Cartilage "shield" tympanoplasty: an effective and practical technique.

The aim of the present study is to report our results with cartilage "shield" tympanoplasty (CST) for total tympanic membrane perforations. A retrospective chart review of patients undergoing CST was performed in tertiary referral centers. One hundred and seventy-seven patients who had undergone CST were identified. Patients' age ranged from 7 to 74 (mean 35) years. The mean postoperative follow-up was 23 (range 6-73) months. The main outcome measures were Graft take, pre and postsurgery audiologic evaluation. Graft take was 97.2%. Three patients developed recurrent cholesteatomas, one an anterior pinhole perforation, and one a minimal graft anterior lateralization. The average pre and postoperative pure-tone air-bone gaps (PTA-ABG) were 32.06 ± 11.21 and 18.69 ± 10.53 dB, respectively. The overall average ABG improvement was 13.37 ± 9.34 dB. An overall postoperative ABG of 25 dB or less was achieved in 140 of the 177 patients (79.1%). Hearing improvement was statistically significant in all cases (p < 0.0001). Cartilage shield tympanoplasty is a very reliable procedure. Graft take is excellent and hearing improvement is significant.

Gender, age and location-related factors in academic otolaryngology - the Greek paradigm.

OBJECTIVE: To map Greek academic otolaryngologists, and assess gender, age and location-related differences in their rank and academic productivity. METHODS: A pre-established database of local and diaspora scientists was used, after adjustment and updating for otolaryngology. The following data were recorded: age, gender, academic rank, country of work, total citations and h-index of December 2022. RESULTS: A total of 276 Greek academic otolaryngologists were identified in the Scopus database. Of Greek otolaryngologists, 15.9 per cent are women. Of all academic otolaryngologists, 27.1 per cent have a university post, but only 4 per cent of them are women. There is an almost linear correlation between university post ranking and citations. Otolaryngologists based in Greece accounted for 3 out of the 10 most cited Greek otolaryngologists. CONCLUSION: There are significant age, gender and location-related differences in academic output. The representation of women and the full use of their potential in medicine require proactive measures, to lift the burdens limiting their participation.

Clinical Predictors of Pulsatile Tinnitus in Patients With Idiopathic Intracranial Hypertension: An Age-Matched Cohort Study.

INTRODUCTION: Pulsatile tinnitus (PT) occurs in many but not all patients with idiopathic intracranial hypertension (IIH). It is poorly understood why some patients with IIH develop PT, yet others do not. The purpose of this study was to determine if any clinical findings differ between those with and without PT in IIH, potentially shedding light on a pathophysiologic mechanism. METHODS: Age-matched cohort analysis of patients with documented IIH and presence or absence of PT was performed, collecting data including body mass index (BMI), blood pressure, visual acuity, cerebrospinal fluid (CSF) opening pressure, sleep apnea, migraines, and transient visual obscurations, among others. Independent-sample t test and χ2 test were used to analyze continuous and binary variables, respectively, with multivariate analysis conducted including variables statistically significant on univariate analysis. RESULTS: Eighty subjects with IIH met the inclusion criteria (40 PT+, 40 PT-). CSF opening pressure showed no significant difference between the two groups. The PT+ cohort was found to have an average BMI of 45.1 kg/m 2 , which was significantly higher than the PT- group (37.7 kg/m 2 ; p = 0.0023). PT+ pulse pressure (60.1 mm Hg) was also significantly higher than the PT- group (51.6 mm Hg; p = 0.019). PT+ patients were also significantly more likely to have sleep apnea ( p < 0.001) and migraines ( p = 0.0036). Multiple logistic regression revealed an adjusted odds ratio of 13.9 for sleep apnea, 4.1 for migraines, and 1.01 for every increase in unit of BMI. CONCLUSION: Among patients with IIH, presence of PT is associated with higher BMI and pulse pressure, and increased incidence of sleep apnea and migraines. Given no significant difference in CSF pressures between the two groups, PT may not be a product of increased disease severity but may be related to sequelae of obesity, such as increased pulse pressure and sleep apnea.

