46%). Success rate of re-treated patients was 35%. Of 13 patients with a normal initial bladder capacity 3 had a bladder decompensation requiring augmentation after 6 months. Also an increase of at least 50% in capacity was observed in 12 of 18 patients with an initial small bladder. No side effect related to the substance was observed. CONCLUSIONS: Endoscopic treatment of pediatric structural urinary incontinence with dextranomer implant, a nontoxic, nonimmunogenic, nonmigrant synthétic substance, was effective after 3 years in half of our patients. It may also be beneficial for patients with small bladder functional capacity. As the success rate decreased during the first year of followup, the result observed at 1 year seems to remain stable subsequently.

Estrogen receptors alpha and beta in the inner ear of the 'Turner mouse' and an estrogen receptor beta knockout mouse.

Estrogen receptors have earlier been shown in the normal mouse, rat and human inner ear. If estrogens are important in normal hearing and development of presbyacusis in the normal population is not known. However it is known that patients with Turner syndrome, where a lack of estrogens is one of the main characteristics, commonly develop an early presbyacusis. A 'Turner mouse' has been developed, as a model for the ear problems in Turner syndrome, and it shows otitis media and a premature aging of the hearing. Estrogen receptors exist in an alpha and a beta form. In this study inner ear tissue, from the Turner mouse and an estrogen receptor beta knockout mouse (betaERKO), was investigated regarding estrogen receptor alpha and beta using immunohistochemistry. Results show that the Turner mouse has the same pattern of inner ear labeling, both concerning the estrogen receptor alpha and beta, as that of a normal CBA/Ca mouse, with positive staining in the organ of Corti and spiral ganglion. The betaERKO mice show close to normal inner ear morphology and positive estrogen receptor alpha immunostaining at the same locations as the CBA/Ca mouse.

Suicide mortality among electricians in the Swedish construction industry.

OBJECTIVES: To investigate the risk of suicide in Swedish electricians employed in the construction industry. A few studies have indicated an increased risk of suicide for electricians in the construction industry and electricians exposed to electromagnetic fields. METHODS: This is a cohort study. Electricians were identified through a computerised register of construction workers who had participated in health examinations in 1971-92. In this register, 33,719 male electricians were identified together with a reference group consisting of 72,653 male glass or woodworkers. Through a linkage with the Swedish Death Register, the cause of death was identified to the end of 1997. Mortality as a result of suicide was also compared with the general population with adjustments for sex, age, and period. RESULTS: The risk of mortality from suicide was decreased for electricians (standardised mortality ratio (SMR) 0.58, 95% confidence interval (95% CI) 0.47 to 0.71) and for the reference group of construction workers (SMR 0.81, 95% CI 0.72 to 0.91) compared with the general population. CONCLUSION: Contrary to some other studies, risk of suicide was not increased among electricians in the construction industry.

Pressure flow measurement of hydronephrosis in children: a new approach to definition and quantification of obstruction.

PURPOSE: Hydronephrosis due to obstruction of the ureteropelvic junction is not uncommon in children. The critical level of outflow resistance characterizing obstruction remains to be established. It was previously found in our laboratory that the outflow resistance could be calculated in an animal model by servoregulating the infused flow to preset pressure levels. We determine whether this procedure is also feasible in patients with hydronephrosis. MATERIALS AND METHODS: A total of 46 patients with 48 hydronephrotic kidneys were examined. There were 32 boys and 14 girls with a diagnosis of hydronephrosis and suspected ureteropelvic junction obstruction who underwent a pressure flow study under general anesthesia for evaluation before possible surgery. The kidney was punctured percutaneously under ultrasonic guidance, and the study was performed under fluoroscopy. Flow levels were measured when steady state flow was achieved at pressures 5, 10, 15, 20, 25 and 30 mm. Hg greater than the intra-abdominal pressure. A conventional Whitaker test at an infusion rate of 10 ml. per minute was performed for comparison. The patient then underwent pyeloplasty if the kidney was considered obstructed. RESULTS: There were 2 patients excluded from evaluation because of significant leakage of contrast medium. Stable and repeatable recordings were obtained in all pressure flow studies but only in 7 of 46 Whitaker tests. A total of 41 kidneys were considered obstructed at pressure flow studies and operated on. The resistance was pressure dependent. Thus, mean resistance in the previous range 5 to 15 mm. Hg was considered a good overall measure of outflow resistance. Mean resistance less than 0.75 was normal and greater than 1.25 obstruction. There were 7 patients who had crossing vessels and a high mean resistance (3 or greater). CONCLUSIONS: The Whitaker test proved to be of no value for calculating the outflow resistance in ureteropelvic junction obstruction. The pressure flow study is superior to the Whitaker test for determining the outflow resistance and also allowing categorization of ureteropelvic junction obstruction.

Long-term followup of children treated with dextranomer/hyaluronic acid copolymer for vesicoureteral reflux.

