Rate Of Sensorineural Hearing Loss Research Articles

Association of Glycemic Index Using HbA1c and Sensorineural Hearing Loss in Diabetes Mellitus Type 2 Patients: A Systematic Review and Meta-Analysis

ABSTRACT
 Objective: To systematically review the available evidence on the association of HBA1c levels and development of sensorineural hearing loss and to quantitatively analyze the available data on HBA1c levels in patients with type 2 diabetes mellitus and sensorineural hearing loss to determine an HbA1c level that may be associated with the risk of having sensorineural hearing loss.
 Methods:
 Design: Systematic Review and Meta-analysis Eligibility Criteria: Cross-sectional studies, or cohort studies which were limited to English language that investigated the correlation of glycemic index using HBA1c and sensorineural hearing loss among adult type 2 diabetic patients which were done from January 2010 to December 2021. Studies with no published outcome, incomplete data or that were ongoing as of August 1, 2022 were also excluded.
 Information Sources: MEDLINE (through PubMed), Cochrane Library, Scopus, Embase (through OVID@journal), Directory of Open Access Journals (DOAJ), Google Scholar and HERDIN Plus
 Risk of Bias: Risk of Bias was assessed using the Guidelines for Cochrane Collaboration Synthesis of Results: Results were presented using forest plots for representation.Results: A total of 8 studies were reviewed with 2,103 participants in all. Six articles compared hearing loss incidence between diabetic and non-diabetic patients. Overall, there were a total of 881 diabetic patients and 1222 non-diabetic patients. There was a significantly lower incidence of sensorineural hearing loss in non-diabetic patients with a risk ratio of 1.89, 95% CI [1.65, 2.16]. Three articles compared the HbA1c levels of diabetic patients with or without sensorineural hearing loss. Diabetic patients without sensorineural hearing loss had significantly lower HbA1c levels compared to those with sensorineural hearing loss with mean difference of 1.04, 95%CI [0.82, 1.25].Conclusion: In conclusion, this meta-analysis showed a higher prevalence rate of sensorineural hearing loss among patients with diabetes mellitus compared to non-diabetic patients. Moreover, poor glycemic control among diabetic patients with a glycemic index based on HbA1c of more than 8.3 (6.97-9.6) is associated with sensorineural hearing loss.

An endoscopic approach to stapes surgery needs less external auditory canal wall removal than a microscopic approach

Background Although many advantages of endoscopic stapes surgery have been reported, there is little objective data on whether it provides better visualization than the microscopic approach. Objectives To evaluate and compare audiological results, external auditory canal wall removal area, and adverse event rates between endoscopic and microscopic stapes surgery. Material and methods Data from patients who received stapedotomy were collected. Pre and postoperative audiometry, procedure-related parameters, and adverse events were analyzed in conventional microscopic and endoscopic groups. Results There were no differences in procedure time or postoperative hearing between the endoscopic and microscopic surgery groups. The mean procedure times were 87.3 ± 18.9 min in the endoscopic group and 79.9 ± 23.5 min in the microscopic group. The mean postoperative air-bone gaps were 10.9 ± 8.3 dB in the endoscopic group and 10.5 ± 7.8 dB in the microscopic group. There were no differences in the rate of sensorineural hearing loss, postoperative pain, facial palsy, vertigo, or dysgeusia between the two groups. The bony removal area of the posterosuperior external auditory canal wall measured by two observers was significantly less in the endoscopic group than in the microscopic group. Conclusions Endoscopic stapedotomy needed less external auditory canal wall removal and showed similar audiological outcomes to microscopic stapedotomy.