Doxycycline-Induced Intracranial Hypertension Presenting as Unilateral Pulsatile Tinnitus.

Background: Pulsatile tinnitus (PT) is increasingly recognized as a cardinal symptom of idiopathic intracranial hypertension (IIH). However, clinicians should remain aware of other causes of nonidiopathic or secondary intracranial hypertension manifesting as PT. We present 2 patients with isolated PT (without accompanying headache, blurred vision, and papilledema) thought to be secondary to tetracycline-induced intracranial hypertension. To our knowledge, these are the first cases of PT as the presenting symptom of this condition. Cases: A 41-year-old female (body mass index [BMI] 29 kg/m2) with ocular rosacea was initially treated with minocycline. Shortly after transitioning to oral doxycycline and erythromycin eye ointment, she noted left-sided PT. Her PT resolved after discontinuing doxycycline. In a second case, a 39-year-old female (BMI 19 kg/m2) with acne presented with a three-year history of left-sided PT while on long-term oral doxycycline for many years. She denied visual or auditory changes and atypical headaches. MRI findings were concerning for intracranial hypertension. Three months later, the patient was seen by neuro-ophthalmology, with findings suggesting prior papilledema. The patient reported PT improvement after discontinuing doxycycline. Conclusions: This case series highlights 2 cases of isolated PT as the sole symptom of intracranial hypertension that resolved with tetracycline cessation. The presentation and unexpected improvement following tetracycline discontinuation are atypical compared with previous reports of tetracycline-induced intracranial hypertension. Clinicians should maintain a high index of suspicion for all types of intracranial hypertension (idiopathic and secondary), even in patients with a lower BMI. Current and prior medications should be reviewed when considering the etiology of intracranial hypertension.

Magnetic Resonance Imaging Findings in Idiopathic Intracranial Hypertension With and Without Pulsatile Tinnitus: An Age-Matched Cohort Study.

OBJECTIVE: Many but not all patients with idiopathic intracranial hypertension (IIH) have pulsatile tinnitus (PT). However, little is known about why some patients with IIH develop PT and others do not. The purpose of this study was to determine if any of the classic magnetic resonance imaging (MRI)-detectable markers of IIH differ between patients with and without PT, thereby shedding light on potential pathophysiology. METHODS: A retrospective age-matched cohort study of patients with documented IIH (diagnosed by neuro-ophthalmologist) was performed. All patients had MRI performed around the time of diagnosis. MRIs were assessed for 16 variables known to be associated with IIH (e.g., pituitary displacement/empty sella, optic nerve tortuosity, transverse sinus stenosis, inferior cerebellar tonsils, arachnoid granulations, slit-like ventricles) by two blinded neuroradiologists. All binary variables were analyzed via χ2 test with Yates correction, or Fisher exact when appropriate. Continuous variables were analyzed via Student t test. Inter-rater reliability for binary variables was assessed by Cohen κ . For continuous variables, intraclass correlation coefficient was calculated. RESULTS: Forty age-matched patients with IIH met the inclusion criteria (20 with PT, 20 without PT). For all known binary MRI findings associated with IIH, there were no statistically significant differences between groups. Likewise, there were no statistically significant differences for continuous variables. CONCLUSIONS: The classic MRI findings associated with IIH do not differ between patients with and without PT, suggesting that systemic (rather than localized intrinsic or extrinsic) factors may play a critical role in the pathophysiology.

Cartilage tympanoplasty in smokers.