PURPOSE: Dextranomer/hyaluronic acid copolymer is a novel substance that has favorable properties for endoscopic treatment of vesicoureteral reflux. We assess the long-term efficacy and safety of this treatment of children. MATERIALS AND METHODS: Children 1 to 15 years old with grade III or greater vesicoureteral reflux were eligible for enrollment in our study. All patients received endoscopic treatment with dextranomer/hyaluronic acid copolymer and were scheduled to have a voiding cystourethrogram 3 and 12 months after implantation. Children with reflux grade III or greater after treatment received up to 2 more implantations, and those with persistent reflux were referred for open surgery. In some cases long-term clinical followup was accompanied by a late voiding cystourethrogram. RESULTS: A total of 228 patients received endoscopic treatment. The efficacy population was comprised of 221 children, including 67 who received 2 and 8 who received 3 implantations. Endoscopic treatment was performed without complications in all cases. Patients were followed clinically for 2 to 7.5 years (mean 5). On the last voiding cystourethrogram 68% of patients had a positive response (grade I or less) and 81% had no dilating reflux. The corresponding results for treated ureters were 75% and 85%, respectively. Only 27 (12%) patients were referred for open surgery. A late voiding cystourethrogram was performed in 49 patients 2 to 5 years after treatment. Of the ureters free of reflux (grade 0) 3 to 12 months after treatment 96% remained free of dilating reflux. Adverse events occurred in association with implantation in only 2% of patients, although urinary tract infection subsequently developed in 8%. CONCLUSIONS: Endoscopic treatment with dextranomer/hyaluronic acid copolymer was effective and well tolerated in children with vesicoureteral reflux. Long-term followup indicated that there was no deterioration in patients responding positively to treatment.

Estrogen receptors in the normal adult and developing human inner ear and in Turner's syndrome.

The influence of estrogens, the female sex hormone, on the ear and hearing is yet not fully investigated, though some studies have suggested that estrogens may influence hearing functions. The presence of estrogen receptors alpha and beta has earlier been shown in the inner ear of mice and rats. The aim of this study was to map possible estrogen receptors in the human inner ear. Inner ear tissue from human adults, aborted human normal fetuses and fetuses with Turner's syndrome were collected. Paraffin embedded sections of adult and fetal inner ears were immunostained with antibodies against estrogen receptors alpha and beta. Estrogen receptor alpha containing cells were found in the adult human inner ear only in the spiral ganglion, and estrogen receptor beta in the stria vascularis solely. The human fetal inner ear tissue from both normal and Turner fetuses showed a very weak staining of estrogen receptor alpha in the spiral ganglion cells, but no specific labeling of the Kölliker's organ of Corti at 13, 14 and 18 weeks of age. No staining of estrogen receptor beta was seen in the fetal inner ear.

Effect of thromboxane and nitric oxide blockade on renal blood flow increase during volume expansion in hydronephrotic rats.

OBJECTIVE: It has recently been found that hydronephrotic rats, despite low diuresis, show a significant increase in renal blood flow (RBF) during volume expansion. The present experiments were designed to evaluate the mechanisms underlying this phenomenon. MATERIAL AND METHODS: Three-week-old Sprague-Dawley rats underwent partial obstruction of the left ureter using the Ulm-Miller psoas groove technique. The effects on RBF were studied 3 weeks later under general anesthesia using real-time ultrasound flowmetry, first during normohydration and then during extracellular volume expansion, in both untreated animals, and after prior blockade of either thromboxane or nitric oxide. RESULTS: Significant hydronephrosis developed in all cases. RBF was normal under control conditions. During volume expansion RBF increased significantly in untreated experimental animals (mean 7.5%). In contrast to this finding, RBF remained unchanged during volume expansion in both the thromboxane and nitric oxide blockade groups. CONCLUSION: It is concluded that a thromboxane- and/or nitric oxide-dependent RBF redistribution takes place in hydronephrotic kidneys during volume expansion.

Renal blood flow increase during volume expansion in hydronephrotic rats.

PURPOSE: We assessed the renal blood flow pattern in experimental hydronephrosis during normal hydration and extracellular volume expansion. MATERIALS AND METHODS: Partial obstruction of the left ureter was created in 3-week-old Sprague-Dawley rats by embedding the ureter in a psoas muscle groove. Moderate hydronephrosis without kidney weight reduction developed in all cases. The effects on renal hemodynamics were studied with real-time ultrasound flowmetry 3 weeks later during normal hydration and then during volume expansion. The degree of hydronephrosis was classified as mild, moderate or severe. RESULTS: Under baseline conditions renal blood flow was normal in mild and moderate hydronephrosis but low in severe hydronephrosis. During volume expansion renal blood flow increased significantly in all experimental animals (mean 14%) compared to that in controls, which remained unaffected or decreased (mean -3%). The flow increase was related to the degree of dilatation, which was 2% in mild, 13% in moderate and 44% in severe hydronephrosis when the groups were considered separately. CONCLUSIONS: A significant increase in renal blood flow proportional to the degree of hydronephrosis occurred as a result of volume expansion. This finding may be explained by a state of vasodilatation combined with a reduction in the filtration coefficient.