Otitis Media with Persistent Inflammation is a Common Cause of Sensorineural Hearing Loss

Objective: The purpose of the research was to determine the rate of sensorineural hearing loss that is associated with chronic otitis media. Study Design: A Descriptive Cross-sectional study Place and Duration: Frontier Medical College, Abbottabad, from June, 2021 to November, 2021. Methods: Total 87 patients of both genders had age 15-55 years were presented. All the included patients had chronic suppurative otitis media clinical symptoms. Patients were enrolled after getting informed written consent from the patients. Through the use of a pure tone audiogram, we were able to determine the mean threshold of voice frequencies for sick as well as normal contralateral ears. In sick ears, a mean of more than 25 dB was considered to represent a positive case of SNHL. SPSS 24.0 was used to analyze all data. Results: We found that 49 (56.9%) patients were males and 38 (43.1%) patients were females. 17 (19.5%) cases had age 15-25 years, 35 (40.2%) patients had age 26-35 years, 25 (28.7%) patients had age 36-45 years and 10 (11.5%) patients were aged between 46-55 years. Majority of the cases 51 (58.6%) were from urban areas. There were 50 (57.5%) cases had chronic otitis media duration <10 months and remaining had disease duration >10 months. Frequency of sensorineural hearing loss was found in 47 (54.02%) cases. Among 47 cases SNHL, 32 cases were aged between 31-55 years. Conclusion: We concluded in this study that patients with chronic otitis media had significantly higher number of sensorineural hearing loss, Among SBHL cases, patients were mostly aged between 31-55 years and disease duration was >10months. Keywords: Otitis Media, Sensorineural Hearing Loss,

Association of Metabolic Syndrome with Sensorineural Hearing Loss

The prevalence of sensorineural hearing loss has increased along with increases in life expectancy and exposure to noisy environments. Metabolic syndrome (MetS) is a cluster of co-occurring conditions that increase the risk of heart disease, stroke and type 2 diabetes, along with other conditions that affect the blood vessels. Components of MetS include insulin resistance, body weight, lipid concentration, blood pressure, and blood glucose concentration, as well as other features of insulin resistance such as microalbuminuria. MetS has become a major public health problem affecting 20–30% of the global population. This study utilized health examination to investigate whether metabolic syndrome was related to hearing loss. Methods: A total of 94,223 people who underwent health check-ups, including hearing tests, from January 2010 to December 2020 were evaluated. Subjects were divided into two groups, with and without metabolic syndrome. In addition, Scopus, Embase, PubMed, and Cochrane libraries were systematically searched, using keywords such as “hearing loss” and “metabolic syndrome”, for studies that evaluated the relationship between the two. Results: Of the 94,223 subjects, 11,414 (12.1%) had metabolic syndrome and 82,809 did not. The mean ages of subjects in the two groups were 46.1 and 43.9 years, respectively. A comparison of hearing thresholds by age in subjects with and without metabolic syndrome showed that the average pure tone hearing thresholds were significantly higher in subjects with metabolic syndrome than in subjects without it in all age groups. (p < 0.001) Rates of hearing loss in subjects with 0, 1, 2, 3, 4, and 5 of the components of metabolic syndrome were 7.9%, 12.1%, 13.8%, 13.8%, 15.5% and 16.3%, respectively, indicating a significant association between the number of components of metabolic syndrome and the rate of hearing loss (p < 0.0001). The odds ratio of hearing loss was significantly higher in subjects with four components of metabolic syndrome: waist circumference, blood pressure, and triglyceride and fasting blood sugar concentrations (p < 0.0001). Conclusions: The number of components of the metabolic syndrome is positively correlated with the rate of sensorineural hearing loss.

Sensorineural Hearing Loss After Balloon Eustachian Tube Dilatation.

Objective: Eustachian tube function is of central importance for the ventilation of the middle ear. A dysfunction can be associated with chronic otitis media, and cholesteatoma. Balloon Eustachian tube dilatation (BET) is a treatment option used to solve eustachian tube dysfunction. Although BET is widely performed, little is known about the occurrence rate of the complications associated with BET. The aim of the present study was to observe the rate of sensorineural hearing loss (SNHL) after BET.Methods: We retrospectively evaluated in a chart review 1,547 patients and 2,614 procedures of BET performed in a single center between 2015 and 2019 using the Spiggle and Theis, Overath, Germany eustachian tube dilatation system.Results: We observed seven cases of SNHL after BET. In two cases, the SNHL persisted, and in five cases, the SNHL was transient. In two cases of SNHL, a simultaneous tympanoplasty was performed. The overall rate of SNHL per procedure is 0.3%. The rate of permanent SNHL is 0.08%.Conclusion: BET has a low rate of SNHL. Rapid middle ear pressure changes are assumed to cause BET-related hearing loss.