OBJECTIVES: We compared the outcomes of cartilage tympanoplasty in nonsmokers and smokers. METHODS: We performed a retrospective chart review of patients who underwent cartilage tympanoplasty in a tertiary academic medical center from 1991 to 2010. There were 129 operations in 118 patients; 84 operations were performed in nonsmokers and 45 operations were performed in smokers. The primary outcome measure was the tympanic membrane graft take rate at the interval and most recent follow-up visits. Secondary measures included recurrence, the need for revision surgery, and hearing outcomes. RESULTS: Nonsmokers and smokers had comparable long-term rates of intact eardrums (90.6% versus 92.9%; p = 0.99). There was a trend toward a higher rate of recurrent or persistent disease requiring further operation for smokers (13.3% versus 4.7%; p = 0.09). Both groups had improvement in pure tone averages (12.1 dB in nonsmokers and 12.8 dB in smokers) and air-bone gaps (9.6 dB in nonsmokers and 5.1 dB in smokers), although the rates were not statistically significantly different from each other. The rates of success of cartilage tympanoplasty in smokers appear superior to previously published rates of noncartilage tympanoplasty. CONCLUSIONS: Cartilage tympanoplasty has success rates and postoperative audiological measures that are comparable between smokers and nonsmokers. In smokers, cartilage grafting is superior to noncartilage grafting and is recommended for any patient who smokes and is undergoing tympanoplasty.

Etiology of unilateral hearing loss in a national hereditary deafness repository.

PURPOSE: The aim of this study was to characterize the genetic, audiologic, and epidemiologic characteristics of unilateral hearing loss (HL) in a national hereditary deafness repository. MATERIALS AND METHODS: This is a prospective clinical study involving 34 subjects identified in a national hereditary deafness repository. Clinical data and family history of HL were obtained on enrollment. Candidate deafness genes were screened by single-stranded conformation polymorphism, and mutations were confirmed with sequencing. RESULTS: Thirty-four subjects (19 males, 15 females) with unilateral HL were identified, ranging in age from 2 months to 36 years. The mean age at diagnosis was 7 years, and the left ear was affected in 62% of the cases. The racial distribution of our sample was 62% white, 23% African American, and 15% Hispanic. Imaging results were available in 47%, and most (69%) were considered normal. Nineteen percent had enlarged vestibular aqueducts, 2 had ipsilateral Mondini dysplasia, and 1 had a common cavity deformity. Twenty subjects (59%) had a family history of HL, with 26% specifically reporting familial unilateral HL. Mutational screening revealed sequence variants in the GJB2 (connexin 26), GJB3 (connexin 31), TECTA, and COCH genes. Two novel mutations were detected in COCH and TECTA. CONCLUSIONS: Sequence variants in known deafness genes were detected in more than one-third of our study population, suggesting that gene/gene or gene/environmental interactions may indeed play a role in the etiology of some cases of unilateral deafness. Further prospective studies including congenital cytomegalovirus screening at birth and molecular screening of deafness genes in children with congenital unilateral HL will be required to establish the etiology of unilateral deafness with certainty.

Dural Venous Sinus Arachnoid Granulations in Patients with Pulsatile Tinnitus.

OBJECTIVE: To determine the relationship, if any, between dural venous sinus arachnoid granulations (AGs) and pulsatile tinnitus. STUDY DESIGN: Retrospective case-control study. METHODS: Between October 1999 and March 2020, magnetic resonance imaging of patients with tinnitus (pulsatile [PT] and nonpulsatile [NPT]) were assessed for the presence of dural venous sinuses AG. During the same interval, patients with AGs found incidentally on all magnetic resonance imagings ordered without an indication of tinnitus were reviewed. Demographic variables recorded included patient age, sex, race, body mass index, and a history of idiopathic intracranial hypertension (IIH) or obstructive sleep apnea. Location of AGs, when present, were recorded. RESULTS: A total of 651 (PT 250, NPT 401) were found to have AGs. AGs had a higher prevalence in PT patients (10.4% [n = 26]) versus NPT patients (0.3% [n = 1]; odds ratio, 31.0; confidence interval 4.1-234; p < 0.001). Of the 77,607 patients who had an indication for imaging other than tinnitus, 230 patients (0.30%) were found to have incidental AGs, suggesting that the NPT cohort was an adequate control. Patients with PT were more likely to have a higher body mass index, be female, be non-White, and have an existing diagnosis of IIH. For all patients with AGs, AGs were more likely to be found in the lateral sinuses (i.e., sigmoid, transverse) in the PT group (odds ratio, 8.1; confidence interval, 1.1-61.1; p = 0.0218). CONCLUSIONS: This study evaluates the association between AG and PT, finding higher rates of AG in patients with PT than in NPT. However, despite the increased prevalence of AG in patients with IIH, these data combined with existing literature would suggest that AGs are not necessarily the missing link to explain PT pathophysiology in IIH.