Bladder capacity and renal concentrating ability in enuresis: pathogenic implications.

PURPOSE: We compared bladder volume and renal concentrating capacity in dry children and 2 distinct groups of children with enuresis to hypothesize about the pathogenesis of various types of enuresis. MATERIALS AND METHODS: A total of 55 dry children and 100 with enuresis underwent an overnight thirst provocation test to assess renal concentrating capacity and completed a 2-day voiding chart to assess functional bladder capacity. The enuretic children were subdivided into 27 desmopressin responders and 73 desmopressin nonresponders before study inclusion. RESULTS: The desmopressin responder group had lower average renal concentrating capacity +/-1 standard deviation than dry children and desmopressin responders (856 +/- 158 mOsm./kg. versus 939 +/- 147 and 962 +/- 151, respectively, p <0.05). Analogously average daytime urine production in the desmopressin responder group was greater than in dry children and desmopressin responders (22.2 +/- 10.2 ml./kg. body weight versus 15.4 +/- 7.3 and 15.3 +/- 7.2, respectively, p <0.01). Average functional bladder capacity expected for age was less in desmopressin nonresponders than in dry children and responders (52.2% +/- 19.9% versus 79.2% +/- 30.4% and 69.5% +/- 25.7%, respectively, p <0.001). CONCLUSIONS: Desmopressin responders produced larger amounts of less concentrated urine than the other children, while desmopressin nonresponders had smaller bladder capacity than the other groups. These results support the idea that enuretic children who respond favorably to desmopressin treatment have polyuria, whereas children with therapy resistant enuresis have detrusor hyperactivity.

The overactive bladder in children: a potential future indication for tolterodine.

OBJECTIVE: To determine the safety, efficacy and pharmacokinetics of tolterodine in children with an overactive bladder. PATIENTS AND METHODS: Thirty-three children (20 boys and 13 girls, aged 5-10 years) with an overactive bladder and symptoms of urgency, frequency and/or urge incontinence were enrolled in an open, dose-escalation study. Patients were treated with oral tolterodine 0.5 mg (n = 11), 1 mg (n = 10) or 2 mg (n = 12) twice daily for 14 days. The primary safety endpoint was the change in residual urinary volume, as determined by ultrasonography. In addition, voiding diary variables (frequency and incontinence episodes) and pharmacokinetics were evaluated. Other safety endpoints included laboratory variables, electrocardiogram recordings and reported adverse events. RESULTS: There were no safety concerns in terms of the change in residual urinary volume for any of the three dosage groups; values were comparable with baseline after 2 weeks of treatment for all three dosages. Adverse events were reported by 20 patients (six on 0.5 mg, five on 1 mg, and nine on 2 mg). Most adverse events were not considered to be drug-related; of the 13 possibly related events, 10 occurred in those taking 2 mg. Headache was the most commonly reported adverse event. No serious adverse events were reported and there were no general safety concerns. There was an improvement in voiding diary variables in all treatment groups after 2 weeks of treatment, although the efficacy was greatest in those taking 1 mg and 2 mg. Pharmacokinetic findings were consistent with dose linearity over the range 0.5-2 mg. CONCLUSION: The results support the use of 1 mg twice daily as the optimal dose of tolterodine for treating children aged 5-10 years with an overactive bladder.

Characterization of hearing in an X,0 'Turner mouse'.

Turner's syndrome is due to total (45,X) or partial (mosaicism) loss of one X-chromosome. The main features are short stature, ovarian dysgenesis with no estrogen production and infertility. In addition to ear and hearing disorders, middle ear problems including acute/serous otitis media and chronic middle ear disease are frequent. Sensorineural hearing loss is often seen with a dip in the mid-frequencies and also an early high frequency loss. In this study, middle-and inner-ear pathology was characterized using physiological and morphological techniques in a 'Turner mouse' that has been generated with the chromosomal aberration X,0. Otitis media was found in some of these X,0 animals, a symptom that is seldom found in control animals. The auditory brainstem responses (ABR) of the Turner mouse showed a progressive hearing loss in the high frequency region that exceeded the normal age-related hearing loss of control mice and increased latencies of the first ABR wave. Outer hair cell loss was apparent in the cochlear basal turn of Turner mice. Decreases in the amplitude of distortion product otoacoustic emissions were correlated with the loss of ABR threshold sensitivity. These results indicate that hearing problems in the Turner mouse seems to be of cochlear origin with an eighth nerve component. This Turner mouse model appears to have ear and hearing problems quite similar to humans and can therefore be used as a model to determine the auditory pathology underlying this syndrome.