Adolescent Obesity Is an Independent Risk Factor for Sensorineural Hearing Loss: Results From the National Health and Nutrition Examination Survey 2005 to 2010

We investigated the hypothesis that childhood obesity is a risk factor for sensorineural hearing loss (SNHL) independent of other metabolic risk factors. A complex, multistage, stratified geographic area design for collecting representative data from noninstitutionalized US population. A total of 5,638 adolescents between age 12 and 19 from the NHANES database (2005-2010) were studied. Subjects with body mass index >= 95th percentile were classified as obese. SNHL was defined as average pure-tone greater than 15 dB HL for 0.5, 1, and 2 kHz or 3, 4, 6, and 8 kHz in at least 1 ear. Multivariable logistic regression models assessed incident hearing loss odds across obese patients in comparison with normal weight individuals (5th-85th percentile). Multivariable models included age, sex, socioeconomic status, race, smoke exposure, high density lipoprotein level, triglyceride level, elevated blood pressure measurement, hemoglobin A1C level, and C-reactive protein level. The rate of SNHL was 21.5% in obese and 13.44% in normal weight adolescents (p < 0.0001). In multivariable analyses, obesity was associated with 1.73-fold increase in the odds of SNHL (95% CI: 1.25-2.40, p value = 0.006). Potentially confounding and mediating factors had minimal effect on the odds of SNHL in obese study participants (OR range of 1.69-1.75, all p values <= 0.01). Obesity is associated with higher prevalence of SNHL in adolescents independent of other potential risk factors. Future longitudinal investigations and mechanistic studies are warranted.

Down syndrome: An electrophysiological and radiological profile

To assess the hearing status of trisomy 21 patients by analyzing electrophysiological and radiological findings of any correlation between hearing impairment and major or minor inner ear malformations. Prospective radiological and electrophysiological study. A group of 34 ears of Down syndrome subjects and 20 ears of a volunteer age- and sex-matched control group of 10 normal subjects were studied electrophysiologically by means of otoacoustic emissions and auditory brainstem response. Temporal bone computed tomography (CT) scans were carried out in both groups; radiological findings were compared. Inner ear structure measurements were applied attempting to disclose subtle bony labyrinthine anomalies. The findings from both groups were statistically analyzed employing the t test. The rate of sensorineural hearing loss (SNHL) in Down syndrome group was 41%. Temporal bone CT scans showed no ossicular malformation in all Down syndrome cases. Major inner ear abnormalities were disclosed in 5.8%; they corresponded to two cases of lateral semicircular canal dysplasia detected on CT images by visual inspection. The application of various inner ear structure measurements increased the overall detection rate of common inner ear malformations to 47%. A statistically significant correlation was found between hearing level and vestibule length (P = .009) and internal auditory canal length (P = .028). Vestibular aqueduct width was correlated to different otic abnormalities. SNHL is mainly secondary to the underestimated subtle inner ear malformations that are adequately demonstrated by adopting standardized inner ear structure measurements on petrous bone imaging. Vestibular height and internal auditory canal length were correlated to SNHL. 3b.

Relationship between clinical features and therapeutic approach for benign paroxysmal positional vertigo outcomes