Complications of cochlear implantation in adults.

OBJECTIVES: We describe our experience of intraoperative and postoperative complications of cochlear implantation in an adult population. METHODS: Between April 1986 and June 2010, the senior author (A.S.) performed 449 cochlear implantations in two different institutions. Of these, 212 implantations were in adults. The operative techniques were similar in all cases. RESULTS: Complications were observed in 12 of the 212 adult cases (5.7%), of which 10 were major (4.7%) and 2 minor (1%). In 7 cases, reimplantation was necessitated by device failure (6 cases; 2.8%) or device extrusion (1 case; 0.5%). In 2 elderly patients (1%), a minor dural injury with a cerebrospinal fluid leak was controlled during the operation with temporalis fascia grafting. In 1 patient (0.5%), a subdural hematoma was observed after bipolar cauterization of a prominent diploic vein. In 2 subjects (1%), a wound infection was noted soon after implantation and was treated successfully on an outpatient basis. CONCLUSIONS: Cochlear implantation is generally a safe procedure. The most common complication was device failure. Although complications in this adult population were rather uncommon, some of them were serious, and an immediate intervention was necessary for a successful outcome.

Canalithiasis of the anterior semicircular canal (ASC): treatment options based on the possible underlying pathogenetic mechanisms.

Benign paroxysmal positional vertigo (BPPV) of the anterior semicircular canal (ASC) is an uncommon disorder currently diagnosed with the Dix-Hallpike (D-H) examination. According to the literature, nystagmus and vertigo may be more pronounced when the affected ear is either up or down. In some patients, both right and left D-H tests can trigger nystagmus with the same direction. The proposed treatment options with the addition of a different manoeuvre applied by the authors of the present study in cases of ASC lithiasis, seem to present a respective variety regarding the position of the affected ASC during the procedure of canalith repositioning. The aim of this study is to analyse the mechanisms underlying both the proposed treatment options and the clinical findings in the D-H examination. The results of this analysis stimulate further investigation, since they probably imply that repositioning manoeuvres might vary in their effectiveness when applied to different clinical subgroups of ASC BPPV.

Receptive language outcomes in children after cochlear implantation.

OBJECTIVES: The objectives of the present study were (1) to assess receptive language scores in children after cochlear implantation and compare them with scores in normal hearing children and children with hearing loss that use hearing aids and (2) to determine how demographic factors, such as age of implantation, impact language outcomes. STUDY DESIGN: Case series. SUBJECTS/METHODS: Receptive language scores in children with profound prelingual hearing loss who received cochlear implants between 1996 and 2004 were analyzed. RESULTS: Standardized language assessments were available for 36 children. The average age at implantation was 33 months. The mean language scores for implanted children were within 1 standard deviation of scores of normal hearing individuals. Children with cochlear implants had significantly higher subtest scores (P < 0.05) than children with hearing aids. Children with additional disabilities had significantly (P < 0.05) poorer language performance. CONCLUSIONS: Pediatric cochlear implant recipients acquire receptive language skills that approach those of their hearing peers and exceed those of children with hearing aids.

Cartilage "shield" grafts in revision tympanoplasty.