To examine the clinical features, age and gender distribution of patients, treatment methods, and outcomes of benign paroxysmal positional vertigo. This paper reports a review of 357 patients treated for this condition at a single institution over a duration of 5 years. Patients with posterior canal benign paroxysmal positional vertigo were divided into two groups: one group underwent the Epley manoeuvre and the other received medication. The lateral canal canalolithiasis patients were also divided into two groups: one underwent the Lempert manoeuvre and the other received medication. Lastly, the lateral canal cupulolithiasis patients were treated with medication and non-specific physical techniques. For patients with posterior canal benign paroxysmal positional vertigo, resolution time was significantly shorter in the Epley manoeuvre group than in the medication group. For the lateral canal canalolithiasis patients, resolution time was significantly shorter in the Lempert manoeuvre group than in the medication group. Resolution time was significantly longer in the lateral canal cupulolithiasis patients than in the other patients. The average age of patients increased with the number of recurrences, as did predominance in females. Average age and rate of sensorineural hearing loss were significantly higher in patients with intractable benign paroxysmal positional vertigo compared with those in the curable benign paroxysmal positional vertigo group.

The Use of KTP Laser in Revision Stapedectomy

Revision stapes operations have lower success rates than primary surgeries. Success rates have increased since the introduction of the laser. Visible (KTP, argon) and infrared (CO2) spectrum lasers have been used in stapes surgery. A concern has been raised regarding the use of visible lasers in revision stapedectomy because of their properties of tissue penetrance. The objective of our study is to evaluate a series of patients who underwent revision stapedectomy with the use of KTP laser to determine whether there is an increased risk of sensorineural hearing loss associated with the use of this laser. Patients with otosclerosis requiring revision stapedectomy. Revision stapedectomy or stapedotomy were performed using the KTP laser. The primary outcome evaluated was the incidence of postoperative sensorineural hearing loss. Improvement in air-bone gap was evaluated as a secondary outcome. There were no cases of postoperative sensorineural hearing loss or deafness. Approximately 56.5% of patients achieved closure of the postoperative ABG to within 10 dB; 91.3% of patients achieved a postoperative ABG within 20 dB. The KTP laser was used most commonly to lyse fibrous adhesions. Although there is a theoretical risk of penetrance of the KTP laser through the oval window and underlying perilymph with resultant damage to the sense organ-containing endolymph, this theoretical risk did not translate into an increased rate of sensorineural hearing loss in our series. The KTP laser allowed for less traumatic clearing of middle ear adhesions and good hearing results.

Hearing loss in children with mitochondrial disorders

Objective At least 1–5 children per 1000 suffer from congenital hearing loss, and 50% of these cases can be attributed to genetic causes. It has been estimated that 1% of pre-lingual hearing loss is due to mutations in mitochondrial DNA. Previous literature reports audiometric data for few patients, usually less than 20 per study. The goal of this study was to characterize the hearing loss associated with mitochondrial mutations and determine whether previously characterized patterns of hearing loss in these patients (progressive, sensorineural, high frequency losses) are found in our population as well. Methods An IRB-approved retrospective chart review of the electronic medical records in the Nemours/Alfred I. dupont Hospital for Children system from January 2004 to October 2009 (a five-year period) was undertaken using ICD-9 codes 277.87 (mitochondrial disorder) and 359.89BA (mitochondrial myopathy). These 149 records were then evaluated for audiologic data, resulting in 26 charts with both a mitochondrial disorder and hearing evaluation. Results Of 26 patients with known mitochondrial disorders and audiometric documentation, 15 (58%) had hearing loss, and 11 patients had normal hearing (42%). Ten patients had sensorineural hearing loss (38%), two patients had conductive hearing loss (7.7%), one patient had a mixed hearing loss (3.8%), and two patients had an as yet undefined hearing loss (ABR had not yet been performed at the time of this study) (7.7%). Conclusion In comparison with previous studies, generally including less than 20 patients, this is one of the largest collections of audiometric data on children with mitochondrial disorders. Unlike prior studies describing a progressive, sensorineural loss across all frequencies or mainly affecting high frequencies, the hearing loss in our patients was more variable including low frequency losses, mid-frequency losses, and conductive losses and was often not progressive or even improved. Our overall 38% rate of sensorineural hearing loss correlates well with previous case series; this study clearly justifies the use of routine audiometric screening in children with mitochondrial disorders, including use of ABR and OAEs as ASND can be seen in this population, as well as repeat testing over time to evaluate for progression.