OBJECTIVE: To report our experience with cartilage "shield" grafts in revision tympanoplasty. STUDY DESIGN: Retrospective chart review at a tertiary referral center. Patients underwent revision tympanoplasties by replacing the entire tympanic membrane with concha cymba cartilage shaped as a shield. MAIN OUTCOME MEASURES: Successful graft take was defined as having no perforation, graft retraction, or lateralization. Hearing results were analyzed by comparing the preoperative and postoperative pure-tone average air-bone gap and speech discrimination scores. RESULTS: Forty-three patients underwent 46 procedures. Graft take was successful in 43 procedures (93.5%). There was no graft lateralization or displacement into the middle ear. An overall postoperative air-bone gap of 25 dB or less was achieved in 22 of the 39 patients (56.4%), and speech discrimination scores remained unchanged. CONCLUSION: Cartilage shield tympanoplasty is a reliable procedure for revision tympanoplasty patients, with excellent graft take and significant improvement of hearing.

Horizontal (vs. vertical) closure of the neo-pharynx is associated with superior postoperative swallowing after total laryngectomy.

We conducted a cross-sectional study to compare the horizontal and vertical methods used in the surgical closure of the neo-pharynx after total laryngectomy in terms of their effect on swallowing function, swallowing-related quality of life (QOL), and overall QOL. We also assessed the potential influence of age (≤64 vs. ≥65 yr) and the type of treatment modality (primary, salvage, or total laryngectomy with radiotherapy) on outcomes. Our final study population was made up of 34 patients-31 men and 3 women, aged 49 to 89 years (mean: 66.8)-who had undergone a total laryngectomy. One year after surgery, all patients were asked to complete the M.D. Anderson dysphagia inventory (MDADI), which quantifies swallowing function and swallowing-related QOL, and the University of Washington quality-of-life questionnaire (UW-QOL), which quantifies overall QOL. Of the 34 patients, 16 had undergone a horizontal surgical closure of their neo-pharynx and 18 a vertical closure. According to the MDADI, patients in the horizontal group experienced significantly better swallowing function/QOL; the mean composite MDADI scores were 91.5 in the horizontal group and 68.3 in the vertical group (p = 0.005). We found no significant difference in terms of overall QOL, as the respective mean UW-QOL scores were 81.0 and 80.8 (p = 0.93). The population correlation coefficient was positive in both groups, but more so in the horizontal group (ρhorizontal = 0.876 and ρvertical = 0.676). Neither age nor the type of treatment modality employed influenced swallowing function/QOL (page = 0.10, ptreatment modality = 0.78) or overall QOL (page = 0.08, ptreatment modality = 0.59). We conclude that horizontal closure of the neo-pharynx is superior to vertical closure in terms postoperative swallowing function/QOL but not overall QOL.

Type III cartilage "shield" tympanoplasty: an effective procedure for hearing improvement.

OBJECTIVE: To report the hearing results following type III cartilage "shield" tympanoplasty. STUDY DESIGN: Retrospective chart review at a tertiary referral center. METHODS: Conchal cartilage, shaped as a shield, was used to replace the entire tympanic membrane and reconstruct the ossicular chain in patients with an absent incus and diminished space between the stapes superstructure and malleolar manubrium. RESULTS: Between January 1998 and June 2005, 52 patients were treated. The mean age was 32.4 years (range, 7 to 72 years). The mean follow-up was 24 months (range, 12 to 36 months). Graft take was successful in all patients. The average hearing improvement was 11.22 dB (P<0.0001). An air-bone gap of 25 dB or less was achieved in 41 (78.8%) patients. Speech discrimination scores remained unchanged. No complications were identified. CONCLUSION: Type III cartilage "shield" tympanoplasty is an effective technique for hearing improvement in selected patients with chronic otitis media. The results of this procedure are similar to those obtained with partial ossicular replacement prosthesis.

Intracranial complications of cochlear implantation.