Comparison of the Traditional and New Classification System of Temporal Bone Fractures for Clinical Relevance

Objective: The traditional classification of temporal bone fracture is subdivided into transverse, longitudinal, and mixed fracture. But questions have arisen about its limited correlation with clinical symptoms. Recent studies have been introduced, including petrous bone invasion and otic capsule violation. And a newer system is introduced about invasion of four areas of temporal bone. Method: Clinical records and computed tomography scans were reviewed retrospectively on patients diagnosed with temporal bone fractures for the last seven years. We evaluated correlations of clinical presentation with each category of temporal bone fracture. Results: A total of 129 temporal bone fracture patients were reviewed. In the traditional classification scheme, transverse fracture presented at a significantly higher rate of sensorineural hearing loss (SNHL). The invasion of petrous bone system showed a significantly higher rate of SNHL, dizziness, and perforation of the tympanic membrane. The otic capsule-based system demonstrated a statistically significant rate and severity of SNHL among patients with otic capsule violation ( P &lt; .05). Conclusion: Although the traditional classification scheme, otic capsule involvement, and four areas of temporal bone systems did not demonstrate clear clinical correlations, petrous bone involvement showed significant correlations. So a petrous involvement based scheme better reflects clinical aspects.

Screening Newborns for Congenital Cytomegalovirus Infection

IN THIS ISSUE OF JAMA, BOPPANA AND COLLEAGUES 1 DEscribe their attempts to validate universal screening methods for congenital cytomegalovirus (CMV) infection, the most common congenital viral infection in the United States and many other regions, by using polymerase chain reaction (PCR) analysis of newborn dried blood spots. Congenital CMV infection remains an important public health problem not only because it has accounted for as much or more disability over the past 50 years than was associated with congenital rubella syndrome, but also because CMV infection, whether symptomatic or silent at birth, represents the most common nongenetic cause of permanent hearing loss among children in the United States. Congenital CMV infection causes a substantial proportion of sensorineural hearing loss even in regions with a low prevalence. Culture surveillance of nearly 4000 newborns in Utah revealed a congenital infection rate of 0.29%, corresponding to approximately 3 infected infants for every 1000 newborns or 165 infected newborns annually statewide, given the state’s current birth rate. Because as many as 10% of these infants will have CMV disease, a condition associated with a 30% to 50% prevalence of sensorineural hearing loss, and the remainder will have silent CMV infection, a condition conservatively associated with a 6% to 8% rate of sensorineural hearing loss, CMV accounts for hearing loss in 15 to 20 children each year in Utah, or approximately 20% of the 90 to 120 Utah children identified with permanent hearing loss annually (J. C. Carey, University of Utah School of Medicine, written communication, March 2010). Worldwide, thousands of infants have CMV-induced hearing loss annually. Universal newborn screening for many disorders has immense public health benefits. The history of phenylketonuria (PKU) illustrates the enormous benefit of such strategies. Universal screening of newborn hearing has led to improvement in language outcomes, with greater improvements when deaf infants are identified earlier and interventions are begun sooner. Thus, it seems logical to infer that if CMV-infected infants can be identified in the neonatal period, hearing could be tracked closely, interventions could begin early, and the hearing outcomes of congenital CMV infection might be improved. However, 2 challenges confront universal screening for congenital CMV infection. First, universal screening is only justified when an effective intervention exists that prevents adverse health outcomes, such as averting intellectual disability when infants who lack phenylalanine hydroxylase initiate a diet low in phenylalanine early in life. At present, no such solution exists for CMV. Although neonatal ganciclovir therapy improves hearing outcomes in some infants, the benefits are not universal and the risk/benefit ratio of ganciclovir therapy in infants with silent congenital CMV infection has not been sufficiently well-established to recommend such therapy for all CMV-infected infants. Nonetheless, knowing which infants are infected with CMV has benefit through early identification of infants at risk of CMVinduced hearing loss and may have additional benefits in the future when new and more effective antiviral strategies become available. The second challenge, the one addressed by Boppana et al, is the availability of a highly sensitive and specific screening method. Neonatal screening of hearing alone is insufficient to detect all CMV-infected infants because most infants with congenital CMV infection have normal hearing in the neonatal period. Fowler et al demonstrated that nearly 20% of the children with hearing loss due to silent congenital CMV infection have delayed onset of sensorineural hearing loss. In addition, most infants with congenital hearing loss do not have CMV infection. In 1996, when Barbi et al demonstrated that newborn dried blood spots and PCR assays could be used to detect CMV DNA in the blood of congenitally infected infants, there was optimism that the opportunity for universal screening for CMV had arrived. However, the study by Barbi et al was not population-based and did not have a sufficient sample size to establish convincingly the sensitivity and specificity of mass screening of newborn dried blood spots. In contrast, the large, prospective study by Boppana et al shows that even though real-time PCR has excellent specificity (approaching 100%), the sensitivity of the PCR

Extremely low birth weight infants are at high risk for auditory neuropathy

Auditory neuropathy (AN) is a condition in which transmission of sound to the brain is abnormal. This is reflected as an electrophysiologic profile of normal otoacoustic emissions (OAE), with abnormal auditory brainstem evoked responses (ABR). Functionally speech perception is impaired and management strategies remain controversial. AN can be missed if high-risk newborns are screened for hearing loss with only OAE testing. The rate of sensorineural hearing loss (SNHL) in high-risk nursery infants is 10 times greater compared with normal term newborns. Therefore, we hypothesize that infants from the neonatal intensive care unit (NICU) are at significantly higher risk for AN than normal term infants. The objective of this study is to establish a prevalence rate and characterize risk factors for NICU graduates who demonstrate the AN electrophysiologic profile. This retrospective study examined infants admitted to the NICU at Kapi'olani Medical Center for Women and Children in Honolulu, HI from 1999 through 2003. Infants were screened with automated ABR. Diagnostic testing and OAE were performed before discharge if the ABR was abnormal. Hospital courses of 24 AN, 71 SNHL and 95 gestational age (GA)-matched control infants with normal hearing were reviewed. With a SNHL prevalence of 16.7/1000, the rate for AN was 5.6/1000 NICU infants. Compared to infants with SNHL, infants with AN were significantly younger (GA 28.3+/-4.8 AN vs 32.9+/-5.2 weeks SNHL, P<0.0001) and smaller (BW 1318+/-894 AN vs 1968+/-1006 g SNHL). Nearly two-thirds of the AN infants were ELBW and had significantly longer hospital stays compared to SNHL infants of the same birth weight group. Exposure to furosemide, aminoglycosides, vancomycin or dexamethasone was associated with increased AN but not SNHL. Peak bilirubin level correlated with SNHL but not AN. Low birth weight NICU infants are at significant risk for AN. ELBW infants are at significantly higher risk for both AN and SNHL. Infants admitted to the NICU should be routinely screened by automated ABR and if abnormal, further evaluation should be started before hospital discharge. Early identification of AN will result in better understanding of this disorder and lead to the development of appropriate intervention strategies.

Transcanal Approach to the Singular Nerve

Intractable benign paroxysmal positional vertigo is rare, and surgery is indicated in only a very small number of cases. Transcanal singular neurectomy is considered a difficult and risky procedure possibly leading to hearing loss and vertigo. The objective of this study was to evaluate the feasibility of the singular neurectomy through the external ear canal in an attempt to explain the contradictory results of previous reports of anatomists and of surgeons who abandoned the technique, considering that the singular neurectomy could not be reached via the external auditory canal without damaging the labyrinth. Anatomical study on 100 halves of human heads in which the canal of the singular nerve (SN) was identified and opened at its extremities, the internal auditory canal and the ampulla of the posterior semicircular canal, via a posterior fossa approach. Next, the canal of the SN was dissected via the external auditory canal, at the floor of the round window (RW) niche. The relation of the SN canal to the ampulla of the posterior semicircular canal was evaluated. In 90 cases, the canal was transected medially to and away from the ampulla of the posterior semicircular canal, and in 8, at its emergence from the posterior ampullary recess. In these 98 cases, the RW membrane and the bony labyrinth were kept intact. In two cases, the canal of the SN could not be reached at the floor of the RW niche. Singular neurectomy is feasible via the external auditory canal, without damaging the RW membrane or the labyrinth in 98% of the cases. Because singular neurectomy is indicated in a very small number of cases, it is difficult to master this particular surgical procedure. This may explain why most surgeons abandoned the technique after a few attempts, followed by an unacceptable rate of sensorineural hearing loss.