OBJECTIVE: To describe intracranial complications after cochlear implantation in the pediatric and adult populations. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: A chart review of the intracranial complications and their management in 345 patients undergoing cochlear implantation was undertaken. INTERVENTIONS: Variables, including age, sex, implant manufacturer, cause of deafness, intraoperative findings, and postoperative complications, were collected and analyzed. MAIN OUTCOME MEASURE: Presence of intracranial complication of cochlear implantation. RESULTS: There were 134 Nucleus-22 (Cochlear, Englewood, CO) devices, 50 Nucleus-24 devices, 118 Med-El (Durham, NC) devices, and 43 Advanced Bionics Corporation (Sylmar, CA) devices in 151 adults and 194 children. There was a 9.3% overall complication rate, with most (59%) being related to device failure. There were three intracranial complications (<1%), two in elderly individuals and one in a child. Two minor dural defects with cerebrospinal fluid leak at the site of the receiver/stimulator recess in Med-El devices were repaired intraoperatively with temporalis fascia. One elderly patient experienced an acute extensive subdural hematoma after Nucleus-24 implantation, which was treated successfully with immediate evacuation. CONCLUSION: Intracranial complication rates associated with cochlear implantation are low, although potentially very serious. Surgeons should be aware of intracranial complications, especially in older individuals, and take immediate appropriate action.

Orbital sequelae of rhinosinusitis after cochlear implantation in children.

OBJECTIVES: The objectives of this retrospective case review were to describe orbital complications in children after cochlear implantation, to define rhinosinusitis as a possible preoperative risk factor, and to suggest a possible pathophysiological mechanism for this previously unreported occurrence. METHODS: Records of children undergoing cochlear implantation over a 7-year period at a tertiary academic medical center were reviewed. Four children who experienced postoperative orbital sequelae were identified. We describe the demographics, clinical course, and radiologic findings in these children. RESULTS: The records of 91 children who underwent cochlear implantation were reviewed. The mean age was 6.0 years (range, 0.9-16.9 years). Forty-nine children (54%) were female and 51 (56%) were white. Four children developed postoperative orbital complications on the ipsilateral side to implantation. Orbital complications were characterized by periorbital edema and preseptal cellulitis necessitating prolonged hospitalization in all four children (mean length of stay, 3.3 days). Each child's orbital complication resolved with medical therapy that included intravenous antibiotics and nasal saline. Temporal bone images before implantation showed evidence of rhinosinusitis in all four cases. Of 76 available preoperative scans from the unaffected children, only 11 (14%) studies showed evidence of rhinosinusitis. CONCLUSIONS: Children with preoperative radiologic evidence of rhinosinusitis may be at risk of orbital sequelae after cochlear implantation. Positioning of the patient during surgery, length of surgery, and minor trauma to the lamina papyracea during drilling of the mastoid may be important etiologic factors. A careful review of medical history and computed tomography imaging before implantation may identify at-risk children.

High BAX/BCL2 mRNA ratio predicts favorable prognosis in laryngeal squamous cell carcinoma, particularly in patients with negative lymph nodes at the time of diagnosis.

OBJECTIVES: Laryngeal squamous cell carcinoma (LSCC), a common type of head and neck cancer, is associated with high rates of metastasis and recurrence. Therefore, accurate prognostic stratification of LSCC patients based on molecular prognostic tumor biomarkers would definitely lead to a better clinical management of this malignancy. The aim of this study was the investigation of the potential combinatorial prognostic value of BCL2 and BAX mRNA expression in LSCC. DESIGN AND METHODS: Total RNA was isolated from 105 cancerous laryngeal tissue specimens obtained from patients having undergone surgical treatment for primary LSCC. After cDNA preparation, a low-cost, in-house developed, sensitive and accurate real-time quantitative PCR (qPCR) methodology was applied for the quantification of BCL2 and BAX mRNA levels. Then, we carried out a biostatistical analysis to assess the prognostic value of the BAX/BCL2 mRNA expression ratio. RESULTS: High BAX/BCL2 mRNA expression constitutes a favorable prognosticator in LSCC, predicting significantly longer disease-free survival (P=0.011) and overall survival (P=0.014) of patients. More importantly, the significant prognostic value of the BAX/BCL2 mRNA expression appeared to be independent of the histological grade and size of the malignant laryngeal tumor as well as TNM stage, as revealed by the multivariate bootstrap Cox regression analysis. Kaplan-Meier survival analysis demonstrated also that the BAX/BCL2 ratio can stratify node-negative (N0) LSCC patients into two subgroups with significantly different DFS and OS (P=0.021 and P=0.009, respectively). CONCLUSIONS: The BAX/BCL2 mRNA ratio is a putative molecular tissue biomarker in CLL and hence deserves further validation in larger cohorts of LSCC patients.