Hearing results with the titanium ossicular replacement prostheses

The purpose was to study the hearing results in patients receiving a Kurz titanium Bell partial ossicular replacement prosthesis (PORP) or an Aerial total ossicular replacement prosthesis (TORP). The study was a retrospective chart review in a tertiary otologic referral center. A computerized otologic database was used to identify 111 patients implanted with either a PORP or TORP prosthesis. Audiograms were reviewed and air-bone gaps were calculated for each patient. The improvement of the average air-bone gap (ABG) was 10.2 and 12.7 dB at 3 and 20 months after ossiculoplasty, respectively. Sixty-six percent of patients (73/111) had a postoperative air-bone gap of 20 dB or less. The ABG for the titanium PORP prosthesis was 14.3+/-9.7 dB, compared with 25.2+/-13.7 dB for the TORP prosthesis (P <0.05). The ABG to within 20 dB or less was obtained in the PORP group in 77% of the cases, versus 52% of the cases in the TORP group (P <0.05). Two extrusions of the prostheses were observed at 17 and 20 months after surgery (1.8%). Revision procedures for functional failure were carried out in 20 patients (18%). The rate of sensorineural hearing loss was 3.6%. The major factors influencing good audiometric results were the surgical procedure preserving the external auditory canal and the presence of the stapes. The best hearing results were achieved when a PORP was used in an intact canal wall (ICW) procedure, and the worst hearing results were achieved when a TORP was used in a canal wall down (CWD) procedure. The titanium Kurz prosthesis has been an effective implant at our institution for ossicular reconstruction.

Coenzyme Q-10 treatment of patients with a 7445A→G mitochondrial DNA mutation stops the progression of hearing loss

ConclusionCoQ10 may be helpful in delaying the progression of hearing loss in patients with the 7445A→G mitochondrial mutation.ObjectiveTo assess the effect of an antioxidant drug (Coenzyme Q-10) on the hearing level of patients with the mitochondrial DNA 7445A→G mutation and associated sensorineural hearing loss (SNHL).Material and methodsWe identified three patients with bilateral non-syndromic SNHL harboring the mitochondrial 7445A→G mutation. Two patients had a family history of hearing loss with a strong matrilineal inheritance. The other patient did not have a family history of hearing loss. Two patients (1 with familial and 1 with sporadic SNHL) received treatment with 75 mg of Coenzyme Q-10 (CoQ10) twice a day for 1 year. The remaining patient with a familial form of hearing loss did not agree to take the treatment. Average bone conduction pure-tone thresholds for 0.5, 1, 2 and 4 kHz were obtained before and after diagnosis of mitochondrial hearing loss, and before and after treatment with CoQ10.ResultsCoQ10-treated patients did not show any additional deterioration of their SNHL after 12 (familial case) and 13 months (sporadic case). The progression rate of SNHL was 6 dB/year in the 2 years prior to initiation of treatment in the familial case who received CoQ10 treatment. One year after being diagnosed with mitochondrial hearing loss, the patient who refused CoQ10 treatment exhibited an 11-dB deterioration of his hearing thresholds. There were no side-effects related to treatment with CoQ10.

Stapedectomy Outcomes: Titanium versus Teflon Wire Prosthesis

To compare the effectiveness of two stapes prostheses in hearing improvement of patients undergoing stapes surgery for otosclerosis. Retrospective chart review. Titanium and Teflon wire stapes prostheses were compared with regard to effectiveness in closing the air-bone gap. The charts of 461 stapedectomies performed by one surgeon from 1996 to 2001 were reviewed. Patients who underwent stapedectomy for reasons other than otosclerosis, revision cases, and those with inadequate preoperative or postoperative bone-conduction threshold data were excluded. Small fenestra technique using either laser or drill was used for all patients. Inclusion criteria were met by 218 patients. Patients were then grouped according to type of prosthesis used, and hearing outcomes were compared. Measured outcomes were four frequency air-bone gap closure, pure-tone threshold, and rate of sensorineural hearing loss (SNHL). The study group was comprised of 35 titanium and 183 Teflon wire prostheses. Closure of the air-bone gap to less than 10 dB was achieved in 86% of the patients with Teflon prosthesis compared with 71% of those with titanium prostheses. The groups were equivalent in regard to site of otosclerotic disease as well as technique, laser or drill, used to create the fenestra. Rate of SNHL was low for both groups and not significantly different. Both prostheses provided comparable results, although the Teflon platinum wire prosthesis was slightly superior. The smaller numbers in the titanium group may confound these results. The design of the titanium prosthesis provides a crimp that is circumferential around the incus, and that prosthesis was selected in cases with a narrow incus. The selection bias may also influence the results seen in this study.

Stapedectomy in congenital stapes fixation: Are hearing outcomes poorer?

Objectives: To evaluate outcomes of stapedectomy surgery for congenital stapes fixation. Methods: The charts of 463 stapedectomies performed by one surgeon from 1996–2003 were reviewed. Patients with a history of childhood hearing loss and operative findings consistent with congenital fixation of the stapes were included. Patients with a history of trauma or chronic otitis media and those with otosclerosis were excluded. Thirty-six patients underwent stapedectomy for congenital fixation. Revision cases and those with inadequate bone conduction threshold data were excluded. Inclusion criteria were met for 22 patients. Operative findings and hearing outcomes were analyzed. Results: The study group comprised 22 stapedectomies. Closure of the air-bone gap to less than 10 dB was achieved in 54.5% of the cases and 77.1% had closure within 20 dB. A gap of more than 30 dB remained in 13.6% of the cases. The rate of sensorineural hearing loss was 13.6%. In addition to an excluded case that was not reconstructed due to facial nerve position, 3 of the included patients were found to have a dehiscent facial nerve at surgery. Abnormalities of the malleus or incus were not found in any of the included patients. Conclusions: We recently reviewed our stapedectomy results in otosclerosis patients and found closure to within 10 dB to be 86%. This difference in results likely reflects subtle anatomic variations in the congenital group that affect the mobility of the prosthesis. Despite the difference in results, stapedectomy for congenital fixation remains an effective technique to achieve significant hearing improvement in the majority of patients.

Congenital cytomegalovirus infection and neonatal auditory screening

Auditory screening of newborn infants has been recommended on the basis of the presence of risk criteria, including congenital infection. We assessed the ability of risk criteria-based neonatal auditory brain stem response to identify infants with hearing loss resulting from congenital cytomegalovirus (CMV) infection. Data from 6 1/2 years of risk criteria-based neonatal auditory screening were compared with the results of screening of all newborn infants for congenital CMV infection. Infants with congenital CMV infection received follow-up hearing evaluations. Congenital CMV infection was found in 167 (1.3%) of 12,371 infants; 134 had follow-up hearing evaluations, and 14 (10.4%) had confirmed sensorineural hearing loss. The rate of sensorineural hearing loss resulting from congenital CMV infection was 14 per 12,371 infants, of 1.1 per 1000 live births; the rate of bilateral loss > or = 50 dB was 0.6 per 1000. Although 2036 infants received auditory screening because of risk criteria, only 34 (20%) of 167 infants with congenital CMV infection were included. Only 2 (14%) of 14 children with sensorineural hearing loss caused by CMV were identified by risk criteria-based screening. We conclude that congenital CMV infection is an important cause of hearing impairment. Neonatal auditory screening based on the presence of risk criteria will fail to identify the majority of cases of sensorineural hearing loss caused by congenital CMV infection.