Traumatic cholesteatoma presenting as a brain abscess: a case report.

OBJECTIVE: To discuss the clinical aspects and management of traumatic cholesteatomas of the temporal bone. STUDY DESIGN: Case report. SETTING: University hospital, tertiary referral center. PATIENT, INTERVENTION, AND RESULTS: The authors describe an uncommon case of otogenic brain abscess resulting from an infected cholesteatoma arising from an old temporal bone fracture line involving the external auditory canal in an otherwise healthy 21-year-old man. The patient was successfully treated with brain abscess drainage, tympanomastoidectomy, and broad-spectrum intravenous antibiotic therapy. CONCLUSIONS: The authors recommend long-term follow-up in any patient with a longitudinal or mixed temporal bone fracture with low threshold for obtaining temporal bone computer tomography imaging for any new otologic complaints. Traumatic cholesteatomas complicated by brain abscess should be treated with broad-spectrum intravenous antibiotic therapy and aggressive surgical intervention.

Cartilage shield tympanoplasty: a reliable technique.

OBJECTIVE: Cartilage shield tympanoplasty is a procedure for repairing total tympanic membrane perforations. This procedure is indicated primarily for patients with total perforations, severely atelectatic tympanic membranes, and failures of previous tympanoplasty associated with chronic eustachian tube dysfunction. Although the graft take of this technique has been reported to be excellent, there have been concerns regarding hearing results because it replaces the entire tympanic membrane with cartilage. The purpose of this study was to report our experience with this technique. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary care hospital: Virginia Commonwealth University Medical Center, Richmond, Virginia, USA. PATIENTS: Between 1998 and 2003, 62 patients were identified who had a cartilage shield tympanoplasty. The patients' ages ranged from 7 to 72 years (mean, 32 years). INTERVENTION: Therapeutic. MAIN OUTCOME MEASURES: Graft take was evaluated in all patients and postoperative complications were noted. In 58 patients, pre- and postoperative audiograms were available and pure-tone average air-bone gaps were compared using the Student's t test. RESULTS: Graft take was accomplished in 61 patients (98.4%) and there were no postoperative complications. The average preoperative and postoperative pure-tone average air-bone gap was 32.4 +/- 14.1 dB and 24 +/- 13.7 dB, respectively (p < 0.005). CONCLUSION: This study reveals that cartilage shield tympanoplasty has a high degree of graft take, and hearing results are satisfactory.

Localization of biotinidase in the brain: implications for its role in hearing loss in biotinidase deficiency.

Biotinidase deficiency is an autosomal recessively inherited disorder characterized by neurological and cutaneous features, including sensorineural hearing loss. Although many of the features of the disorder are reversible following treatment with biotin, the hearing loss appears to be irreversible. To better characterize the nature of the hearing loss in this disorder, location of the expression and presence of biotinidase within the brain was examined using Northern blot analysis, in vitro hybridization of a cDNA panel, and immunohistochemical staining. Results indicate low, but detectable expression of biotinidase throughout the brain, but increased concentrations of biotinidase within the dorsal cochlear nucleus, ventral cochlear nucleus, and superior olivary complex of the brainstem, as well as, in the hair cells and spiral ganglion of the cochlea. These findings suggest that biotinidase and possibly biotin plays an important role in hearing.